Nomenclature for congenital heart disease

Introduction

The art and science of outcomes analysis and quality improvement for pediatric and congenital cardiac care continues to evolve. This chapter reviews the evolution of systems of nomenclature and classification for pediatric and congenital cardiac care and presents a global system of nomenclature for pediatric and congenital cardiac care that unifies clinical and administrative nomenclature.

Congenital cardiac malformations are the most common types of birth defects. In 1936, Maude Abbott, curator of the McGill Pathological Museum at McGill University in Montreal, Canada, published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease (CHD). Since that time, substantial progress has been made in the standardization of nomenclature and classification for pediatric and congenital cardiac care.

The Merriam-Webster Dictionary [ https://www.merriam-webster.com/ ] defines nomenclature as “a system or set of names, terms, or symbols in a particular science, discipline or field of knowledge.” Meanwhile, the Cambridge Dictionary [ https://dictionary.cambridge.org/us/dictionary/english/ ] defines nomenclature as “a system for naming things in a particular area of science.” This chapter will review nomenclature for pediatric and congenital cardiac care, discuss historical considerations and the basic principles of nomenclature, and summarize The International Pediatric and Congenital Cardiac Code ( IPCCC ) , which is the pediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases ( ICD-11 ) . ^, ^, ^,

Contemporary estimates of the prevalence of congenital cardiac disease range from 8 to 12 per 1000 live births. Survival after surgery for congenital heart defects has increased over the past decade, especially for the most complex operations. The etiology of this improvement is multifactorial, but the ability to compare risk-stratified and risk-adjusted outcomes at individual programs to national and international aggregate benchmarks certainly facilitated these improved cardiac surgical outcomes over time. This benchmarking and improvement in quality requires standardization of the nomenclature and classification of pediatric and congenital cardiac disease.

Historical considerations

Advances in the nomenclature and classification of pediatric and congenital cardiac care and the congenitally malformed heart evolved from the major contributions of pioneers in the descriptions and understanding of the anatomy and morphology of congenital cardiac malformations, including Maude Abbott from McGill University in Montreal, Canada, Lodewyk H.S. van Mierop from University of Florida, Richard and Stella Van Praagh from Harvard University and Boston Children’s Hospital, and Robert H. Anderson from The Great Ormond Street Hospital for Children in London, England, United Kingdom.

The development of classification schemes specific to the congenitally malformed heart began with Maude Abbott’s pioneering work in the early 1900s. ^, Her landmark publication in 1936, Atlas of Congenital Cardiac Disease, was the first formal attempt to classify the lesions seen in the congenitally malformed heart. ^,

•
In 1906, Maude Abbott cofounded the International Association of Medical Museums, with William Osler. In 1907, she became the International Secretary of the International Association of Medical Museums, and she edited the institution’s articles for 31 years (1907–1938).
•
In 1910, Abbott was awarded an honorary medical degree from McGill and was made a lecturer in Pathology (8 years prior to the university admitting female students to the Faculty of Medicine).
•
In 1936, Abbott published the Atlas of Congenital Cardiac Disease , which illustrated a new classification system and described records of over a thousand clinical and postmortem cases.

In the 1990s, efforts were made to create a truly international system of nomenclature and classification to support pediatric and congenital cardiac care. Prior to these efforts, multiple systems were used across the world and were the basis of internal, national, and even international registries and databases of pediatric and congenital cardiac care. ^, ^,

Facilitated by advances in information technology, two independent international pediatric and congenital cardiac systems of nomenclature and classification were established in the 1990s:

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The European Paediatric Cardiac Code (EPCC) of The Association for European Paediatric and Congenital Cardiology (AEPC), published in 2000 ^,
•
The nomenclature system of the International Congenital Heart Surgery Nomenclature and Database Project of The Society of Thoracic Surgeons (STS) in North America, The European Association for Cardio-Thoracic Surgery (EACTS), and The European Congenital Heart Defects Database of The European Congenital Heart Surgeons Foundation (ECHSF; renamed The European Congenital Heart Surgeons Association [ECHSA] in 2003), published in 2000 in The Annals of Thoracic Surgery as a 372-page free-standing supplement

Both the EPCC and the International Congenital Heart Surgery Nomenclature and Database Project included a comprehensive Long List with thousands of terms and a Short List designed to be used as part of a minimum data set for multi-institutional registries and databases. Both Long Lists are mapped fully to their respective Short Lists.

The nearly simultaneous publication of these two complementary systems of nomenclature led to the problematic situation of having two competing systems of nomenclature, with the potential risk of duplicate or inaccurate coding within institutions secondary to confusion between the two systems. Therefore, on October 6, 2000, in Frankfurt, Germany, during the meeting of ECHSF prior to the 14th Annual Meeting of EACTS, representatives of the involved Societies met and established The International Nomenclature Committee for Paediatric and Congenital Heart Disease , which was to include representatives of the four societies (AEPC, STS, ECHSF, and EACTS), as well as representatives from the remaining continents of the world: Africa, Asia, Australia (Oceania), and South America. ^, ^, In January 2005, The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD) was constituted and legally incorporated in Canada.

Following the meeting in Frankfurt in 2000, a rule-based bidirectional crossmap between the two Short Lists was created and published by The International Working Group for Mapping and Coding of Nomenclatures for Pediatric and Congenital Heart Disease (also known as the Nomenclature Working Group [ NWG ]). ^, ^,

Over the next 8 years, the NWG met 10 times on all continents except Antarctica, over a combined period of 47 days, to achieve the main goal of mapping the two comprehensive Long Lists to each other to create the IPCCC, which is available for download from the Internet at [ https://www.IPCCC.net ]. The IPCCC has two primary versions :

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The version of the IPCCC derived from the EPCC of AEPC
•
The version of the IPCCC derived from the International Congenital Heart Surgery Nomenclature and Database Project of EACTS, ECHSA, and STS

These two versions of the IPCCC are crossmapped to each other and are also often referred to with the following abbreviated short names:

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EACTS-STS derived version of the IPCCC
•
AEPC derived version of the IPCCC

This common nomenclature, the IPCCC and the common minimum database data set created and published by The International Congenital Heart Surgery Nomenclature and Database Project , are now utilized by the overwhelming majority of multi-institutional databases of pediatric and congenital cardiac care throughout the world. The following databases all use the EACTS-STS derived version of the IPCCC:

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The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) ^, ^,
•
The European Congenital Heart Surgeons Association Congenital Heart Surgery Database (ECHSA CHSD) ^, ^,
•
The Japan Congenital Cardiovascular Surgery Database (JCCVSD) ^, ^,
•
The World Database for Pediatric and Congenital Heart Surgery (WDPCHS)

Both versions of the IPCCC Short Lists have been used to develop empirical systems for the adjustment of risk following surgical procedures, based on the operation type and comorbidities, for quality assurance and quality improvement purposes.

Principles of nomenclature

Clinical versus administrative nomenclature

Several studies have examined the relative utility of clinical and administrative nomenclature for the evaluation of quality of care for patients undergoing treatment for pediatric and congenital cardiac disease. The validity of coding of lesions seen in the congenitally malformed heart via the 9th Revision of the International Classification of Diseases (ICD-9) is poor, as evidenced by the following analyses ^, ^, ^, :

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In a series of 373 infants with congenital cardiac defects at Children’s Hospital of Wisconsin, only 52% of the cardiac diagnoses in the medical records had a corresponding code from the ICD-9 in the hospital discharge database.
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The Hennepin County Medical Center discharge database in Minnesota identified all infants born during 2001 with a code for congenital cardiac disease using ICD-9. A review of these 66 medical records by physicians was able to confirm only 41% of the codes that were contained in the ICD-9 based administrative database.
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The Metropolitan Atlanta Congenital Defect Program of the Birth Defect Branch of the Centers for Disease Control and Prevention of the United States government carried out surveillance of infants and fetuses with cardiac defects delivered to mothers residing in Atlanta during the years 1988 through 2003. These records were reviewed and classified using both administrative coding and the clinical nomenclature used in the STS CHSD. This study concluded that analyses based on the codes available in ICD-9 are likely to “have substantial misclassification” of congenital cardiac disease.
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A study using linked patient data (2004–2010) from the STS CHSD, a clinical registry, and the Pediatric Health Information Systems (PHIS) database, an administrative database, from hospitals participating in both, evaluated differential coding/classification of operations between datasets and subsequent impact on outcomes assessment. The cohort included 59,820 patients from 33 centers. There was a greater than 10% difference in the number of cases identified between data sources for half of the benchmark operations. The negative predictive value (NPV) of the administrative (versus clinical) data was high (98.8%–99.9%); the positive predictive value (PPV) was lower (56.7%–88.0%). These differences translated into significant differences in outcomes assessment, ranging from an underestimation of mortality associated with truncus arteriosus repair by 25.7% in the administrative versus clinical data (7.01% versus 9.43%; P =.001) to an overestimation of mortality associated with ventricular septal defect (VSD) repair by 31.0% (0.78% versus 0.60%; P =.1). This study demonstrated differences in case ascertainment between administrative and clinical registry data for children undergoing cardiac operations, which resulted in important differences in the assessment of outcomes.

These challenges and problems persist with the 10th Revision of the International Classification of Diseases (ICD-10). Several potential reasons can explain the poor diagnostic accuracy of administrative databases and codes from ICD-9 and even ICD-10:

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Accidental miscoding
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Coding performed by medical records clerks who have never seen the patient (i.e., coding performed by personnel not involved in the care of the patient)
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Contradictory or poorly described information in the medical record
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Lack of diagnostic specificity for congenital cardiac disease in ICD-9
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Inadequately trained medical coders

Although one might anticipate some improvement in diagnostic specificity with the adoption of ICD-10, it is still substantially deficient compared to that currently achieved with the clinical nomenclature used in clinical registries. (ICD-9 has only 29 congenital cardiac codes and ICD-10 has 73 possible congenital cardiac terms.) Only the implementation of the pediatric and congenital cardiac components of ICD-11 produces harmonization of the nomenclature used in clinical and administrative databases.

The meaning of the words right and left

When discussing cardiac chambers, such as atriums and ventricles, and spatial relationships, the words left and right can be confusing. Rules were therefore created to provide consistency and accuracy of descriptive terms of anatomical phenotypes. For cardiac chambers, unless otherwise stated, left refers to morphologically left, and right refers to morphologically right. Thus, left ventricle means the morphologically left ventricle, left atrium refers to the morphologically left atrium, and right atrial appendage refers to the morphologically right atrial appendage, and so on. When discussing cardiac chambers, the words left and right do not imply sidedness or position. If one wishes to describe the position or sidedness of a cardiac chamber, it is necessary to use terms such as left – sided ventricle . The term left ventricle , therefore, merely means the morphologically left ventricle and does not mean or imply left-sidedness or right-sidedness. Similarly, it does not imply connections to the right or left atrium, or the pulmonary or systemic circulations. In contrast, when describing the superior caval vein or inferior caval vein, and using the prefix left or right , it is the spatial position that is being alluded to, rather than any other connection or phenotypic variation that may exist.

Structural differences between the systems of nomenclature

The fundamental structure of systems for nomenclature are of two general types :

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Molecular structure, with an incrementally more complex diagnostic or procedural combination of terms. Each combination is considered a single diagnostic unit, which theoretically could have its own numerical code.
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Atomic structure, in which a complex diagnosis would have separate numerical codes for each element .

This variation in structure of the various systems of nomenclature means that a map between an atomic system and a molecular system would have a series of atomic codes being equivalent to one molecular code. Thus, the combination term from the molecular nomenclature “ TGA, VSD, LVOTO ” (transposition of the great arteries with VSD and left ventricular outflow tract obstruction) is equivalent to the three entries in an atomic system: “ Transposition of the great arteries (Discordant VA connections) ”(01.05.01), “ Ventricular Septal Defect ” (07.10.00), and “ Left ventricular outflow tract obstruction ” (07.09.01). Exceptions to this configuration are a few common combinations of lesions or procedures that are so routinely associated with each other that they have been grouped as one discrete diagnosis or procedure in both systems. Examples are “ Pulmonary atresia + Ventricular Septal Defect (including Fallot type)” (01.01.06) or “ Arterial and atrial switch procedures (double switch) ” (12.29.25).

In general, the EACTS-STS-derived version of the IPCCC uses a molecular structure, whereas the AEPC-derived version of the IPCCC uses an atomic structure. Meanwhile, IPCCC ICD-11 uses a combination of atomic and molecular approaches.

The international pediatric and congenital cardiac code (IPCCC)

As described earlier, IPCCC has two primary versions:

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EACTS-STS-derived version of the IPCCC
•
AEPC-derived version of the IPCCC

Both versions have a Short List and a Long List. The Long List of each version has been mapped to its corresponding Short List, and the two Long Lists have been crossmapped to each other. Both Long Lists also include hundreds of qualifiers and modifiers, some specific (such as anatomical sites) and some generic (such as gradings of severity).

The Short List and Long List of both versions of IPCCC are available for free download from the Internet at [ https://www.IPCCC.net ].

For all terms within the two versions of IPCCC , a unique six-digit code corresponds to a single entity, whether it be a morphologic phenotype, procedure, symptom, or genetic syndrome, with the mapped terms in each of the two versions being synonymous to each other (and sharing the same six digit code[s]). By 2013, there were 12,168 items in the AEPC-derived version of the IPCCC and 17,176 in the EACTS-STS-derived version of the IPCCC. The EACTS-STS-derived version of the IPCCC includes several thousand more terms than the AEPC-derived version of the IPCCC because of the differing structures of the systems. As discussed before, the molecular EACTS-STS-derived version of the IPCCC includes all of the atomic terms as well as thousands of derived molecular terms; meanwhile, the atomic AEPC-derived version of the IPCCC contains the atomic terms but very few molecular terms.

It is primarily the Short Lists, rather than the Long Lists, of the two crossmapped versions of the IPCCC that have been used for analyses of multi-institutional and international outcomes following operations and procedures for patients with congenitally malformed hearts, with over a million patients coded with the IPCCC in registries worldwide. Although both the Short Lists and the comprehensive Long Lists of each version of IPCCC have been crossmapped, the two Short Lists emanating from their respective Long List versions are not the same in terms of structure or content. ISNPCHD recognized this disparity and believed that the creation of a congenital cardiac subset within ICD-11 would accomplish several goals:

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Help resolve the differences between the Short List of the EACTS-STS-derived version of the IPCCC and the Short List of the AEPC-derived version of the IPCCC
•
Present a single common comprehensive and hierarchical Short List of diagnostic terms that could serve all communities involved with pediatric and congenital cardiac care
•
Harmonize the administrative nomenclature for pediatric and congenital cardiac care with the clinical nomenclature for pediatric and congenital cardiac care

Eleventh revision of the international classification of diseases (ICD-11)

The history of The International Classification of Diseases ( ICD ) dates back to the late 1800s ( Figure 27.1 ):

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In 1891, the International Statistical Institute commissioned a committee chaired by Jacques Bertillon (1851–1922), Chief of Statistical Services of the City of Paris, to create what became the Bertillon [International] Classification of Causes of Death, with associated sequential numeric codes. Over the following decades, this classification scheme was adopted by many countries in the Americas and Europe, with conferences for revision occurring roughly every 10 years to update the system, which became known as The International Classification of Diseases ( ICD ).
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In 1893, Bertillon presented the (International) Classification of Causes of Death at the meeting of the International Statistical Institute in Chicago, where it was adopted by several cities and countries.
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In 1898, the American Public Health Association recommended its adoption in North America and that the classification be revised every 10 years.
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In 1900, the First International Conference to revise the Bertillon Classification of Causes of Death was held in Paris.
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In 1909, non-fatal diseases (morbidity) were added.
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From 1948 until now, the World Health Organization (WHO ) has promoted and managed ICD, starting in 1948 with the sixth revision of the International Classification of Diseases, Injuries and Causes of Death.

A bar chart shows the interval in years between I C D revisions from 1900 to 2021 and the number of congenital heart disease terms per version. The chart plots intervals in years on the vertical axis from 0 to 30 and lists I C D versions on the horizontal axis from I C D 1 through I C D 1 with years beneath each version. Bars show intervals of 7 years for I C D1, 9 years for I C D 2, 10 years for I C D 3, 10 years for I C D 4, 9 years for I C D 5, 10 years for I C D6, 7 years for I C D 7, 10 years for I C D 8, 10 years for I C D 9, 14 years for I C D 10, and 32 years for I C D 11, with the last bar labeled 32 at the top. Two downward arrows highlight specific revisions, one labeled Non-fatal added above the I C D2 bar and one labeled W H O above the I C D6 bar. A lower strip labeled C H D lists term counts aligned under each version as 0, 1, 1, 1, 1, 8, 9, 21, 29, 73, and 367. The intervals appear similar in size through most early versions, then increase at I C D 10 and again at I C D 11. The C H D counts remain low in early versions and rise progressively, reaching the largest value at I C D 11. — • Figure 27.1

According to WHO , “ICD is the foundation for the identification of health trends and statistics globally, and the international standard for reporting diseases and health conditions. It is the diagnostic classification standard for all clinical and research purposes. ICD defines the universe of diseases, disorders, injuries and other related health conditions, listed in a comprehensive, hierarchical fashion” [ https://www.who.int/standards/classifications/classification-of-diseases ]. The ICD-11 development mission was “to produce an international disease classification that is ready for electronic health records that will serve as a standard for scientific comparability and communication.”

ICD-11 was officially launched online by the WHO in June 2018 and endorsed by the World Health Assembly on 25 May 2019. The WHO states that ICD-11 is to be “the global standard for health data, clinical documentation, and statistical aggregation,” that it is “scientifically up-to-date and designed for use in the digital world with state-of-the art technology to reduce the costs of training and implementation,” and that its “multilingual design facilitates global use” [ https://www.who.int/classifications/classification-of-diseases ].

The purpose of ICD-11 “is to allow the systematic recording, analysis, interpretation, and comparison of mortality and morbidity data collected in different countries or areas and at different times.”

The ICD-11 project began in earnest in 2007. Importantly, ICD-11 incorporates textual definitions. With the creation of ICD-11, for the first time, the revision process moved away from reliance on large meetings of national delegations of health statisticians, wherein those who voiced their opinion strongest would dominate the content of the paper-based output—a concept popularly known as “ decibel” diplomacy ”! In contrast, the ICD-11 revision process is dependent upon international expert clinicians, with:

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Digital curation
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Incorporation of wide peer review
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Εxtensive field testing

ICD-11 was adopted by the Seventy-second World Health Assembly in May 2019 and came into effect on January 1, 2022 [ https://www.who.int/standards/classifications/classification-of-diseases ].

The task of creating ICD-11 was divided into content specific Topic Advisory Groups (TAGs), with related Working Groups led by Managing Editors and chaired by specialist clinicians with an intentionally wide geographic spread. From 2009 through to 2016, the Managing Editor coordinated a series of meetings, some face-to-face but mostly teleconferences, beginning with the hierarchical structure and terms within ICD-10, and initially producing an evolving alpha draft. In 2012, a beta draft was published online [ https://icd.who.int/dev11/f/en ], coinciding with the authoring process moving to a Web-based platform for its entire content. The tool allows online global peer review and submission of comments by both the authors and worldwide interested parties in the field-testing stage. From the start, clinicians involved in the TAGs have been encouraged to enlist the advice of specialist societies to aid the process, and to ensure that the content was both up-to-date and had societal endorsement. This process has resulted in a huge increase in the number of individual terms within ICD-11, with secondary expanse of the hierarchical structure when compared with ICD-10.

In collaboration with WHO , ISNPCHD developed the pediatric and congenital cardiac nomenclature that is now within ICD-11. This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for pediatric and congenital cardiac care and the administrative nomenclature for pediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 CHD diagnostic terms and codes in ICD-9 and 73 CHD diagnostic terms in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10 and some of which provide greater granularity than the ISNPCHD thought was originally acceptable, such as individual codes for the various types of aortic arch branch isolation or having an aberrant origin. Thus, the total number of pediatric and congenital cardiac terms in ICD-11 is 367 ( E-Tables 27.1 , 27.2 , and 27.3 , available in online version).

E-TABLE 27.1

IPCCC ICD-11 Diagnostic Hierarchy ^,

ICD-11 New Row Number or Letter	ICD-11 Old Row Number or New Letter	IPCCC code	ICD-11 Level 0	ICD-11 Level I	ICD-11 Level II	ICD-11 Level III	ICD-11 Level IV	ICD-11 Level V	ICD-11 Level VI	ICD-11 Level VII
1	1	01.01.59	Structural developmental anomaly of heart or great vessels
2	2	03.01.13		Congenital anomaly of position or spatial relationships of thoraco-abdominal organs
3	3	02.01.09			Anomalous position-orientation of heart
4	4**	02.01.03				Laevocardia
5	5	02.01.02				Dextrocardia
6	6	02.01.04				Mesocardia
7	7	02.01.01				Extrathoracic heart
8	8**	01.03.00			Usual atrial arrangement
9	9	01.03.06			Abnormal atrial arrangement
10	10	01.03.01				Atrial situs inversus
11	11	01.03.02				Isomerism of right atrial appendages
12	12	01.03.03				Isomerism of left atrial appendages
13	13	02.04.12			Abnormal ventricular relationships
14	14**	02.03.01				Right hand pattern ventricular topology
15	15	02.03.02				Left hand pattern ventricular topology
16	16	02.03.03				Crisscross heart
17	17	02.04.00				Superior-inferior ventricular relationship
18	18	02.06.12			Abnormal relationship of great arterial roots
19	19	02.06.03				Aortic root directly anterior to pulmonary root
20	20	02.06.02				Aortic root anterior and rightward to pulmonary root
21	21	02.06.04				Aortic root anterior and leftward to pulmonary root
22	22	02.06.01				Aortic root side by side and directly rightward to pulmonary root
23	23	02.06.05				Aortic root side by side and directly leftward to pulmonary root
24	24	02.06.07				Aortic root directly posterior to pulmonary root
25	25**	02.06.00				Aortic root posterior and rightward to pulmonary root
26	26	02.06.06				Aortic root posterior and leftward to pulmonary root
27	27	02.07.03			Abnormal intrapericardial course of great arteries
28	28**	02.07.00				Spiraling course of great arteries
29	29	02.07.01				Parallel course of great arteries
30	30	03.01.02			Visceral heterotaxy
31	31	03.01.04				Right isomerism
32	32	03.01.05				Left Isomerism
33	33	03.01.03			Total mirror imagery
34	34	01.03.09		Congenital anomaly of an atrioventricular or ventriculo-arterial connection
35	35**	01.04.00			Concordant atrioventricular connections
36	36	01.04.01			Discordant atrioventricular connections
37	37	01.01.03				Congenitally corrected transposition of great arteries
38	38	01.05.01			Transposition of the great arteries
39	39	01.01.02				Transposition of the great arteries with concordant atrioventricular connections and intact ventricular septum
40	40	01.01.10				Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect
41	41	01.01.10 + 07.09.01				Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect and left ventricular outflow tract obstruction
42	42**	01.05.00			Concordant ventriculo-arterial connections
43	43	01.05.10				Concordant ventriculo-arterial connections with parallel great arteries
44	44	01.01.04			Double outlet right ventricle
45	45	01.01.17				Double outlet right ventricle with subaortic or doubly committed ventricular septal defect and pulmonary stenosis, Fallot type
46	46	01.01.17 + 07.13.04					Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis, Fallot type
47	47	01.01.17 + 07.13.02					Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis, Fallot type
48	48	01.01.18				Double outlet right ventricle with subpulmonary ventricular septal defect, transposition type
49	49	01.01.19				Double outlet right ventricle with non-committed ventricular septal defect
50	50	01.01.40				Double outlet right ventricle with subaortic or doubly committed ventricular septal defect without pulmonary stenosis, ventricular septal defect type
51	51	01.01.40 + 07.13.04					Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis
52	52	01.01.40 + 07.13.02					Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis
53	53	01.01.24				Double outlet right ventricle with intact ventricular septum
54	54	01.05.03			Double outlet left ventricle
55	55	09.01.01			Common arterial trunk
56	56	09.01.15				Common arterial trunk with aortic dominance
57	57	09.01.14					Common arterial trunk with aortic dominance and both pulmonary arteries arising from trunk
58	58	09.01.11					Common arterial trunk with aortic dominance and one pulmonary artery absent from the trunk, isolated pulmonary artery
59	59	09.01.12				Common arterial trunk with pulmonary dominance and aortic arch obstruction
60	60	09.01.18					Common arterial trunk with pulmonary dominance and interrupted aortic arch
61	61	09.01.19					Common arterial trunk with pulmonary dominance and aortic coarctation
62	A ⁺	09.02.10				Atypical truncal valve
63	62	09.02.19					Congenital truncal valvar regurgitation
64	63	09.02.18					Congenital truncal valvar stenosis
65	64	09.02.01					Dysplasia of truncal valve
66	65	04.00.07		Congenital anomaly of mediastinal vein
67	66	04.00.08			Congenital anomaly of mediastinal systemic vein
68	67	04.01.09				Congenital anomaly of superior caval vein
69	68	04.01.05					Absent right superior caval vein
70	69	04.01.25					Left superior caval vein
71	70	04.01.01						Left superior caval vein to coronary sinus
72	71	04.01.02						Left superior caval vein to left-sided atrium
73	B ⁺	04.01.07					Congenital stenosis of superior caval vein
74	72	04.03.08				Congenital anomaly of inferior caval vein
75	73	04.03.10					Interrupted inferior caval vein with absent suprarenal segment and azygos continuation
76	C ⁺	04.03.06					Congenital stenosis of inferior caval vein
77	74	04.04.05				Congenital anomaly of the coronary sinus
78	75	04.04.13					Unroofed coronary sinus
79	D ⁺	04.04.02						Completely unroofed coronary sinus
80	E ⁺	04.04.01						Partially unroofed coronary sinus
81	76	04.04.14					Coronary sinus orifice atresia or stenosis
82	77***	04.02.13				Anomalous hepatic venous connection to heart
83	78	04.08.04			Congenital anomaly of pulmonary vein
84	79	04.08.07				Anomalous pulmonary venous connection
85	80	04.08.05					Total anomalous pulmonary venous connection
86	81	04.06.00						Total anomalous pulmonary venous connection of the supracardiac type
87	82	04.08.10						Total anomalous pulmonary venous connection of the cardiac type
88	83	04.08.20						Total anomalous pulmonary venous connection of the infracardiac type
89	84	04.08.30						Total anomalous pulmonary venous connection of the mixed type
90	85	04.07.01					Partial anomalous pulmonary venous connection
91	86	01.01.16					Partial anomalous pulmonary venous connection of Scimitar type
92	87	03.02.23						Scimitar syndrome
93	88	04.08.06					Obstructed anomalous pulmonary venous pathway or connection
94	89	04.08.31				Congenital pulmonary venous stenosis or hypoplasia
95	90	04.08.02				Congenital atresia of pulmonary vein
96	91	05.00.02		Congenital anomaly of an atrium or atrial septum
97	92	05.07.01			Congenital anomaly of atrial septum
98	93	05.06.04				Restrictive interatrial communication or intact atrial septum when an interatrial shunt is physiologically necessary
99	94	05.03.03				Aneurysm of atrial septum
100	95	05.04.01				Interatrial communication
101	96**	05.03.01					Patent oval foramen
102	97	05.04.02					Atrial septal defect within oval fossa
103	98	05.05.00					Sinus venosus defect
104	99	05.06.02					Common atrium with separate atrioventricular junctions
105	100	05.05.03					Interatrial communication through coronary sinus orifice
106	101	05.01.13			Congenital anomaly of right atrium
107	102	05.01.21				Divided right atrium
108	F ⁺	05.01.04				Chiari network
109	103	05.01.06				Left-sided juxtaposition of the atrial appendages
110	104	05.01.12				Congenital giant right atrium
111	105	05.02.11			Congenital anomaly of left atrium
112	106	05.02.01				Divided left atrium
113	107	05.02.04				Right-sided juxtaposition of the atrial appendages
114	108	06.00.15		Congenital anomaly of an atrioventricular valve or atrioventricular septum
115	109	06.01.11			Congenital anomaly of tricuspid valve
116	110	06.01.25				Congenital tricuspid regurgitation
117	111	06.01.07				Congenital tricuspid valvar stenosis
118	112	06.01.04				Tricuspid anular hypoplasia
119	113	06.01.03				Dysplasia of tricuspid valve
120	114	06.01.09				Straddling tricuspid valve
121	115	06.01.05				Overriding tricuspid valve
122	116	06.01.34				Ebstein malformation of tricuspid valve
123	G ⁺	06.01.32				Absent tricuspid valve leaflet
124	H ⁺	06.01.36				True cleft of tricuspid valve leaflet
125	117	06.02.11			Congenital anomaly of mitral valve
126	118	06.02.25				Congenital mitral regurgitation
127	119	06.02.07				Congenital mitral valvar stenosis
128	120	06.02.04				Mitral anular hypoplasia
129	121	06.02.09				Straddling mitral valve
130	122	06.02.05				Overriding mitral valve
131	123	06.02.03				Dysplasia of mitral valve
132	124	05.02.02				Supravalvar or intravalvar mitral ring
133	I ⁺	06.02.23					Congenital intravalvar mitral ring
134	J ⁺	06.02.17					Congenital supravalvar mitral ring
135	125	06.02.72				Congenital mitral valvar prolapse
136	126	06.02.36				True cleft of anterior mitral leaflet
137	127	06.02.21				Congenital anomaly of mitral subvalvar apparatus
138	128	06.02.22					Congenital mitral subvalvar stenosis
139	129	06.02.56					Parachute malformation of mitral valve
140	K ⁺	06.02.39				Accessory tissue on mitral valve leaflet
141	L ⁺	06.02.32				Congenital unguarded mitral orifice
142	M ⁺	06.02.33				Double orifice of mitral valve
143	130	06.04.11			Congenital anomaly of left-sided atrioventricular valve in double inlet ventricle
144	131	06.03.11			Congenital anomaly of right-sided atrioventricular valve in double inlet ventricle
145	132	06.06.11			Common atrioventricular junction
146	133	06.06.00				Common atrioventricular junction with atrioventricular septal defect
147	134	06.07.27					Atrioventricular septal defect with balanced ventricles
148	135	06.07.26					Atrioventricular septal defect with ventricular imbalance
149	136	06.07.05						Atrioventricular septal defect with ventricular imbalance with dominant right ventricle and hypoplastic left ventricle
150	137	06.07.06						Atrioventricular septal defect with ventricular imbalance with dominant left ventricle and hypoplastic right ventricle
151	138	06.06.01					Atrioventricular septal defect with communication at the atrial level only
152	139	06.06.08					Atrioventricular septal defect with communication at the ventricular level only
153	140	06.06.10					Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level
154	141	06.06.09					Atrioventricular septal defect with communication at atrial level and unrestrictive communication at ventricular level
155	142	01.01.20					Atrioventricular septal defect and tetralogy of Fallot
156	143	05.06.03					Common atrium with common atrioventricular junction
157	144	06.05.60					Common atrioventricular valvar regurgitation
158	145	06.05.14					Atypical common atrioventricular valve
159	146	06.07.36						Common atrioventricular valve with unbalanced commitment of valve to ventricles
160	147	06.07.37							Common atrioventricular valve with unbalanced commitment of valve to right ventricle
161	148	06.07.38							Common atrioventricular valve with unbalanced commitment of valve to left ventricle
162	149	06.05.71						Atypical right ventricular component of common atrioventricular valve
163	150	06.05.72						Atypical left ventricular component of common atrioventricular valve
164	151	06.05.25							Double orifice of left ventricular component of common atrioventricular valve
165	152	06.05.98							Deficient mural leaflet of left ventricular component of common atrioventricular valve
166	153	06.07.28				Common atrioventricular junction without an atrioventricular septal defect
167	154	07.14.02			Communication between left ventricle and right atrium
168	155	07.00.00		Congenital anomaly of a ventricle or the ventricular septum
169	156	07.01.07			Congenital right ventricular anomaly
170	157	07.02.00				Right ventricular hypoplasia
171	158	07.05.20				Congenital right ventricular outflow tract obstruction
172	159	07.03.01				Double chambered right ventricle
173	160	07.01.13				Right ventricular myocardial sinusoids
174	161	07.01.06				Parchment right ventricle
175	162	01.01.01				Tetralogy of Fallot
176	163	09.05.25					Tetralogy of Fallot with absent pulmonary valve syndrome
177	164	01.01.26					Tetralogy of Fallot with pulmonary atresia
178	165	01.01.57					Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries
179	166	07.06.07			Congenital left ventricular anomaly
180	167	07.07.00				Left ventricular hypoplasia
181	168	07.06.19				Congenital left ventricular aneurysm or diverticulum
182	N ⁺	07.06.01					Congenital left ventricular aneurysm
183	O ⁺	07.06.03					Congenital left ventricular diverticulum
184	169	07.09.28				Congenital left ventricular outflow tract obstruction
185	170	07.09.08					Congenital left ventricular outflow tract obstruction due to atrioventricular valve
186	171	01.01.33				Left heart obstruction at multiple sites
187	172	07.06.12				Left ventricular myocardial sinusoids
188	P ⁺	07.00.07			Anomalous ventricular bands
189	173	07.20.04			Congenital anomaly of ventricular septum
190	174	07.14.07				Restrictive interventricular communication when an interventricular shunt is physiologically necessary
191	175	07.10.00				Ventricular septal defect
192	176	07.10.01					Perimembranous central ventricular septal defect
193	177	07.14.05					Inlet ventricular septal defect without a common atrioventricular junction
194	178	07.10.02						Inlet perimembranous ventricular septal defect without atrioventricular septal malalignment without a common atrioventricular junction
195	179	07.14.06						Inlet perimembranous ventricular septal defect with atrioventricular septal malalignment and without a common atrioventricular junction
196	180	07.11.02						Inlet muscular ventricular septal defect
197	181	07.11.01					Trabecular muscular ventricular septal defect
198	182	07.11.04						Trabecular muscular ventricular septal defect midseptal
199	183	07.11.03						Trabecular muscular ventricular septal defect apical
200	184	07.11.12						Trabecular muscular ventricular septal defect postero-inferior
201	185	07.11.07						Trabecular muscular ventricular septal defect anterosuperior
202	186	07.11.05						Multiple trabecular muscular ventricular septal defects
203	187	07.12.00					Outlet ventricular septal defect
204	188	07.12.09						Outlet ventricular septal defect without malalignment
205	189	07.11.06							Outlet muscular ventricular septal defect without malalignment
206	190	07.12.01							Doubly committed juxta-arterial ventricular septal defect without malalignment
207	191	07.12.02								Doubly committed juxta-arterial ventricular septal defect without malalignment and with muscular postero-inferior rim
208	192	07.12.03								Doubly committed juxta-arterial ventricular septal defect without malalignment and with perimembranous extension
209	193	07.10.17						Outlet ventricular septal defect with anteriorly malaligned outlet septum
210	194	07.11.15							Outlet muscular ventricular septal defect with anteriorly malaligned outlet septum
211	195	07.10.04							Outlet perimembranous ventricular septal defect with anteriorly malaligned outlet septum
212	196	07.12.12							Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum
213	197	07.12.07								Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and muscular postero-inferior rim
214	198	07.12.05								Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and perimembranous extension
215	199	07.10.18						Outlet ventricular septal defect with posteriorly malaligned outlet septum
216	200	07.11.16							Outlet muscular ventricular septal defect with posteriorly malaligned outlet septum
217	201	07.10.19							Outlet perimembranous ventricular septal defect with posteriorly malaligned outlet septum
218	202	07.12.13							Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum
219	203	07.12.08								Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and muscular postero-inferior rim
220	204	07.12.06								Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and perimembranous extension
221	205	07.15.01					Ventricular septal defect hemodynamically insignificant
222	206	07.15.04					Multiple ventricular septal defects
223	207	01.01.22		Functionally univentricular heart
224	208	01.01.14			Double inlet atrioventricular connection
225	209	01.04.04				Double inlet left ventricle
226	210	01.04.03				Double inlet right ventricle
227	211	01.04.05				Double inlet to solitary ventricle of indeterminate morphology
228	212	06.01.01			Tricuspid atresia
229	213	06.01.26				Tricuspid atresia with absent atrioventricular connection
230	214	06.01.02				Tricuspid atresia with imperforate tricuspid valve
231	215	06.02.01			Mitral atresia
232	216	06.02.26				Mitral atresia with absent atrioventricular connection
233	217	06.02.02				Mitral atresia with imperforate mitral valve
234	218	01.01.09			Hypoplastic left heart syndrome
235	219	09.04.29		Congenital anomaly of a ventriculo-arterial valve or adjacent regions
236	220	09.05.29			Congenital anomaly of pulmonary valve
237	221	09.05.04				Congenital pulmonary valvar stenosis
238	222	09.05.05				Pulmonary anular hypoplasia
239	223	09.05.22				Congenital pulmonary regurgitation
240	224	09.05.24				Dysplasia of pulmonary valve
241	225	09.05.32				Bicuspid pulmonary valve
242	226	07.05.32			Congenital subpulmonary stenosis
243	227	09.07.15			Congenital supravalvar pulmonary stenosis
244	228	09.05.16			Congenital pulmonary atresia
245	Q ⁺	09.05.12				Congenital pulmonary valvar atresia
246	229	01.01.07				Pulmonary atresia with intact ventricular septum
247	230	09.15.19			Congenital anomaly of aortic valve
248	231	09.15.01				Congenital aortic valvar stenosis
249	232	09.15.07				Congenital aortic regurgitation
250	233	09.15.22				Bicuspid aortic valve
251	234	09.15.21				Unicuspid aortic valve
252	235***	09.15.30				Aortic valvar prolapse
253	236	09.15.06				Aortic valvar atresia
254	237	09.15.17				Aortic anular hypoplasia
255	238	09.15.09				Dysplasia of aortic valve
256	239	07.09.50			Congenital subaortic stenosis
257	240	07.09.03				Subaortic stenosis due to fibromuscular shelf
258	241	07.09.16				Subaortic stenosis due to fibromuscular tunnel
259	242	09.16.18			Congenital supravalvar aortic stenosis
260	243	09.18.01			Aneurysm of aortic sinus of Valsalva
261	244	09.17.01			Aortoventricular tunnel
262	R ⁺	09.17.02				Aorto-left ventricular tunnel
263	S ⁺	09.17.04				Aorto-right ventricular tunnel
264	245	09.04.28		Congenital anomaly of great arteries including arterial duct
265	246	09.04.07			Congenital aortopulmonary window
266	247	09.07.16			Congenital anomaly of pulmonary arterial tree
267	248	09.10.36				Congenital dilation of pulmonary arterial tree
268	249	09.07.19				Congenital pulmonary trunk anomaly
269	250	09.07.20					Congenital pulmonary trunk hypoplasia
270	251	09.07.05					Absent or atretic pulmonary trunk
271	252	09.10.41				Congenital pulmonary arterial branch anomaly
272	253	09.10.27					Congenital pulmonary arterial branch stenosis
273	254	09.10.28						Congenital right pulmonary arterial stenosis
274	255	09.10.29						Congenital left pulmonary arterial stenosis
275	256	09.10.71					Congenital pulmonary arterial branch hypoplasia
276	257	09.10.72						Congenital right pulmonary arterial hypoplasia
277	258	09.10.73						Congenital left pulmonary arterial hypoplasia
278	T ⁺	09.10.21					Absent or atretic right or left pulmonary artery
279	259	09.10.75						Absent or atretic right pulmonary artery
280	260	09.10.77						Absent or atretic left pulmonary artery
281	261	09.10.37					Congenital central pulmonary arterial stenosis or hypoplasia proximal to hilar bifurcation
282	262	09.10.38					Congenital peripheral pulmonary arterial stenosis or hypoplasia at or beyond hilar bifurcation
283	263	09.10.30					Congenitally discontinuous, non-confluent right and left pulmonary arteries
284	264	09.09.08					Pulmonary artery origin from ascending aorta
285	265	09.09.03						Right pulmonary artery from ascending aorta
286	266	09.09.05						Left pulmonary artery from ascending aorta
287	267	09.09.11					Pulmonary artery from arterial duct
288	268	09.09.02						Right pulmonary artery from arterial duct
289	269	09.09.04						Left pulmonary artery from arterial duct
290	270	07.09.34			Congenital anomaly of aorta or its branches
291	271	09.16.06				Congenital anomaly of ascending aorta
292	272	09.16.02					Hypoplasia of ascending aortic
293	273	09.16.19					Congenital ascending aortic aneurysm or dilation
294	274	09.28.10				Congenital anomaly of aortic arch
295	275	09.29.11					Hypoplasia of aortic arch
296	277	09.29.31					Interrupted aortic arch
297	278	09.29.32						Interrupted aortic arch distal to subclavian artery, type A
298	279	09.29.33						Interrupted aortic arch between subclavian and common carotid arteries, type B
299	280	09.29.34						Interrupted aortic arch between carotid arteries, type C
300	281	09.28.15					Right aortic arch
301	282	09.28.22					Left aortic arch
302	283	09.28.06					Cervical aortic arch
303	X ⁺	09.30.22					Aortic diverticulum of Kommerell
304	Y ⁺	09.28.08					Persistent fifth aortic arch
305	276	09.29.01				Coarctation of aorta
306	U ⁺	09.29.02					Preductal coarctation of aorta
307	V ⁺	09.29.04					Postductal coarctation of aorta
308	W ⁺	09.29.03					Juxtaductal (paraductal) coarctation of aorta
309	284	09.30.17				Congenital anomaly of aortic arch branch
310	285	09.30.02					Aberrant origin of right subclavian artery
311	286	09.30.04					Aberrant origin of left subclavian artery
312	287	09.30.16					Isolation of an aortic arch branch
313	Z ⁺	09.30.11						Isolation of innominate artery
314	AA ⁺	09.30.14						Isolation of left subclavian artery
315	AB ⁺	09.30.15						Isolation of right subclavian artery
316	AC ⁺	09.30.12						Isolation of left common carotid artery
317	AD ⁺	09.30.13						Isolation of right common carotid artery
318	AE ⁺	09.30.34					Aberrant origin of innominate artery
319	AF ⁺	09.30.31					Common origin of the innominate artery and left common carotid artery
320	AG ⁺	09.30.28					Separate origins of internal and external carotid arteries
321	288	09.28.47				Congenital anomaly of descending thoracic or abdominal aorta
322	289	09.29.44					Descending thoracic or abdominal aortic coarctation
323	AH ⁺	09.29.05						Coarctation of the descending thoracic aorta
324	AI ⁺	09.29.06						Coarctation of the abdominal aorta
325	290	09.31.40			Tracheoesophageal compressive syndrome
326	291	09.30.23				Innominate artery compression syndrome
327	AJ ⁺	09.30.27					Retro-esophageal origin of aberrant innominate artery
328	292	09.31.00			Vascular Ring
329	293	09.28.09				Double aortic arch
330	294	09.31.35				Vascular ring of right aortic arch and left arterial duct or ligament
331	295	09.31.34				Vascular ring of left aortic arch and right arterial duct or ligament
332	296	09.09.06			Anomalous origin of left pulmonary artery from right pulmonary artery
333	297	09.27.05			Congenital arterial duct anomaly
334	298	09.27.21				Patent arterial duct
335	AK ⁺	09.27.03				Absent arterial duct
336	AL ⁺	09.27.04				Congenital aneurysm of arterial duct
337	AM ⁺	09.27.41				Anomalous origin of arterial duct
338	AN ⁺	09.27.82				Anomalous origin of arterial ligament
339	AO ⁺	14.10.51				Fetal arterial duct narrowing-closure
340	299	09.08.18			Systemic-to-pulmonary collateral arteries
341	300	09.46.03		Congenital anomaly of coronary artery
342	301	09.41.01			Anomalous origin of coronary artery from pulmonary arterial tree
343	302	09.41.03				Anomalous origin of left coronary artery from pulmonary artery
344	303	09.42.00			Anomalous aortic origin or course of coronary artery
345	304	09.42.21				Anomalous aortic origin of coronary artery with ventriculo-arterial concordance
346	305	09.46.26					Right coronary artery from left aortic sinus with ventriculo-arterial concordance
347	306	09.46.21					Left coronary artery from right aortic sinus with ventriculo-arterial concordance
348	307	09.43.04				Anterior descending from right coronary artery across right ventricular outflow tract
349	308	09.43.05				Intramural proximal coronary arterial course
350	309	09.43.13				Single coronary artery supplying all of heart
351	310	09.43.12			Myocardial bridging of coronary artery
352	311	09.44.05			Congenital coronary arterial orifice stenosis
353	312	09.44.19			Congenital coronary arterial orifice atresia
354	313	09.45.16			Congenital coronary arterial fistula
355	314	09.45.10				Congenital coronary arterial fistula to right ventricle
356	315	09.45.22				Congenital coronary arterial fistula to left ventricle
357	316	09.46.14			Congenital coronary arterial aneurysm
358	AP ⁺	09.42.09			Accessory coronary artery
359	AQ ⁺	09.46.44			Congenital absence of coronary artery
360	AR ⁺	09.46.19			Coronary arterial hypoplasia
361	317	10.01.05		Congenital pericardial anomaly
362	AS ⁺	10.01.02			Complete agenesis of pericardium
363	AT ⁺	10.01.01			Partial agenesis of pericardium
364	AU ⁺	10.01.03			Pleuropericardial cyst
365	AV ⁺	10.03.53		Congenital cardiac tumor
366	318***	09.19.05		Pulmonary arteriovenous fistula
367	AW ⁺	02.02.03		Bifid apex of heart

E-TABLE 27.2

IPCCC ICD-11 Definitions ^,

ICD-11 New Row Number or Letter	ICD-11 Old Row Number or New Letter	IPCCC code	ICD-11 Congenital Cardiac term	Definition	Commentary	Synonyms	Abbreviations
1	1	01.01.59	Structural developmental anomaly of heart or great vessels	A congenital malformation of the heart and/or great vessels or an acquired abnormality unique to the congenitally malformed heart.	This term should be selected only if a more specific term does not exist.	Congenital anomaly of heart and/or great vessels and related acquired abnormality; Congenital heart disease; Congenital malformation of heart
2	2	03.01.13	Congenital anomaly of position or spatial relationships of thoraco-abdominal organs	A congenital cardiovascular finding or malformation associated with an abnormal position of the heart or thoraco-abdominal organs, or an abnormal relative position of its component parts.
3	3	02.01.09	Anomalous position-orientation of heart	A congenital cardiovascular finding or malformation in which there is an abnormality of the position or orientation of heart.		Malposition of heart; Abnormal position of heart; Congenital abnormality of cardiac position; Congenital malposition of heart
4	4**	02.01.03	Laevocardia	A congenital cardiovascular finding in which the heart is predominantly to the left of the thoracic midline.	This is independent of the orientation of the cardiac apex. This is a normal finding and should be coded only in the context of complex heart disease.	Levocardia; Left-sided heart
5	5	02.01.02	Dextrocardia	A congenital cardiovascular malformation in which the heart is predominantly to the right of the thoracic midline.	This is independent of the orientation of the cardiac apex.	Right-sided heart; Heart in right chest; Congenital dextrocardia of heart
6	6	02.01.04	Mesocardia	A congenital cardiovascular malformation in which the heart is central or midline within the thorax.		Midline heart
7	7	02.01.01	Extrathoracic heart	A congenital cardiovascular malformation in which the heart is at least partially outside of the thorax.		Ectopia cordis
8	8**	01.03.00	Usual atrial arrangement	A congenital cardiac finding in which the atrial laterality (sidedness) is normal.	This is a normal finding and should be coded only in the context of complex congenital heart disease.	Atrial situs solitus
9	9	01.03.06	Abnormal atrial arrangement	A congenital cardiac malformation in which there is an abnormality of the laterality (or sidedness) of the atria.		Abnormal atrial situs
10	10	01.03.01	Atrial situs inversus	A congenital cardiac malformation in which the atrial morphologies and positions are the mirror image of normal.		Mirror-image atrial arrangement
11	11	01.03.02	Isomerism of right atrial appendages	A congenital cardiac malformation in which both atrial appendages have the morphology of a right atrial appendage.		Bilateral right atrial appendages, Isomeric right atrial appendages
12	12	01.03.03	Isomerism of left atrial appendages	A congenital cardiac malformation in which both atrial appendages have the morphology of a left atrial appendage.		Bilateral left atrial appendages, Isomeric left atrial appendages
13	13	02.04.12	Abnormal ventricular relationships	A congenital cardiac malformation in which the ventricular positions relative to each other or their laterality (sidedness) are abnormal.
14	14**	02.03.01	Right hand pattern ventricular topology	A congenital cardiac finding in which the chirality, or handedness, of the ventricles is normal, also known as D-loop.	Chirality or handedness with a right-handed right ventricle and means likening the morphologic right ventricle to a right hand by representing the inflow by the thumb, outflow by the index finger, and septum by the palm. This is a normal finding but should be coded in the presence of abnormal positions of the atria or great arteries where it represents an abnormality of the interrelationships of the ventricles relative to the remainder of the heart.	D-loop ventricles, Dextro-ventricular looping, D-bulboventricular loop
15	15	02.03.02	Left hand pattern ventricular topology	A congenital cardiac malformation in which the chirality, or handedness, of the ventricles is mirror image of normal, also known as L-loop.	Chirality or handedness with a left-handed right ventricle and means likening the morphologic right ventricle to a left hand by representing the inflow by the thumb, outflow by the index finger, and septum by the palm. An example is congenitally corrected transposition of great arteries.	L-loop ventricles, Levo-ventricular looping, Sinistro-ventricular looping, L-bulboventricular loop
16	16	02.03.03	Crisscross heart	A congenital cardiac malformation in which the atrioventricular inflow vectors are approximately orthogonal or perpendicular.		Twisted atrioventricular connections; Criss-cross heart
17	17	02.04.00	Superior-inferior ventricular relationship	A congenital cardiac malformation in which the ventricles are positioned superior-inferior to each other.	Excludes situations where one ventricle does not receive an atrioventricular valve (univentricular atrioventricular connection).	Upstairs-downstairs ventricular relationship, Supero-inferior heart
18	18	02.06.12	Abnormal relationship of great arterial roots	A congenital cardiovascular malformation in which the aortic root or its remnant is abnormally positioned relative to the pulmonary root or its remnant.		Abnormal relationships of great arteries, Abnormal relationships of great vessels
19	19	02.06.03	Aortic root directly anterior to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly anterior to the pulmonary root or its remnant.		Antero-posterior great arteries, Antero-posterior great vessels, A-malposed aorta, A-malposed great arteries, Aorta directly anterior to pulmonary artery
20	20	02.06.02	Aortic root anterior and rightward to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned anterior and to the right of the pulmonary root or its remnant.		D-malposed great arteries with anterior aorta, D-malposed great vessels with anterior aorta, Dextroposed great arteries with anterior aorta, Dextroposed great vessels with anterior aorta, D-transposed great arteries, D-transposed great vessels
21	21	02.06.04	Aortic root anterior and leftward to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned anterior and to the left of the pulmonary root or its remnant.		L-malposed great arteries with anterior aorta, L-malposed great vessels with anterior aorta, Levoposed great arteries with anterior aorta, Levoposed great vessels with anterior aorta, L-transposed great arteries with anterior aorta, L-transposed great vessels with anterior aorta
22	22	02.06.01	Aortic root side by side and directly rightward to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly to the right of the pulmonary root or its remnant.		D-malposed side-by-side great arteries, D-malposed side-by-side great vessels, Dextroposed side-by-side great arteries, Dextroposed side-by-side great vessels
23	23	02.06.05	Aortic root side by side and directly leftward to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly to the left of the pulmonary root or its remnant.		L-malposed side-by-side great arteries, L-malposed side-by-side great vessels, Levoposed side-by-side great arteries, Levoposed side-by-side great vessels
24	24	02.06.07	Aortic root directly posterior to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned directly posterior to the pulmonary root or its remnant.
25	25**	02.06.00	Aortic root posterior and rightward to pulmonary root	A congenital cardiovascular finding in which the aortic root or its remnant is positioned posterior and to the right of the pulmonary root or its remnant.	This is a normal finding but should only be coded in the context of complex congenital heart disease.	Segmental analysis D with posterior aorta, Normally related great arteries, Normally related great vessels, D-malposed great arteries with posterior aorta, D-malposed great vessels with posterior aorta
26	26	02.06.06	Aortic root posterior and leftward to pulmonary root	A congenital cardiovascular malformation in which the aortic root or its remnant is positioned posterior and to the left of the pulmonary root or its remnant.		L-malposed great arteries with posterior aorta, L-malposed great vessels with posterior aorta, Levoposed great arteries, Levoposed great vessels, Mirror imaged normally related great arteries, Mirror imaged normally related great vessels
27	27	02.07.03	Abnormal intrapericardial course of great arteries	A congenital cardiovascular malformation in which the course of the ascending aorta or its remnant is abnormal relative to the course of the pulmonary trunk or its remnant.	The normal course of the proximal great vessels results in a nearly orthogonal relationship of the axis of the aorta to the axis of the pulmonary artery.
28	28**	02.07.00	Spiraling course of great arteries	A congenital cardiovascular finding in which the course of the great arteries results in a nearly orthogonal relationship of the axis of the ascending aorta to the axis of the pulmonary trunk.	This is a normal finding but should be coded in the presence of abnormal positions of the great arteries relative to the ventricles.	Normal course of great arteries
29	29	02.07.01	Parallel course of great arteries	A congenital cardiovascular finding in which the course of the great arteries results in a nearly parallel relationship of the axis of the ascending aorta to the axis of the pulmonary trunk.
30	30	03.01.02	Visceral heterotaxy	A congenital malformation in which the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, in congenital cardiology, heterotaxy syndrome does not include patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as “total mirror imagery” or “situs inversus totalis.”		Situs ambiguus; Situs ambiguous; Heterotaxy; Heterotaxy syndrome, Heterotaxia; Heterotaxia syndrome; Splenic syndromes; Abnormal arrangement of thoraco-abdominal organs
31	31	03.01.04	Right isomerism	A congenital cardiovascular malformation that is a variant of heterotaxy syndrome in which some paired structures on opposite sides of the left-right axis of the body are symmetrical mirror images of each other and have the morphology of the normal right-sided structures.		Bilateral right-sidedness; Ivemark syndrome; Asplenia syndrome; Splenic agenesis syndrome
32	32	03.01.05	Left Isomerism	A congenital cardiovascular malformation that is a variant of an heterotaxy syndrome in which some paired structures on opposite sides of the left-right axis of the body are symmetrical mirror images of each other and have the morphology of the normal left-sided structures.		Bilateral left-sidedness; Polysplenia syndrome; Moller syndrome
33	33	03.01.03	Total mirror imagery	A congenital malformation in which there is complete mirror-imaged arrangement of the internal organs along the left-right axis of the body.		Situs inversus totalis; Situs inversus viscerum; Situs inversus
34	34	01.03.09	Congenital anomaly of an atrioventricular or ventriculo-arterial connection	A congenital cardiovascular malformation in which one or more of the following connections is abnormal: (1) the morphologically right atrium to the morphologically right ventricle, (2) the morphologically left atrium to the morphologically left ventricle, (3) the morphologically right ventricle to the pulmonary trunk, (4) the morphologically left ventricle to the aorta.	This excludes codes for hearts with a univentricular atrioventricular connection (mitral atresia, tricuspid atresia and double inlet ventricle), as these are listed under Functionally Univentricular Heart.
35	35**	01.04.00	Concordant atrioventricular connections	A congenital cardiovascular finding in which the morphologically right atrium connects to the morphologically right ventricle and the morphologically left atrium connects to the morphologically left ventricle.	This is a normal finding that should only be coded when associated with abnormal atrial arrangement and/or ventriculo-arterial connections.	Normal atrioventricular connections; Atrioventricular concordance
36	36	01.04.01	Discordant atrioventricular connections	A congenital cardiac malformation in which the morphologically right atrium connects to the morphologically left ventricle and the morphologically left atrium connects to the morphologically right ventricle.		Atrioventricular discordance
37	37	01.01.03	Congenitally corrected transposition of great arteries	A congenital cardiovascular malformation in which the morphologically right atrium connects to the morphologically left ventricle, the morphologically left atrium connects to the morphologically right ventricle, the morphologically right ventricle connects to the aorta, and the morphologically left ventricle connects to the pulmonary trunk.		Transposition {S,L,L}; Transposition {I,D,D}; Discordant atrioventricular & ventriculo-arterial connections; Corrected transposition of the great arteries; Corrected transposition of the great vessels; Congenitally corrected transposition of the great vessels; Double discordance; Physiologically corrected transposition of the great vessels; Physiologically corrected transposition of the great arteries	ccTGA
38	38	01.05.01	Transposition of the great arteries	A congenital cardiovascular malformation in which the morphologically right ventricle connects to the aorta and the morphologically left ventricle connects to the pulmonary trunk.		Discordant ventriculo-arterial connections; Transposition of the great vessels	TGA; TGV
39	39	01.01.02	Transposition of the great arteries with concordant atrioventricular connections and intact ventricular septum	A congenital cardiovascular malformation in which the morphologically right atrium connects to the morphologically right ventricle, the morphologically left atrium connects to the morphologically left ventricle, the morphologically right ventricle connects to the aorta, the morphologically left ventricle connects to the pulmonary trunk, and a ventricular septal defect is not present.		Transposition {S,D,D}; Transposition {I,L,L}; Concordant atrioventricular and discordant ventriculo-arterial connections with intact ventricular septum; Simple transposition	TGA & IVS; D-TGA
40	40	01.01.10	Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect	A congenital cardiovascular malformation in which the morphologically right atrium connects to the morphologically right ventricle, the morphologically left atrium connects to the morphologically left ventricle, the morphologically right ventricle connects to the aorta, the morphologically left ventricle connects to the pulmonary trunk, and one or more ventricular septal defects are present.		Concordant atrioventricular connections and discordant ventriculo-arterial connections with ventricular septal defect	TGA & VSD
41	41	01.01.10 + 07.09.01	Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect and left ventricular outflow tract obstruction	A congenital cardiovascular malformation in which the morphologically right atrium connects to the morphologically right ventricle, the morphologically left atrium connects to the morphologically left ventricle, the morphologically right ventricle connects to the aorta, the morphologically left ventricle connects to the pulmonary trunk, one or more ventricular septal defects are present, and left ventricular outflow tract obstruction is present.		Concordant atrioventricular connections and discordant ventriculo-arterial connections with ventricular septal defect and left ventricular outflow tract obstruction; Transposition with ventricular septal defect and pulmonary stenosis	TGA & VSD & LVOTO; TGA & VSD & PS
42	42**	01.05.00	Concordant ventriculo-arterial connections	A congenital cardiovascular finding in which the left ventricle is connected to the aorta or its remnant and the right ventricle is connected to the pulmonary trunk or its remnant.	This is a normal finding that should only be coded when associated with abnormal atrioventricular connections and/or ventriculo-arterial arrangement	Concordant ventriculo-arterial alignments
43	43	01.05.10	Concordant ventriculo-arterial connections with parallel great arteries	A congenital cardiovascular malformation in which the left ventricle is connected by way of an infundibulum to the aorta or its remnant, the right ventricle is connected to the pulmonary trunk or its remnant, and the proximal arteries have a parallel course.		Anatomically corrected malposition of the great arteries	ACM
44	44	01.01.04	Double outlet right ventricle	A congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle.			DORV
45	45	01.01.17	Double outlet right ventricle with subaortic or doubly committed ventricular septal defect and pulmonary stenosis, Fallot type	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a subaortic or doubly committed (with absence or deficiency of the conal septum) ventricular septal defect, and pulmonary outflow tract obstruction.		Double outlet right ventricle with subaortic or doubly committed interventricular communication & pulmonary stenosis (Fallot type)	DORV & PS
46	46	01.01.17 + 07.13.04	Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis, Fallot type	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a subaortic ventricular septal defect, and pulmonary outflow tract obstruction.		Double outlet right ventricle with subaortic interventricular communication & pulmonary stenosis (Fallot type)	DORV & PS
47	47	01.01.17 + 07.13.02	Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis, Fallot type	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a doubly committed ventricular septal defect with absence or deficiency of the conal septum, and pulmonary outflow tract obstruction.		Double outlet right ventricle with doubly committed interventricular communication & pulmonary stenosis (Fallot type)	DORV & PS
48	48	01.01.18	Double outlet right ventricle with subpulmonary ventricular septal defect, transposition type	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections that is associated with a subpulmonary ventricular septal defect (includes Taussig-Bing heart).		Double outlet right ventricle with subpulmonary interventricular communication (transposition type); Taussig-Bing syndrome; Taussig-Bing malformation; Taussig-Bing heart
49	49	01.01.19	Double outlet right ventricle with non-committed ventricular septal defect	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections that is associated with ventricular septal defect that is remote from the ventricular outflow tracts and usually within the inlet or muscular septum.		Double outlet right ventricle with remote ventricular septal defect; Double outlet right ventricle with uncommitted ventricular septal defect; Double outlet right ventricle with non-committed interventricular communication
50	50	01.01.40	Double outlet right ventricle with subaortic or doubly committed ventricular septal defect without pulmonary stenosis, ventricular septal defect type	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a subaortic or doubly committed (with absence or deficiency of the conal septum) ventricular septal defect, and unobstructed pulmonary outflow tract.		Double outlet right ventricle with subaortic or doubly committed interventricular communication without pulmonary stenosis (ventricular septal defect type)
51	51	01.01.40 + 07.13.04	Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a subaortic or doubly committed (with absence or deficiency of the conal septum) ventricular septal defect, and unobstructed pulmonary outflow tract.		Double outlet right ventricle with subaortic or doubly committed interventricular communication without pulmonary stenosis (ventricular septal defect type)
52	52	01.01.40 + 07.13.02	Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis	A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a doubly committed ventricular septal defect with absence or deficiency of the conal septum, and unobstructed pulmonary outflow tract.		Double outlet right ventricle with doubly committed interventricular communication without pulmonary stenosis (ventricular septal defect type)
53	53	01.01.24	Double outlet right ventricle with intact ventricular septum	A congenital cardiovascular malformation that is a variant of double outlet right ventricle that is associated with an intact ventricular septum.
54	54	01.05.03	Double outlet left ventricle	A congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically left ventricle.			DOLV
55	55	09.01.01	Common arterial trunk	A congenital cardiovascular malformation in which a single arterial trunk arises from the heart, giving origin sequentially to the coronary arteries, one or more pulmonary arteries, and the systemic arterial circulation.	This category includes Collett and Edwards truncus arteriosus types I, II, III and Van Praagh truncus arteriosus types 1, 2, 3, and 4. This category does not include Collett and Edwards truncus arteriosus type IV (which consists of pulmonary atresia with ventricular septal defect and absent intrapericardial pulmonary arteries) or “Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries.”	Truncus arteriosus communis; Persistent truncus arteriosus; Common truncus arteriosus; Truncus arteriosus	CAT, PTA, TAC, TA
56	56	09.01.15	Common arterial trunk with aortic dominance	A congenital cardiovascular malformation in which a common arterial trunk is associated with an unobstructed aortic arch.
57	57	09.01.14	Common arterial trunk with aortic dominance and both pulmonary arteries arising from trunk	A congenital cardiovascular malformation in which a common arterial trunk is associated with an unobstructed aortic arch and both pulmonary arteries arise from the common arterial trunk.	This category includes Collett and Edwards truncus arteriosus types I, II, III and Van Praagh truncus arteriosus types 1, 2.
58	58	09.01.11	Common arterial trunk with aortic dominance and one pulmonary artery absent from the trunk, isolated pulmonary artery	A congenital cardiovascular malformation in which a common arterial trunk is associated with an unobstructed aortic arch and one pulmonary artery does not arise from the common arterial trunk.		Truncus arteriosus with discontinuous pulmonary arteries; Common arterial trunk with discontinuous pulmonary arteries; Van Praagh truncus arteriosus type 3
59	59	09.01.12	Common arterial trunk with pulmonary dominance and aortic arch obstruction	A congenital cardiovascular malformation in which a common arterial trunk is associated with aortic arch obstruction or interruption.		Common arterial trunk with aortic arch obstruction; Truncus arteriosus with aortic arch obstruction; Van Praagh truncus arteriosus type 4
60	60	09.01.18	Common arterial trunk with pulmonary dominance and interrupted aortic arch	A congenital cardiovascular malformation in which a common arterial trunk is associated with an interrupted aortic arch.		Truncus arteriosus with interrupted aortic arch; Van Praagh truncus arteriosus type 4
61	61	09.01.19	Common arterial trunk with pulmonary dominance and aortic coarctation	A congenital cardiovascular malformation in which a common arterial trunk is associated with aortic coarctation.		Truncus arteriosus with coarctation of aorta; Van Praagh truncus arteriosus type 4
62	A ⁺	09.02.10	Atypical truncal valve	A congenital cardiovascular malformation in which the truncal valve does not have the usual morphologic or functional attributes at birth. Additional information: this may include truncal valves with more than trivial or mild dysplasia, stenosis, or regurgitation at birth, absent or perforated truncal valve leaflet(s), or a truncal valve that is made up of less than three or more than four leaflets.		Congenital abnormality of truncal valve
63	62	09.02.19	Congenital truncal valvar regurgitation	A congenital cardiovascular malformation in which there is backward flow through the truncal valve.		Congenital truncal valvar incompetence; Congenital truncal valvar insufficiency; Congenital truncal valve regurgitation; Congenital truncal valve insufficiency; Truncal insufficiency; Truncal regurgitation; Truncal incompetence
64	63	09.02.18	Congenital truncal valvar stenosis	A congenital cardiovascular malformation in which there is obstruction to flow through the truncal valve due to narrowing or stricture.		Congenital truncal valve stenosis
65	64	09.02.01	Dysplasia of truncal valve	A congenital cardiovascular malformation where the truncal valve leaflets are markedly thickened with restricted mobility, characterized by the presence of myxomatous tissue.		Congenital truncal valve dysplasia
66	65	04.00.07	Congenital anomaly of mediastinal vein	A congenital cardiovascular malformation in which there is an abnormality of a mediastinal vein including but not limited to pulmonary veins, caval veins, coronary sinus, coronary veins, hepatic veins connecting to the heart, brachiocephalic veins, azygos veins, and/or levoatrial cardinal veins.
67	66	04.00.08	Congenital anomaly of mediastinal systemic vein	A congenital cardiovascular malformation in which there is an abnormality of a mediastinal systemic vein including but not limited to caval veins, coronary sinus, coronary veins, hepatic veins connecting to the heart, brachiocephalic veins, and/or azygos veins.
68	67	04.01.09	Congenital anomaly of superior caval vein	A congenital cardiovascular malformation in which there is an abnormality of the superior caval vein (superior vena cava).		Congenital anomaly of superior vena cava	Congenital anomaly of SCV; Congenital anomaly of SVC
69	68	04.01.05	Absent right superior caval vein	A congenital cardiovascular malformation in which there is no right superior caval vein (superior vena cava) in the setting of the usual atrial arrangement (atrial situs solitus).		Absent right superior vena cava	Absent RSCV; Absent RSVC
70	69	04.01.25	Left superior caval vein	A congenital cardiovascular malformation in which there is a left superior caval vein (superior vena cava).	Unless the code for absent right superior caval vein is used, this term assumes that a right superior caval vein is present and, therefore, there are bilateral superior caval veins with or without a bridging vein.	Bilateral superior venae cavae; Bilateral superior caval veins; Persistent left superior caval vein; Persistent left superior vena cava	Persistent LSCV, Persistent LSVC, Bilateral SCVs, Bilateral SVCs
71	70	04.01.01	Left superior caval vein to coronary sinus	A congenital cardiovascular malformation in which there is a left superior caval vein (superior vena cava) entering a left-sided coronary sinus.	Unless the code for absent right superior caval vein is used, this term assumes that a right superior caval vein is present and, therefore, there are bilateral superior caval veins with or without a bridging vein.	Persistent left superior caval vein to coronary sinus; Persistent left superior vena cava to coronary sinus	LSCV to CS, LSVC to CS, PLSCV to CS; PLSVC to CS
72	71	04.01.02	Left superior caval vein to left-sided atrium	A congenital cardiovascular malformation in which there is a left superior caval vein (superior vena cava) connecting directly to the left-sided atrium.	Unless the code for absent right superior caval vein is used, this term assumes that a right superior caval vein is present and, therefore, there are bilateral superior caval veins with or without a bridging vein. This term should not be used in the presence of situs inversus of the atria where this is the expected arrangement.	Left superior caval vein persisting to left-sided atrium; Left superior vena cava to left-sided atrium	Persistent LSCV to LA; Persistent LSVC to LA
73	B ⁺	04.01.07	Congenital stenosis of superior caval vein	A congenital cardiovascular malformation in which there is narrowing or stricture of a superior caval vein (superior vena cava).
74	72	04.03.08	Congenital anomaly of inferior caval vein	A congenital cardiovascular malformation in which there is an abnormality of the inferior caval vein (inferior vena cava).		Congenital malformation of inferior vena cava; Congenital anomaly of the inferior vena cava
75	73	04.03.10	Interrupted inferior caval vein with absent suprarenal segment and azygos continuation	A congenital cardiovascular malformation in which there is an absence of the renal-to-hepatic segment of the inferior caval vein (inferior vena cava) with connection to a superior caval vein (superior vena cava) through the azygos venous system.		Interrupted inferior vena cava with absent suprarenal segment and azygos continuation
76	C ⁺	04.03.06	Congenital stenosis of inferior caval vein	A congenital cardiovascular malformation in which there is narrowing or stricture of the inferior caval vein (inferior vena cava).
77	74	04.04.05	Congenital anomaly of the coronary sinus	A congenital cardiovascular malformation in which there is an abnormality of the coronary sinus.
78	75	04.04.13	Unroofed coronary sinus	A congenital cardiac malformation in which there is direct communication between the left atrium and the coronary sinus.	This term includes partial, and complete unroofing or fenestration of the coronary sinus in the presence or absence of an interatrial communication. If an interatrial communication is present through the coronary sinus orifice, then also select the term “interatrial communication through coronary sinus orifice.” If a left superior caval vein (superior vena cava) is present, then one should also select the term for “left superior caval vein (superior vena cava) to left-sided atrium.”
79	D ⁺	04.04.02	Completely unroofed coronary sinus	A congenital cardiac malformation in which there is complete absence of the walls of the coronary sinus and left atrium that usually separate the lumen of the coronary sinus from the left atrial cavity.
80	E ⁺	04.04.01	Partially unroofed coronary sinus	A congenital cardiac malformation in which there is a communication between the lumen of the coronary sinus and the left atrial cavity.
81	76	04.04.14	Coronary sinus orifice atresia or stenosis	A congenital cardiac malformation in which the orifice of the coronary sinus is narrowed or absent.		Coronary sinus ostial atresia or stenosis
82	77***	04.02.13	Anomalous hepatic venous connection to heart	A congenital cardiovascular malformation in which a hepatic vein or hepatic veins do not connect to the inferior caval vein (inferior vena cava) but connect directly to the heart.	This term does not apply to interruption of the inferior caval vein (inferior vena cava) with a single connection of the hepatic veins to the heart. Coding note: This term in ICD-11 resides in the section entitled “Developmental anomalies of the liver” and is a child of “Congenital anomaly of the hepatic veins.”
83	78	04.08.04	Congenital anomaly of pulmonary vein	A congenital cardiovascular malformation in which there is an abnormality of the pulmonary veins.		Congenital malformation of pulmonary vein
84	79	04.08.07	Anomalous pulmonary venous connection	A congenital cardiovascular malformation in which one or more pulmonary vein(s) do(es) not connect normally to the morphologically left atrium.			APVC
85	80	04.08.05	Total anomalous pulmonary venous connection	A congenital cardiovascular malformation in which none of the pulmonary veins connect to the morphologically left atrium.		Totally anomalous pulmonary venous connection; Total anomalous pulmonary venous return	TAPVC, TAPVR, TAPVD
86	81	04.06.00	Total anomalous pulmonary venous connection of the supracardiac type	A congenital cardiovascular malformation with total anomalous pulmonary venous connection to the superior caval vein (superior vena cava) or one of its venous tributaries.		Total anomalous pulmonary venous connection Type 1	TAPVC Type I
87	82	04.08.10	Total anomalous pulmonary venous connection of the cardiac type	A congenital cardiovascular malformation with total anomalous pulmonary venous connection to the right atrium directly or to the coronary sinus or to both.		Total anomalous pulmonary venous connection Type 2; Total anomalous pulmonary venous connection, intracardiac	TAPVC Type 2
88	83	04.08.20	Total anomalous pulmonary venous connection of the infracardiac type	A congenital cardiovascular malformation with infradiaphragmatic total anomalous pulmonary venous connection.		Total anomalous pulmonary venous connection Type 3	TAPVC Type 3
89	84	04.08.30	Total anomalous pulmonary venous connection of the mixed type	A congenital cardiovascular malformation with total anomalous pulmonary venous connection at two or more levels (supracardiac, cardiac, or infracardiac).		Total anomalous pulmonary venous connection Type 4	TAPVC Type 4
90	85	04.07.01	Partial anomalous pulmonary venous connection	A congenital cardiovascular malformation in which one or more (but not all) of the pulmonary veins connect anomalously to the right atrium or to one or more of its venous tributaries and the remaining pulmonary veins connect to the left atrium.		Partially anomalous pulmonary venous connection; Partial anomalous pulmonary venous return	PAPVC. PAPVD, PAPVR
91	86	01.01.16	Partial anomalous pulmonary venous connection of Scimitar type	A congenital cardiovascular malformation with partial anomalous pulmonary venous connection in which some of the pulmonary veins (usually the right pulmonary veins) connect anomalously to the inferior caval vein (inferior vena cava) or to the right atrium at the insertion of the inferior vena cava.		Partial anomalous pulmonary venous return of Scimitar type
92	87	03.02.23	Scimitar syndrome	A congenital cardiopulmonary malformation with “partial anomalous pulmonary venous connection of Scimitar type” and one or more of the following: hypoplasia of the right lung with bronchial anomalies, dextrocardia, hypoplasia of the right pulmonary artery, lobar lung sequestration, and anomalous systemic arterial supply to the lower lobe of the right lung directly from the aorta or its main branches.		Pulmonary venolobar syndrome
93	88	04.08.06	Obstructed anomalous pulmonary venous pathway or connection	A congenital cardiovascular malformation in which the pathway of one or more anomalous pulmonary veins is blocked or impeded.		Obstructed anomalous pulmonary venous return
94	89	04.08.31	Congenital pulmonary venous stenosis or hypoplasia	A congenital cardiovascular malformation with a pathologic narrowing of one or more pulmonary veins including diffuse hypoplasia, long segment focal/tubular stenosis, and/or discrete stenosis.		Congenital pulmonary vein stenosis and/or hypoplasia
95	90	04.08.02	Congenital atresia of pulmonary vein	A congenital cardiovascular malformation with atresia of one or more pulmonary veins.		Congenital pulmonary vein atresia
96	91	05.00.02	Congenital anomaly of an atrium or atrial septum	A congenital cardiovascular malformation in which there is an abnormality of an atrium and/or atrial septum.		Congenital atrial malformation
97	92	05.07.01	Congenital anomaly of atrial septum	A congenital cardiac malformation in which there is an abnormality of the atrial septum.		Congenital malformation of atrial septum
98	93	05.06.04	Restrictive interatrial communication or intact atrial septum when an interatrial shunt is physiologically necessary	A cardiac finding in which there is either absence of an interatrial communication or the communication is smaller than is required to permit physiologically adequate interatrial flow.	This is always an abnormal finding prenatally but is a postnatal finding that should only be coded when present prenatally or when associated with other cardiac abnormalities that result in physiologic disturbances secondary to the absence of a larger interatrial communication (such as mitral or tricuspid valve atresia).	Restrictive interatrial communication in the presence of an obligatory interatrial shunt; Restrictive interatrial communication or intact atrial septum in the presence of an obligatory interatrial shunt
99	94	05.03.03	Aneurysm of the atrial septum	A congenital cardiac finding in which the septum primum is abnormally large (redundant) and results in aneurysmal protrusion into one or both atria.		Atrial septal aneurysm	ASA
100	95	05.04.01	Interatrial communication	A congenital cardiac malformation in which there is a hole or pathway between the atrial chambers.	*Although loosely often termed an “atrial septal defect,” not all interatrial communications have a defect in the true atrial septum, and so “atrial septal defect” in this context is not a true synonym	Atrial septal defect*; Congenital atrial septal defect; Auricular septal defect; Interatrial septal defect	ASD
101	96**	05.03.01	Patent oval foramen	A congenital cardiovascular finding in which there is a small interatrial communication (or potential communication) confined to the region of the oval fossa (fossa ovalis) characterized by no deficiency of the primary atrial septum (septum primum) and a normal limbus with no deficiency of the septum secundum (superior interatrial fold).		Patent foramen ovale; Open foramen ovale; Open oval foramen; Persistent foramen ovale	PFO
102	97	05.04.02	Atrial septal defect within oval fossa	A congenital cardiac malformation in which there is an interatrial communication confined to the region of the oval fossa (fossa ovalis), most commonly due to a deficiency of the primary atrial septum (septum primum) but deficiency of the septum secundum (superior interatrial fold) may also contribute.		Secundum atrial septal defect; Ostium secundum atrial septal defect	ASD II; OSASD
103	98	05.05.00	Sinus venosus defect	A congenital cardiovascular malformation in which there is a caval vein (vena cava) and/or pulmonary vein (or veins) that overrides the atrial septum or the septum secundum (superior interatrial fold) producing an interatrial or anomalous veno-atrial communication.	Although the term sinus venosus atrial septal defect is commonly used, the lesion is more properly termed a sinus venosus communication because, while it functions as an interatrial communication, this lesion is not a defect of the atrial septum.	Sinus venosus atrial septal defect; Sinus venosus communication
104	99	05.06.02	Common atrium with separate atrioventricular junctions	A congenital cardiovascular malformation in which there is complete or near-complete absence of the interatrial septum.		Single atrium; Atrium communis
105	100	05.05.03	Interatrial communication through coronary sinus orifice	A congenital cardiovascular malformation in which there is a communication between the left atrium and the coronary sinus allowing interatrial communication through the coronary sinus ostium.	“Interatrial communication through coronary sinus orifice” may or may not be associated with a persistent left superior caval vein (superior vena cava). This occurs in the absence of the coronary sinus (total unroofing of the coronary sinus) or partial unroofing of the coronary sinus.	Coronary sinus atrial septal defect
106	101	05.01.13	Congenital anomaly of right atrium	A congenital cardiac malformation in which there is an abnormality of the right atrium.
107	102	05.01.21	Divided right atrium	A congenital cardiac malformation in which the right atrium is partially divided by a large or obstructive Eustachian valve.		Cor triatriatum dexter; Cor triatriatum dextrum; Right cor triatriatum
108	F ⁺	05.01.04	Chiari network	A congenital cardiac finding in which there is a filamentous, web-like structure originating from the Eustachian valve near the orifice of the inferior caval vein (inferior vena cava) and connecting to variable parts of the right atrium.		Filigreed network of venous valves
109	103	05.01.06	Left-sided juxtaposition of the atrial appendages	A congenital cardiac malformation in which the right atrial appendage extends from the right atrium, behind the great arteries, to lie adjacent to the left atrial appendage.	This is frequently associated with horizontal orientation of the atrial septum, hypoplasia or atresia of the tricuspid valve, hypoplasia of the right ventricle, transposition of the great arteries or double outlet right ventricle, and subpulmonary or subaortic stenosis.
110	104	05.01.12	Congenital giant right atrium	A congenital cardiac malformation in which the right atrium is severely dilated. This is an isolated finding not secondary to abnormalities of the tricuspid valve or right ventricle.		Congenital right atrial aneurysm
111	105	05.02.11	Congenital anomaly of left atrium	A congenital cardiac malformation in which there is an abnormality of the left atrium.
112	106	05.02.01	Divided left atrium	A congenital cardiac malformation in which there is a partition that divides the left atrium into a posterior chamber that receives some or all of the pulmonary veins and an anterior chamber that communicates with the left atrial appendage and atrioventricular junction (usually the mitral valve).	In differentiating cor triatriatum from supravalvar mitral ring, in cor triatriatum the posterior compartment contains the pulmonary veins while the anterior contains the left atrial appendage and atrioventricular junction (usually the mitral valve); in supravalvar mitral ring, the anterior compartment contains only the mitral valve orifice.	Cor triatriatum sinister; Cor triatriatum sinistrum; Left cor triatriatum
113	107	05.02.04	Right-sided juxtaposition of the atrial appendages	A congenital cardiac malformation in which the left atrial appendage extends from the left atrium, behind the great arteries, to lie adjacent to the right atrial appendage.	This is commonly associated with hypoplasia or atresia of the mitral valve, hypoplasia of the left ventricle, normal (i.e., subpulmonary only) conus, and may be seen with atrioventricular septal defect (common atrioventricular canal) or subaortic or aortic stenosis or atresia.
114	108	06.00.15	Congenital anomaly of an atrioventricular valve or atrioventricular septum	A congenital cardiac malformation in which there is an abnormality of an atrioventricular valve or atrioventricular septum.
115	109	06.01.11	Congenital anomaly of tricuspid valve	A congenital cardiac malformation in which there is an abnormality of the tricuspid valve.	Tricuspid atresia is listed in the functionally univentricular heart section.	Congenital tricuspid valve anomaly
116	110	06.01.25	Congenital tricuspid regurgitation	A congenital cardiac finding in which there is backward flow through the tricuspid valve.		Congenital tricuspid insufficiency; Congenital tricuspid incompetence; congenital tricuspid valve regurgitation; Congenital tricuspid valve insufficiency; Congenital tricuspid valve incompetence
117	111	06.01.07	Congenital tricuspid valvar stenosis	A congenital cardiovascular malformation of the tricuspid valve in which there is narrowing or stricture (obstruction to flow)		Congenital tricuspid valve stenosis; Congenital stenosis of tricuspid valve; Congenital tricuspid stenosis
118	112	06.01.04	Tricuspid anular hypoplasia	A congenital cardiac malformation of the tricuspid valve in which there is anular hypoplasia (incomplete development or underdevelopment so that it is abnormally small [below the lower limit of normal adjusted for body size]). Hypoplasia may or may not be associated with stenosis.		Hypoplasia of tricuspid valvar anulus; Hypoplasia of the tricuspid anulus
119	113	06.01.03	Dysplasia of tricuspid valve	A congenital cardiac malformation of the tricuspid valve, commonly consisting of leaflet thickening and restricted mobility, with normally hinged leaflets.	This diagnosis is not used for patients with Ebstein malformation of tricuspid valve, which is characterized by abnormally hinged tricuspid valve.	Dysplasia of tricuspid valve; Tricuspid valvar dysplasia; Dysplastic tricuspid valve
120	114	06.01.09	Straddling tricuspid valve	A congenital cardiac malformation in which the tricuspid subvalvar apparatus has attachments within both ventricles.	This may or may not be associated with valvar overriding.
121	115	06.01.05	Overriding tricuspid valve	A congenital cardiac malformation in which the tricuspid valve anulus lies in part above both the right and left ventricles.
122	116	06.01.34	Ebstein malformation of tricuspid valve	A congenital cardiac malformation of the tricuspid valve and right ventricle that is characterized by incomplete delamination of the septal and inferior (posterior) tricuspid valvar leaflets from the myocardium of the right ventricle, and varying degrees of downward (apical) rotational displacement of the functional anulus.	Associated cardiac anomalies include an interatrial communication, the presence of accessory conduction pathways, and varying degrees of right ventricular outflow tract obstruction, including pulmonary atresia. In the setting of discordant atrioventricular and ventriculo-arterial connections [“Congenitally corrected transposition of the great arteries”], “Ebstein malformation of tricuspid valve” may be present.	Ebstein syndrome; Ebstein anomaly; Ebstein disease; Ebstein anomaly of tricuspid valve; Ebstein’s malformation of tricuspid valve; Ebstein anomaly or syndrome; Tricuspid valve, Ebstein anomaly; Congenital Ebstein deformity of tricuspid valve
123	G ⁺	06.01.32	Absent tricuspid valve leaflet	A congenital cardiac malformation in which a tricuspid valve leaflet is missing along with its corresponding subvalvar apparatus, thereby leaving a gap between the two other leaflets, particularly at the level of the atrioventricular junction.
124	H ⁺	06.01.36	True cleft of tricuspid valve leaflet	A congenital cardiac malformation in which a leaflet of the tricuspid valve is divided into two parts.
125	117	06.02.11	Congenital anomaly of mitral valve	A congenital cardiac malformation in which there is an abnormality of the mitral valve.	Mitral atresia is listed in the functionally univentricular heart section.	Congenital mitral valve anomaly
126	118	06.02.25	Congenital mitral regurgitation	A congenital cardiac finding in which there is backward flow through the mitral valve.		Congenital mitral insufficiency; Congenital mitral incompetence; Congenital mitral valve regurgitation; Congenital mitral valve insufficiency; Congenital mitral valve incompetence
127	119	06.02.07	Congenital mitral valvar stenosis	A congenital cardiac malformation of the mitral valve in which there is narrowing or stricture of the valvar orifice (obstruction to flow).		Duroziez disease; Congenital mitral stenosis; Congenital stenosis of mitral valve
128	120	06.02.04	Mitral anular hypoplasia	A congenital cardiac malformation of the mitral valve in which there is anular hypoplasia (incomplete development or underdevelopment so that it is abnormally small [below the lower limit of normal adjusted for body size]). Hypoplasia may or may not be associated with stenosis.		Hypoplasia of mitral valvar anulus; Hypoplasia of the mitral anulus
129	121	06.02.09	Straddling mitral valve	A congenital cardiac malformation in which the mitral subvalvar apparatus has attachments within both ventricles.	This may or may not be associated with valvar overriding.
130	122	06.02.05	Overriding mitral valve	A congenital cardiac malformation in which the mitral valve anulus lies in part above both the right and left ventricles.
131	123	06.02.03	Dysplasia of mitral valve	A congenital cardiac malformation that includes any structural abnormality of the mitral valvar leaflet(s), commonly consisting of leaflet thickening and restricted mobility.		Mitral valve dysplasia; Dysplastic mitral valve
132	124	05.02.02	Supravalvar or intravalvar mitral ring	A congenital cardiac malformation in which a ridge of tissue is immediately adjacent or integrally attached to the atrial side of the mitral valve.	The intravalvar variant is distinguished from the supravalvar variant because the intravalvar ring is within the funnel of the mitral valvar leaflets. Both the intravalvar variant and the supravalvar variant are differentiated from divided left atrium because in the latter the posterosuperior compartment contains the pulmonary veins while the antero-inferior contains the left atrial appendage (and the atrioventricular valve [usually mitral valve] in the absence of left atrioventricular valvar atresia); in supravalvar or intravalvar mitral ring, the antero-inferior compartment contains only the mitral valve orifice and not the left atrial appendage.	Supravalvar mitral membrane; Membranous supravalvular mitral stenosis; Congenital supravalvar mitral ring; supravalvar mitral stenosis; Supravalvular congenital mitral stenosis
133	I ⁺	06.02.23	Congenital intravalvar mitral ring	A congenital cardiac malformation in which there is a fibrous shelf-like ridge of tissue adherent to the atrial surface of the mitral valve.
134	J ⁺	06.02.17	Congenital supravalvar mitral ring	A congenital cardiac malformation in which there is a fibrous shelf-like ridge of tissue located between the mitral valve and the opening to the left atrial appendage.
135	125	06.02.72	Congenital mitral valvar prolapse	A congenital cardiac malformation of the mitral valve in which one or both leaflets move to the atrial side of the plane of the anulus in systole.		Congenital mitral valve prolapse	Congenital MVP
136	126	06.02.36	True cleft of anterior mitral leaflet	A congenital cardiac malformation of the mitral valve in which the anterior leaflet is divided into two parts.	This term should not be used for the so-called “mitral cleft” of a common atrioventricular valve, as this malformation in a patient with a common atrioventricular valve is actually the zone of apposition between the superior and inferior bridging leaflets, and not a divided valve leaflet.	Isolated cleft of the anterior mitral leaflet (without common atrioventricular junction); True cleft of anterior mitral leaflet (without common atrioventricular junction)	Cleft MV, MV cleft
137	127	06.02.21	Congenital anomaly of mitral subvalvar apparatus	A congenital cardiac malformation in which the mitral chords, chordal attachments, or papillary muscles are abnormal.
138	128	06.02.22	Congenital mitral subvalvar stenosis	A congenital cardiac malformation in which there is stenosis (narrowing or stricture of a duct or a canal) of the subvalvar components (chordae tendineae and/or papillary muscles) of the mitral valve. This diagnosis includes mitral stenosis associated with parachute mitral valve, mitral arcade, and hammock mitral valve.		Subvalvar mitral stenosis
139	129	06.02.56	Parachute malformation of mitral valve	A congenital cardiac malformation in which the chords of the mitral valve attach to a single or to closely adjacent papillary muscles.		Parachute mitral valve
140	K ⁺	06.02.39	Accessory tissue on mitral valve leaflet	A congenital cardiac malformation in which there is extraneous leaflet material forming a string, sheet, or pedunculated mass attached to the atrial or ventricular surface of the mitral valve.
141	L ⁺	06.02.32	Congenital unguarded mitral orifice	A congenital cardiac malformation of the mitral valve in which there is a patent valve orifice with complete absence of both leaflets.
142	M ⁺	06.02.33	Double orifice of mitral valve	A congenital cardiac malformation of the mitral valve in which there are two separate flow orifices through the mitral valve into the left ventricle.	To differentiate the congenital term “Double orifice of mitral valve” from a mitral valve with a fenestrated or perforated mitral valve leaflet, this term should only be used when both orifices are supported by tensor apparatuses.
143	130	06.04.11	Congenital anomaly of left-sided atrioventricular valve in double inlet ventricle	A congenital cardiac malformation in association with double inlet ventricle (excluding common atrioventricular valve) in which the atrioventricular valve morphology cannot be determined. This term should be used for the left-sided atrioventricular valve in those hearts.		Left atrioventricular valve stenosis or regurgitation
144	131	06.03.11	Congenital anomaly of right-sided atrioventricular valve in double inlet ventricle	A congenital cardiac malformation in association with double inlet ventricle (excluding common atrioventricular valve) in which the atrioventricular valve morphology cannot be determined. This term should be used for the right-sided atrioventricular valve in those hearts.
145	132	06.06.11	Common atrioventricular junction	A congenital cardiac malformation where both atria connect to a common atrioventricular valve which characteristically has 4 or 5 leaflets including superior and inferior bridging leaflets with a single anulus. The common valve may have one or two major orifices depending on the absence or presence of fusion of the bridging leaflets to each other or the septal crest.	The left ventricular zone of apposition between the superior and inferior bridging leaflets is commonly referred to as a “cleft.”	Common atrioventricular canal
146	133	06.06.00	Common atrioventricular junction with atrioventricular septal defect	A congenital cardiac malformation where both the right atrium and left atrium connect to a common atrioventricular valve, and there is a hole in the atrioventricular septum that produces a communication between the left- and right-sided cardiac chambers.	The common atrioventricular valve characteristically has 4 or 5 leaflets including superior and inferior bridging leaflets with a single anulus. The common valve may have one or two major orifices depending on the absence or presence of fusion of the bridging leaflets to each other or to the septal crest.	Atrioventricular septal defect; Atrioventricular canal; Atrioventricular canal defect	AVSD, AVC, AVC defect
147	134	06.07.27	Atrioventricular septal defect with balanced ventricles	A congenital cardiac malformation that is a variant of atrioventricular septal defect (atrioventricular canal defect) with ventricles that are equal or nearly equal in size.		Balanced atrioventricular canal
148	135	06.07.26	Atrioventricular septal defect with ventricular imbalance	A congenital cardiac malformation that is a variant of atrioventricular septal defect (atrioventricular canal defect) with one ventricle significantly larger than the other.	Unbalanced ventricular size and unbalanced relation of the common atrioventricular valve to the ventricles are to be distinguished by coding unbalanced ventricular size as “Atrioventricular septal defect with ventricular imbalance” and the unbalanced relation of the common atrioventricular valve to the ventricles should also be coded as “Common atrioventricular valve with unbalanced commitment of valve to ventricles.”	Unbalanced atrioventricular canal
149	136	06.07.05	Atrioventricular septal defect with ventricular imbalance with dominant right ventricle and hypoplastic left ventricle	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with the right ventricle significantly larger than the left.		Right dominant AV canal/AVSD
150	137	06.07.06	Atrioventricular septal defect with ventricular imbalance with dominant left ventricle and hypoplastic right ventricle	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with the left ventricle significantly larger than the right.		Left dominant AV canal/AVSD
151	138	06.06.01	Atrioventricular septal defect with communication at the atrial level only	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with an interatrial communication just above the atrioventricular valve, no interventricular communication just below the atrioventricular valve, separate right and left atrioventricular valvar orifices, and varying degrees of malformation of the left-sided component of the common atrioventricular valve. The bridging leaflets of the common atrioventricular valve are bound down to the crest of the scooped-out ventricular septum so that the potential for shunting through the atrioventricular septal defect is possible only at the atrial level and not at the ventricular level.		Incomplete atrioventricular septal defect with isolated atrial component; Incomplete atrioventricular canal defect with isolated atrial component; Ostium primum atrial septal defect; Atrioventricular defect with atrial shunting only; Primum atrial septal defect; Partial atrioventricular canal defect with isolated atrial component; Partial atrioventricular septal defect: ostium primum type; Partial atrioventricular septal defect	PAVSD, PAVC
152	139	06.06.08	Atrioventricular septal defect with communication at the ventricular level only	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with an interventricular communication just below the atrioventricular valve, no interatrial communication just above the atrioventricular valve, separate right and left atrioventricular valvar orifices, and varying degrees of malformation of the left-sided component of the common atrioventricular valve. The bridging leaflets of the common atrioventricular valve are bound to the atrial septum so that the potential for shunting through the atrioventricular septal defect is possible only at the ventricular level and not at the atrial level.		AV canal/AVSD with isolated VSD; Atrioventricular canal defect with isolated ventricular communication
153	140	06.06.10	Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with an interatrial communication immediately above the atrioventricular valve, and a restrictive interventricular communication immediately below the atrioventricular valve.	This term is used to identify hearts with a restrictive interventricular communication. If there is a single atrioventricular valvar orifice this term should still be used. If the ventricular component of the atrioventricular septal defect is unrestrictive (no interventricular pressure gradient), this malformation should not be coded as “Atrioventricular septal defect (atrioventricular canal defect) with communication at atrial level and restrictive communication at ventricular level” and instead the term “Atrioventricular septal defect (atrioventricular canal defect) with communication at atrial level and unrestrictive communication at ventricular level” should be used.	Intermediate atrioventricular septal defect with atrial and ventricular components and separate atrioventricular valvar orifices; Atrioventricular canal defect associated with a restrictive ventricular septal defect; Atrioventricular septal defect with atrial shunting and restrictive ventricular shunting; Intermediate atrioventricular canal defect with atrial and ventricular components and separate atrioventricular valve; Transitional atrioventricular canal defect with atrial and ventricular components and separate atrioventricular valve; Transitional atrioventricular septal defect with atrial and ventricular components and separate atrioventricular valves
154	141	06.06.09	Atrioventricular septal defect with communication at atrial level and unrestrictive communication at ventricular level	A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal defect) with an interatrial communication just above the atrioventricular valve, an interventricular communication just below the atrioventricular valve, and varying degrees of malformation of the left ventricular component of the common atrioventricular valve. There is unrestrictive interventricular communication (no interventricular pressure gradient) and the bridging leaflets usually float to varying extent within the atrioventricular septal defect.		Complete atrioventricular septal defect; Complete atrioventricular canal defect	CAVSD, CAVC
155	142	01.01.20	Atrioventricular septal defect and tetralogy of Fallot	A congenital cardiac malformation with both an atrioventricular septal defect (atrioventricular canal defect) and tetralogy of Fallot.	Tetralogy of Fallot with atrioventricular septal defect (common atrioventricular canal) is always the complete form (unrestrictive interventricular component) and usually has four leaflets of the common atrioventricular valve (undivided superior leaflet) and very few or no attachments of that leaflet to the crest of the ventricular septum.	Atrioventricular septal defect associated with tetralogy of Fallot; Atrioventricular canal and tetralogy of Fallot	TOF/AVC, TOF/AVSD
156	143	05.06.03	Common atrium with common atrioventricular junction	A congenital cardiac malformation in which there is complete absence of the interatrial septum in the setting of a common atrioventricular junction (common atrioventricular canal).		Single atrium
157	144	06.05.60	Common atrioventricular valvar regurgitation	A congenital cardiac finding where there is backward flow through the common atrioventricular valve.
158	145	06.05.14	Atypical common atrioventricular valve	A congenital cardiac malformation in which the common atrioventricular valve does not have the usual morphology of 4-5 non-dysplastic leaflets that relate relatively equally to both ventricles.
159	146	06.07.36	Common atrioventricular valve with unbalanced commitment of valve to ventricles	A congenital cardiac malformation in which the common atrioventricular valve is primarily related to one ventricle, usually but not always associated with hypoplasia of the other ventricle.	Unbalanced ventricular size and unbalanced relation of the common atrioventricular valve to the ventricles are to be distinguished by coding unbalanced ventricular size as “Atrioventricular septal defect with ventricular imbalance” and the unbalanced relation of the common atrioventricular valve to the ventricles should also be coded as “Common atrioventricular valve with unbalanced commitment of valve to ventricles.”
160	147	06.07.37	Common atrioventricular valve with unbalanced commitment of valve to right ventricle	A congenital cardiac malformation in which the common atrioventricular valve is primarily related to the right ventricle, usually but not always associated with hypoplasia of the left ventricle.
161	148	06.07.38	Common atrioventricular valve with unbalanced commitment of valve to left ventricle	A congenital cardiac malformation in which the common atrioventricular valve is primarily related to the left ventricle, usually but not always associated with hypoplasia of the right ventricle.
162	149	06.05.71	Atypical right ventricular component of common atrioventricular valve	A congenital cardiac malformation in which the right ventricular component of the common atrioventricular valve does not have the usual structure (independently supported and well developed right ventricular components of the common atrioventricular valve).
163	150	06.05.72	Atypical left ventricular component of common atrioventricular valve	A congenital cardiac malformation in which the left ventricular component of the common atrioventricular valve does not have the usual structure (independently supported and well developed left ventricular components of the common atrioventricular valve).
164	151	06.05.25	Double orifice of left ventricular component of common atrioventricular valve	A congenital cardiac malformation in which the left ventricular component of the common atrioventricular valve has two orifices.
165	152	06.05.98	Deficient mural leaflet of left ventricular component of common atrioventricular valve	A congenital cardiac malformation in which the left ventricular component of the common atrioventricular valve is composed primarily of the bridging leaflets due to hypoplasia or absence of the mural (lateral) leaflet.		Deficient lateral leaflet of left ventricular component of common atrioventricular valve
166	153	06.07.28	Common atrioventricular junction without an atrioventricular septal defect	A congenital cardiac malformation in which there is a common atrioventricular junction without any communication at the level of the atrioventricular septum.	This code could be used when there is a trifoliate left atrioventricular valve, commonly known as cleft mitral valve secondary to atrioventricular septal defect, and also has evidence of spontaneous obliteration of the atrioventricular septal defect by valvar or subvalvar tissue. Additional defects in the atrial or ventricular septums that do not involve the atrioventricular septum are not excluded by this term and should be coded separately.	Common atrioventricular junction with spontaneous fibrous closure of atrioventricular septal defect; Common atrioventricular junction with intact atrioventricular septal structures
167	154	07.14.02	Communication between left ventricle and right atrium	A deficiency of the atrioventricular component of the membranous septum permitting shunting of blood from the left ventricle to the right atrium.	This is an isolated absence of the atrioventricular septum without other components that may be commonly seen in association with spectrum of disorders associated with atrioventricular septal defects such as a common atrioventricular valve or interatrial or interventricular communications. The Gerbode defect may be confused with ventricular septal defects where a deficiency of tricuspid valvar tissue permits ventriculo-atrial shunting subsequent to initial interventricular shunting (left ventricle to right ventricle to right atrium).	Gerbode defect
168	155	07.00.00	Congenital anomaly of a ventricle or the ventricular septum	A congenital cardiac malformation in which there is an abnormality of a ventricle and/or the ventricular septum. The ventricles include the ventricular inlet, ventricular body, and ventricular outflow tract.		Congenital ventricular or ventricular septal malformation
169	156	07.01.07	Congenital right ventricular anomaly	A congenital cardiac malformation in which there is an abnormality of the right ventricle.
170	157	07.02.00	Right ventricular hypoplasia	A congenital cardiac malformation in which the right ventricle is abnormally small (below the lower limit of normal adjusted for body size).	This morphologic abnormality usually is an integral part of other congenital cardiovascular anomalies and, therefore, frequently does not need to be coded separately. It should, however, be coded as secondary to an accompanying congenital cardiovascular anomaly if the right ventricular hypoplasia is not considered an integral and understood part of the primary congenital cardiovascular diagnosis. It would rarely be coded as a primary and/or isolated diagnosis.	Hypoplastic right ventricle
171	158	07.05.20	Congenital right ventricular outflow tract obstruction	A congenital cardiac condition in which the flow through the right ventricular outflow tract (proximal to the valve[s] guarding the outflow from the right ventricle) is blocked or impeded.			RVOTO
172	159	07.03.01	Double chambered right ventricle	A congenital cardiac malformation in which the right ventricle is divided into two chambers, one inferior including the inlet and trabecular portions of the right ventricle and one superior including the trabecular portion and infundibulum.	Double chamber right ventricle is often associated with one or several closing ventricular septal defects. In some cases, the ventricular septal defect is already closed. Double chamber right ventricle is differentiated from the rare isolated infundibular stenosis that develops more superiorly.	Double-chamber right ventricle; Anomalous right ventricular muscle bundle; Double chamber right ventricle	DCRV
173	160	07.01.13	Right ventricular myocardial sinusoids	A congenital cardiac malformation in which there are deep, endothelial lined, blind-ended intramyocardial tunnels communicating with the right ventricular chamber. These occur in the setting of pulmonary atresia or severe right ventricular outflow tract obstruction, intact ventricular septum, and a patent tricuspid valve.	These RV sinusoids do not communicate with the coronary arteries. Where connections from the right ventricle to the coronary arteries exist, one should code for “Congenital coronary artery-to-right ventricular fistula.”
174	161	07.01.06	Parchment right ventricle	A congenital cardiac malformation in which there is almost complete absence of right ventricular myocardium and preserved septal and left ventricular myocardium.	Parchment right ventricle can occur in association with a normal tricuspid valve (Uhl anomaly) or with an abnormal tricuspid valve, such as Ebstein Anomaly. This should be distinguished from arrhythmogenic right ventricular cardiomyopathy by the presence of fatty infiltration in the latter.	Uhl’s anomaly; Uhl anomaly
175	162	01.01.01	Tetralogy of Fallot	A group of congenital cardiac malformations with biventricular atrioventricular alignments or connections characterized by anterosuperior deviation of the conal or outlet septum or its fibrous remnant, narrowing or atresia of the pulmonary outflow, a ventricular septal defect of the malalignment type, and biventricular origin of the aorta. Tetralogy of Fallot will always have a ventricular septal defect, narrowing or atresia of the pulmonary outflow, aortic override, and most often right ventricular hypertrophy.		Tetrad of Fallot; Fallot tetralogy; Fallot tetrad; Fallot disease; Fallot complex; Fallot’s tetralogy; Subpulmonic stenosis, ventricular septal defect, overriding aorta, and right ventricular hypertrophy; Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta, and hypertrophy of right ventricle	TOF
176	163	09.05.25	Tetralogy of Fallot with absent pulmonary valve syndrome	A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with absent or rudimentary leaflets (cusps) that do not coapt. In its usual form there is dilatation of the pulmonary trunk and central right and left pulmonary arteries, which when extreme, is associated with abnormal arborization of lobar and segmental pulmonary artery branches and with compression of the trachea and mainstem bronchi, often with tracheobronchomalacia.	A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with absent or rudimentary leaflets (cusps) that do not coapt. In its usual form there is dilatation of the pulmonary trunk and central right and left pulmonary arteries, which when extreme, is associated with abnormal arborization of lobar and segmental pulmonary artery branches and with compression of the trachea and mainstem bronchi, often with tracheobronchomalacia. The physiologic consequence is usually a combination of variable degrees of both stenosis and regurgitation of the pulmonary valve.
177	164	01.01.26	Tetralogy of Fallot with pulmonary atresia	A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree.	This term should not be used when major systemic-to-pulmonary artery collaterals (including major aortopulmonary collateral arteries [MAPCAs]) are known to be present.	Pulmonary atresia with ventricular septal defect [Fallot type]
178	165	01.01.57	Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries	A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree and there are collateral blood vessels between the systemic and pulmonary arteries.		Pulmonary atresia with ventricular septal defect and systemic-to-pulmonary collateral artery(ies) [Fallot type]; Pulmonary atresia, ventricular septal defect, and MAPCAs
179	166	07.06.07	Congenital left ventricular anomaly	A congenital cardiac malformation in which there is an abnormality of the left ventricle.
180	167	07.07.00	Left ventricular hypoplasia	A congenital cardiac malformation in which the left ventricle is abnormally small (below the lower limit of normal adjusted for body size).	This morphologic abnormality usually is an integral part of other congenital cardiovascular anomalies and does not need to be coded separately if this is the case. It should be coded as secondary to an accompanying congenital cardiovascular anomaly if the left ventricular hypoplasia is not considered an integral and understood part of the primary congenital cardiovascular diagnosis such as hypoplastic left heart syndrome.	Hypoplastic left ventricle
181	168	07.06.19	Congenital left ventricular aneurysm or diverticulum	A congenital cardiac malformation in which there is an outpouching of the left ventricular wall.
182	N ⁺	07.06.01	Congenital left ventricular aneurysm	A congenital cardiac malformation in which there is a discrete region of the ventricular wall that is thinner than the adjacent myocardial segments, bulges away from the lumen of the left ventricle during systole, and exhibits either akinesis or dyskinesis.
183	O ⁺	07.06.03	Congenital left ventricular diverticulum	A congenital cardiac malformation in which there is an outpouching from the ventricular wall that contains endocardium, myocardium, and pericardium and displays normal contraction.
184	169	07.09.28	Congenital left ventricular outflow tract obstruction	A congenital cardiac condition in which the flow through the left ventricular outflow tract (proximal to the valve[s] guarding the outflow from the left ventricle) is blocked or impeded.	This code should not be used for obstruction immediately under the arterial valve such as subaortic stenosis due to fibromuscular shelf or tunnel.		LVOTO
185	170	07.09.08	Congenital left ventricular outflow tract obstruction due to atrioventricular valve	A congenital cardiac malformation in which the flow through the left ventricular outflow tract is blocked or impeded due to abnormal position or attachments of tricuspid, mitral, or common atrioventricular valvar tissue.
186	171	01.01.33	Left heart obstruction at multiple sites	A congenital cardiovascular malformation in which more than one of the following lesions are present: (1) supravalvar or intravalvar mitral ring, (2) mitral subvalvar stenosis, (3) a parachute deformity of the mitral valve, (4) subaortic stenosis, (5) valvar aortic stenosis, and (6) aortic coarctation.	Shone’s syndrome consists of multilevel hypoplasia or obstruction of the left-heart. The syndrome is based on the original report from Shone that was based on analysis of 8 autopsied cases and described the tendency of these four obstructive, or potentially obstructive, conditions to coexist. Only 2 of the 8 cases exhibited all four conditions, with the other cases exhibiting only two or three of the anomalies.	Shone syndrome; Shone’s syndrome; Shone’s anomaly; Shone’s complex; Shone’s disease
187	172	07.06.12	Left ventricular myocardial sinusoids	A congenital cardiac malformation in which there are deep, endothelial lined, blind-ended intramyocardial tunnels communicating with the left ventricular chamber. These occur in the setting of aortic atresia or severe left ventricular outflow tract obstruction, intact ventricular septum and a patent mitral valve.	These left ventricular sinusoids do not communicate with the coronary arteries. Where connections from the left ventricle to the coronary arteries exist, one should code for “Congenital coronary artery-to-left ventricular fistula.” This code should not be used for left ventricular non-compaction.
188	P ⁺	07.00.07	Anomalous ventricular bands	A congenital cardiac malformation in which fibromuscular tissue crosses through a ventricular cavity, inserting at two or more points on the ventricular wall or papillary muscles.
189	173	07.20.04	Congenital anomaly of ventricular septum	A congenital cardiac malformation in which there is an abnormality of the interventricular septum.		Anomaly; ventricular septum; Ventricle septa anomaly; Ventricle septal heart anomaly; Ventricle septum heart anomaly; Ventricular septa anomaly; Ventricular septal heart anomaly
190	174	07.14.07	Restrictive interventricular communication when an interventricular shunt is physiologically necessary	A congenital cardiac finding in which there is an interventricular communication that is smaller than is required to permit physiologically adequate interventricular flow.	This should be coded only when associated with other cardiac abnormalities that result in physiologic disturbances secondary to the absence of a larger interventricular communication. Clinically important examples include double outlet right ventricle or tetralogy of Fallot with restrictive ventricular septal defect, double inlet left ventricle or tricuspid atresia with concordant or discordant great arterial connections and restrictive ventricular septal defect, and others.
191	175	07.10.00	Ventricular septal defect	A congenital cardiac malformation in which there is a hole or pathway between the ventricular chambers.	The definitions offered for a “ventricular septal defect,” in its various forms, will be used most frequently in the setting of patients who do not have abnormalities of either the atrioventricular or ventriculo-arterial connections. The definitions themselves, however, are equally applicable for the description and categorization of holes or pathways between the ventricles when the segmental connections between the cardiac components are abnormal. The key to understanding the definitions is to appreciate that the hole or pathway between the ventricles is defined both on the basis of its geographic location within the ventricular septum and its margins as seen from the aspect of the morphologically right ventricle. In this respect, when the ventriculo-arterial connections are concordant or discordant, the roof of the channel, as viewed from the right ventricle, is the muscular outlet septum or its fibrous remnant. When both arterial trunks arise from the morphologically right ventricle, in contrast, the channel between the ventricles provides the outflow from the morphologically left ventricle, and is roofed by the inner heart curvature. In the setting of double outlet right ventricle, therefore, the interventricular communication is not the same geometric locus as the ventricular septal defect, although many still continue to describe the outlet from the left ventricle as the “ventricular septal defect.” In univentricular atrioventricular connections with functionally single left ventricle with an outflow chamber, the communication, which used to be called a bulboventricular foramen, is both a ventricular septal defect and an interventricular communication. The situation in the setting of double outlet right ventricle, however, shows that the terms cannot always be used as being synonymous. A similar situation pertains in the setting of common arterial trunk, since the hole usually closed by the surgeon to provide septal integrity is the right ventricular margin of the channel between the ventricles, rather than the geometric interventricular communication.	Interventricular communication; Interventricular septal defect; Congenital ventricular septal defect	VSD
192	176	07.10.01	Perimembranous central ventricular septal defect	A congenital cardiovascular malformation in which there is a ventricular septal defect that (1) occupies the space that is usually closed by the interventricular part of the membranous septum, (2) is adjacent to the area of fibrous continuity between the leaflets of an atrioventricular valve and an arterial valve, (3) is adjacent to an area of mitral-tricuspid fibrous continuity, and (4) is located at the center of the base of the ventricular mass.	Although best used to describe the perimembranous defect that opens centrally at the base of the right ventricle, this term might be used to code perimembranous defects with inlet or outlet extension. It is recommended, however, that the more precise terms be used whenever possible for coding the latter lesions. This code is used by some as synonymous with the perimembranous, conoventricular, Type II, or the paramembranous defects. It should not be used to code an inlet VSD, or the so-called atrioventricular canal VSD. More specific terms exist for coding these entities. It is used by some to describe an isolated perimembranous VSD without extension, although it is unlikely that perimembranous defects exist in the absence of deficiency of their muscular perimeter. The conoventricular VSD with malalignment should be coded as an outlet defect, as should the perimembranous defect opening to the outlet of the right ventricle. All perimembranous defects, nonetheless, have part of their margins made up of fibrous continuity either between the leaflets of an atrioventricular and an arterial valve or, in the setting of double outlet right ventricle or overriding of the tricuspid valve, by fibrous continuity between the leaflets of the mitral and tricuspid valves. Such defects can also extend to become doubly committed and juxta-arterial (conal septal hypoplasia) when there is also fibrous continuity between the leaflets of the arterial valves or when there is a common arterial valve. Specific codes exist for these variants, which ideally should not be coded using this term.	Perimembranous ventricular septal defect; Membranous ventricular septal defect; Paramembranous ventricular septal defect; Type 2 ventricular septal defect; Central perimembranous ventricular septal defect	PMVSD
193	177	07.14.05	Inlet ventricular septal defect without a common atrioventricular junction	A congenital cardiac malformation in which there is a ventricular septal defect that opens predominantly into the inlet component of the right ventricle in the absence of a common atrioventricular junction.		Type 3 ventricular septal defect; Inlet atrioventricular canal type ventricular septal defect; Inlet ventricular septal defect
194	178	07.10.02	Inlet perimembranous ventricular septal defect without atrioventricular septal malalignment without a common atrioventricular junction	A congenital cardiac malformation in which there is a ventricular septal defect that usually, but not always, extends beneath the full anular length of the septal leaflet of the tricuspid valve and to the membranous septum, into the inlet component of the right ventricle in the absence of atrioventricular septal malalignment and of a common atrioventricular junction.		Perimembranous ventricular septal defect with extension to right ventricular inlet (posterior); Inlet perimembranous ventricular septal defect; Perimembranous inlet ventricular septal defect
195	179	07.14.06	Inlet perimembranous ventricular septal defect with atrioventricular septal malalignment and without a common atrioventricular junction	A congenital cardiac malformation in which there is an inlet ventricular septal defect with malalignment between the atrial septum and the postero-inferior part of the muscular ventricular septum, in the absence of a common atrioventricular junction, such that there is overriding with or without straddling of the tricuspid valve.		Perimembranous ventricular septal defect with extension to right ventricular inlet (posterior) and atrioventricular septal malalignment; Inlet perimembranous ventricular septal defect with atrioventricular septal malalignment; Perimembranous inlet ventricular septal defect with atrioventricular septal malalignment
196	180	07.11.02	Inlet muscular ventricular septal defect	A congenital cardiac malformation in which there is a ventricular septal defect with exclusively muscular borders that opens into the inlet component of the right ventricle.		Muscular ventricular septal defect opening into right ventricular inlet; Inlet muscular ventricular septal defect
197	181	07.11.01	Trabecular muscular ventricular septal defect	A congenital cardiac malformation in which there is a ventricular septal defect within the trabeculated component of the ventricular septum.	The code specifying defects within the trabecular part of the ventricular septum should not be used to code the inlet or outlet muscular defects as there are specific codes for these.	Type 4 ventricular septal defect; Trabecular ventricular septal defect; Muscular trabecular ventricular septal defect
198	182	07.11.04	Trabecular muscular ventricular septal defect midseptal	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the right ventricle in the middle of the trabeculated component of the ventricular septum.		Midmuscular ventricular septal defect; Trabecular muscular ventricular septal defect: mid; Muscular trabecular ventricular septal defect: midseptal
199	183	07.11.03	Trabecular muscular ventricular septal defect apical	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the right ventricle in the apical region of the trabeculated component of the ventricular septum.		Apical muscular ventricular septal defect; Muscular trabecular ventricular septal defect: apical
200	184	07.11.12	Trabecular muscular ventricular septal defect postero-inferior	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the right ventricle in the postero-inferior region of the trabeculated component of the ventricular septum.	It can be arbitrary as to when an inlet muscular defect becomes an inferior apical defect. The distinction should be made on whether or not the defect is partly shielded by the septal leaflet of the tricuspid valve.	Postero-inferior muscular ventricular septal defect; Posterior muscular ventricular septal defect; Inferior muscular ventricular septal defect; Muscular ventricular septal defect in postero-inferior trabecular septum; Muscular trabecular ventricular septal defect: postero-inferior
201	185	07.11.07	Trabecular muscular ventricular septal defect anterosuperior	A congenital cardiac malformation in which there is a muscular ventricular septal defect that opens to the right ventricle anterior to the body of the septomarginal trabeculation, or septal band.		Anterosuperior muscular ventricular septal defect; Anterior muscular ventricular septal defect; Superior muscular ventricular septal defect; Muscular ventricular septal defect opening anterior to body of septomarginal trabeculation; Muscular trabecular ventricular septal defect: anterosuperior
202	186	07.11.05	Multiple trabecular muscular ventricular septal defects	A congenital cardiac malformation in which there are multiple muscular ventricular septal defects that open to the right ventricle in the trabeculated component of the ventricular septum.		Multiple muscular trabecular ventricular septal defects; Muscular trabecular ventricular septal defect: multiple (“Swiss cheese” septum)
203	187	07.12.00	Outlet ventricular septal defect	A congenital cardiac malformation in which there is a ventricular septal defect that opens to the outlet of the right ventricle between or above the limbs of the septal band.		Supracristal ventricular septal defect; Conal septal defect; Infundibular ventricular septal defect; Type 1 ventricular septal defect; Subpulmonary ventricular septal defect; Subarterial ventricular septal defect; Juxtaarterial ventricular septal defect; Conal ventricular septal defect; Conoseptal hypoplasia; Intraconal ventricular septal defect
204	188	07.12.09	Outlet ventricular septal defect without malalignment	A congenital cardiac malformation in which there is an outlet ventricular septal defect, in the absence of malalignment of the outlet septum with the trabecular muscular septum.
205	189	07.11.06	Outlet muscular ventricular septal defect without malalignment	A congenital cardiac malformation in which there is an outlet ventricular septal defect with exclusively muscular borders, in the absence of malalignment of the outlet septum with the trabecular muscular septum.		Muscular outlet ventricular septal defect with aligned outlet septum; Conal septal hypoplasia with exclusively muscular borders; Muscular outlet ventricular septal defect
206	190	07.12.01	Doubly committed juxta-arterial ventricular septal defect without malalignment	A congenital cardiac malformation in which there is an outlet ventricular septal defect bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, that is aligned with the trabecular muscular septum.		Doubly committed subarterial ventricular septal defect with aligned outlet septum; Conoseptal hypoplasia with aligned outlet septum; Doubly committed juxta-arterial outlet ventricular septal defect; Doubly committed subarterial outlet ventricular septal defect
207	191	07.12.02	Doubly committed juxta-arterial ventricular septal defect without malalignment and with muscular postero-inferior rim	A congenital cardiac malformation in which there is an outlet ventricular septal defect with muscular postero-inferior rim, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, that is aligned with the trabecular muscular septum.		Doubly committed juxta-arterial ventricular septal defect with muscular postero-inferior rim; Doubly committed subarterial ventricular septal defect with muscular postero-inferior rim
208	192	07.12.03	Doubly committed juxta-arterial ventricular septal defect without malalignment and with perimembranous extension	A congenital cardiac malformation in which there is an outlet ventricular septal defect with perimembranous extension, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, that is aligned with the trabecular muscular septum.		Doubly committed subarterial ventricular septal defect with aligned outlet septum with perimembranous extension; Conal septal hypoplasia without cranial muscular border with aligned outlet septum and with perimembranous extension; Doubly committed juxta-arterial outlet ventricular septal defect with perimembranous extension; Doubly committed juxta-arterial ventricular septal defect with without malaligned fibrous outlet septum and fibrous postero-inferior rim (perimembranous)
209	193	07.10.17	Outlet ventricular septal defect with anteriorly malaligned outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect and the muscular outlet septum is malaligned in an antero-cranial fashion with respect to the trabecular muscular septum such that there is overriding of the arterial valve supported predominantly by the left ventricle.		Ventricular septal defect opening into right ventricular outlet with anterior malalignment of outlet septum; Outlet ventricular septal defect “Fallot type”
210	194	07.11.15	Outlet muscular ventricular septal defect with anteriorly malaligned outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect with exclusively muscular borders, and the muscular outlet septum is malaligned in an antero-cranial fashion with respect to the trabecular muscular septum such that there is overriding of the arterial valve supported predominantly by the left ventricle.		Muscular ventricular septal defect opening into right ventricular outlet with anterior malalignment of outlet septum; Muscular outlet ventricular septal defect “Fallot type”; Muscular outlet ventricular septal defect with anteriorly malaligned outlet septum
211	195	07.10.04	Outlet perimembranous ventricular septal defect with anteriorly malaligned outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect with perimembranous extension and the muscular outlet septum is malaligned in an antero-cranial fashion with respect to the trabecular muscular septum such that there is overriding of the arterial valve supported predominantly by the left ventricle.		Perimembranous ventricular septal defect with outlet extension and anterior malalignment of outlet septum; Perimembranous outlet ventricular septal defect “Fallot type”; Perimembranous outlet ventricular septal defect with anteriorly malaligned outlet septum
212	196	07.12.12	Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, and the fibrous outlet septum is malaligned in an antero-cranial fashion with respect to the trabecular muscular septum such that there is overriding of the arterial valve supported predominantly by the left ventricle.		Doubly committed subarterial ventricular septal defect and anterior malalignment of the outlet septum; Doubly committed juxta-arterial ventricular septal defect “Fallot type”
213	197	07.12.07	Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and muscular postero-inferior rim	A congenital cardiac malformation in which there is an outlet ventricular septal defect with muscular postero-inferior rim, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, and the fibrous outlet septum is malaligned in an antero-cranial fashion with respect to the trabecular muscular septum such that there is overriding of the arterial valve supported predominantly by the left ventricle.		Doubly committed subarterial ventricular septal defect with muscular posterior inferior rim and anterior malalignment of outlet septum; Doubly committed juxta-arterial ventricular septal defect with muscular posterior inferior rim “Fallot type”
214	198	07.12.05	Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and perimembranous extension	A congenital cardiac malformation in which there is an outlet ventricular septal defect with perimembranous extension, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, and the fibrous outlet septum is malaligned in an antero-cranial fashion with respect to the trabecular muscular septum such that there is overriding of the arterial valve supported predominantly by the left ventricle.		Doubly committed subarterial ventricular septal defect with perimembranous extension and anterior malalignment of outlet septum; Doubly committed juxta-arterial ventricular septal defect with perimembranous extension “coarctation type”; Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and fibrous postero-inferior rim (perimembranous)
215	199	07.10.18	Outlet ventricular septal defect with posteriorly malaligned outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect and the muscular outlet septum is malaligned in a postero-caudal fashion with respect to the trabecular muscular septum such that there is usually obstruction to the left ventricular outflow tract.		Ventricular septal defect opening into right ventricular outlet with posterior malalignment of outlet septum; Outlet ventricular septal defect “coarctation type”
216	200	07.11.16	Outlet muscular ventricular septal defect with posteriorly malaligned outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect with exclusively muscular borders, and the muscular outlet septum is malaligned in a postero-caudal fashion with respect to the trabecular muscular septum such that there is usually obstruction to the left ventricular outflow tract.		Muscular ventricular septal defect opening into right ventricular outlet with posterior malalignment of outlet septum; Muscular outlet ventricular septal defect “coarctation type”; Muscular outlet ventricular septal defect with posteriorly malaligned outlet septum
217	201	07.10.19	Outlet perimembranous ventricular septal defect with posteriorly malaligned outlet septum
218	202	07.12.13	Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum	A congenital cardiac malformation in which there is an outlet ventricular septal defect bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, and the fibrous outlet septum is malaligned in a postero-caudal fashion with respect to the trabecular muscular septum such that there is usually obstruction to the left ventricular outflow tract.		Doubly committed subarterial ventricular septal defect with posterior malalignment of outlet septum; Doubly committed juxta-arterial ventricular septal defect “coarctation type”
219	203	07.12.08	Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and muscular postero-inferior rim	A congenital cardiac malformation in which there is an outlet ventricular septal defect with muscular postero-inferior rim, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, and the fibrous outlet septum is malaligned in a postero-caudal fashion with respect to the trabecular muscular septum such that there is usually obstruction to the left ventricular outflow tract.		Doubly committed subarterial ventricular septal defect with muscular posterior inferior rim and posterior malalignment of outlet septum; Doubly committed juxta-arterial ventricular septal defect with muscular posterior inferior rim “coarctation type”
220	204	07.12.06	Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and perimembranous extension	A congenital cardiac malformation in which there is an outlet ventricular septal defect with perimembranous extension, bordered superiorly by the area of fibrous continuity or a fibrous outlet septum between the aortic and pulmonary valves, and the fibrous outlet septum is malaligned in a postero-caudal fashion with respect to the trabecular muscular septum such that there is usually obstruction to the left ventricular outflow tract.		Doubly committed subarterial ventricular septal defect with perimembranous extension and posterior malalignment of outlet septum; Doubly committed juxta-arterial ventricular septal defect with perimembranous extension “coarctation type”; Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and fibrous postero-inferior rim (perimembranous)
221	205	07.15.01	Ventricular septal defect hemodynamically insignificant	A congenital cardiac malformation in which there is one or more small, clinically insignificant ventricular septal defect(s) in the absence of flow-related cardiac chamber dilation or abnormal elevation of pulmonary arterial pressure.	Though restrictive ventricular septal defect is listed as a synonym of hemodynamically insignificant VSD, it should be recognized that some pressure restrictive ventricular septal defects will lead to flow-related chamber dilation, and thus would be hemodynamically significant. In such instances, the term hemodynamically insignificant ventricular septal defect should not be coded.	Maladie de Roger; Restrictive ventricular septal defect; Hemodynamically insignificant ventricular septal defect
222	206	07.15.04	Multiple ventricular septal defects	A congenital cardiac malformation in which there are multiple ventricular septal defects, which could be of any type.	For multiply fenestrated trabecular septums one should use the term “Multiple trabecular muscular ventricular septal defects.”
223	207	01.01.22	Functionally univentricular heart	The term “functionally univentricular heart” describes a spectrum of congenital cardiac malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation.	A heart may be functionally univentricular because of its anatomy or because of the lack of feasibility or lack of advisability of surgically partitioning the ventricular mass. Common lesions in this category typically include double inlet right ventricle (DIRV), double inlet left ventricle (DILV), tricuspid atresia, mitral atresia, and hypoplastic left heart syndrome. Other lesions which sometimes may be considered to be a functionally univentricular heart include complex forms of atrioventricular septal defect, double outlet right ventricle, congenitally corrected transposition, pulmonary atresia with intact ventricular septum, and other cardiovascular malformations. Specific diagnostic codes should be used whenever possible, and not the term “functionally univentricular heart.”	Univentricular cardiopathy; Single ventricle; Univentricular heart
224	208	01.01.14	Double inlet atrioventricular connection	A congenital cardiovascular malformation with a univentricular atrioventricular connection wherein both atria connect to one ventricle either via two separate atrioventricular valves or a common atrioventricular valve, such that all or nearly all of the total atrioventricular junctional (anular) area is committed to one ventricular chamber.		Double inlet ventricle
225	209	01.04.04	Double inlet left ventricle	A congenital cardiovascular malformation with a univentricular atrioventricular connection wherein both atria connect to a morphologically left ventricle either via two separate atrioventricular valves or a common atrioventricular valve, such that all or nearly all of the total atrioventricular junctional (anular) area is committed to the left ventricular chamber.			DILV
226	210	01.04.03	Double inlet right ventricle	A congenital cardiovascular malformation with a univentricular atrioventricular connection wherein both atria connect to a morphologically right ventricle either via two separate atrioventricular valves or a common atrioventricular valve, such that all or nearly all of the total atrioventricular junctional (anular) area is committed to the right ventricular chamber.			DIRV
227	211	01.04.05	Double inlet to solitary ventricle of indeterminate morphology	A congenital cardiovascular malformation with a univentricular atrioventricular connection wherein both atria connect to a solitary and morphologically indeterminate ventricle either via two separate atrioventricular valves or a common atrioventricular valve.
228	212	06.01.01	Tricuspid atresia	A congenital cardiovascular malformation with absence of the tricuspid valvar anulus (connection/junction) or an imperforate tricuspid valve.
229	213	06.01.26	Tricuspid atresia with absent atrioventricular connection	A congenital cardiovascular malformation with absence of the tricuspid valve and its associated anulus (connection/junction).		Tricuspid atresia with absent valvar anulus
230	214	06.01.02	Tricuspid atresia with imperforate tricuspid valve	A congenital cardiovascular malformation with an imperforate tricuspid valve (the tricuspid valve and associated atrioventricular connection/junction are present but the valve is imperforate).
231	215	06.02.01	Mitral atresia	A congenital cardiovascular malformation with absence of the mitral valvar anulus (connection/junction) or an imperforate mitral valve.
232	216	06.02.26	Mitral atresia with absent atrioventricular connection	A congenital cardiovascular malformation with absence of the mitral valve and its associated anulus (connection/junction).		Mitral atresia with absent valvar anulus
233	217	06.02.02	Mitral atresia with imperforate mitral valve	A congenital cardiovascular malformation with an imperforate mitral valve (the mitral valve and associated atrioventricular connection/junction are present but the valve is imperforate).
234	218	01.01.09	Hypoplastic left heart syndrome	A spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch.		Hypoplasia of the left heart; Hypoplasia of the left heart; HLHS; hlh- hypoplastic left heart syndrome; Left heart hypoplasia syndrome	HLHS
235	219	09.04.29	Congenital anomaly of a ventriculo-arterial valve or adjacent regions	A congenital cardiovascular malformation of a ventriculo-arterial valve or its immediate subvalvar and supravalvar regions. This does not include anomalies of the truncal valve which are classified along with codes related to the common arterial trunk.
236	220	09.05.29	Congenital anomaly of pulmonary valve	A congenital malformation of the heart where the pulmonary valve is abnormal.
237	221	09.05.04	Congenital pulmonary valvar stenosis	A congenital cardiovascular malformation of the pulmonary valve in which there is narrowing or stricture causing obstruction to flow	Congenital pulmonary valvar stenosis ranges from critical neonatal pulmonic valve stenosis with hypoplasia of the right ventricle to valvar pulmonary stenosis in the infant, child, or adult.	Congenital pulmonary stenosis; Congenital pulmonary valve stricture; Congenital stenosis of pulmonary valve; Congenital pulmonary valve stenosis
238	222	09.05.05	Pulmonary anular hypoplasia	A congenital cardiovascular malformation of the pulmonary valve in which its “anulus” is hypoplastic (incomplete development or underdevelopment so that it is abnormally small [below the lower limit of normal adjusted for body size]).
239	223	09.05.22	Congenital pulmonary regurgitation	A congenital cardiovascular malformation of the pulmonary valve allowing backward flow into the ventricle	Congenital pulmonary valve regurgitation may be due to primary anular dilation, prolapse, and leaflet underdevelopment.	Congenital pulmonary insufficiency; Congenital pulmonary incompetence; Congenital insufficiency of pulmonary valve
240	224	09.05.24	Dysplasia of pulmonary valve	A congenital cardiovascular malformation where the pulmonary valve leaflets are markedly thickened with restricted mobility, characterized by the presence of myxomatous tissue.		Pulmonary valve dysplasia; Dysplastic pulmonary valve; Pulmonary valve cusp dysplasia
241	225	09.05.32	Bicuspid pulmonary valve	A congenital cardiovascular malformation where the pulmonary valve has only two leaflets (cusps) and includes absent or fused commissure.		Bicommisural pulmonary valve
242	226	07.05.32	Congenital subpulmonary stenosis	A congenital cardiovascular malformation associated with narrowing within the outflow tract supporting the pulmonary valve.	Subvalvar (infundibular) pulmonary stenosis is a narrowing of the outflow tract of the ventricle immediately below the pulmonic valve. This term should preferably be used in the setting of abnormal ventriculo-arterial connections, such as double outlet ventricle. Although subpulmonary obstruction is a type of right ventricular outflow tract obstruction if the ventriculo-arterial connections are normal, in this setting “Congenital right ventricular outflow tract obstruction” should be used. Subvalvar pulmonary stenosis is also a type of left ventricular outflow tract obstruction in the setting of discordant ventriculo-arterial connections; this term should be used when obstruction is only apparent immediately below the pulmonary valve, otherwise the term “Congenital left ventricular outflow tract obstruction” should be used.	Infundibular pulmonary stenosis
243	227	09.07.15	Congenital supravalvar pulmonary stenosis	A congenital cardiovascular malformation associated with narrowing at the level of the pulmonary sinotubular junction.
244	228	09.05.16	Congenital pulmonary atresia	A congenital cardiovascular malformation in which there is no opening between any ventricle and the pulmonary arterial tree.	For “pulmonary atresia with VSD,” please see the section under “Tetralogy of Fallot.”	Pulmonary atresia.
245	Q ⁺	09.05.12	Congenital pulmonary valvar atresia	A congenital cardiac malformation in which the pulmonary valve leaflet structure is present but imperforate.	This term is limited to describe hearts in which the pulmonary subvalvar or supravalvar regions are not atretic.	Congenital pulmonary valve atresia; Imperforate pulmonary valve; Pulmonary valvar atresia; Atretic pulmonary valve; Pulmonary valvular atresia.
246	229	01.01.07	Pulmonary atresia with intact ventricular septum	A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication.	Pulmonary atresia with intact ventricular septum is a duct-dependent congenital malformation that forms a spectrum of lesions including atresia of the pulmonary valve, a varying degree of right ventricle and tricuspid valve hypoplasia, and anomalies of the coronary circulation. A right ventricular dependent coronary artery circulation is present when coronary artery fistulas are associated with a proximal coronary artery stenosis. Associated Ebstein anomaly of the tricuspid valve can be present.		PA/IVS
247	230	09.15.19	Congenital anomaly of aortic valve	A congenital cardiovascular malformation where the aortic valve is abnormal
248	231	09.15.01	Congenital aortic valvar stenosis	A congenital cardiovascular malformation of the aortic valve in which there is narrowing or stricture (obstruction to flow). “Congenital aortic valvar stenosis” arises most commonly as a result of partial or complete fusion of one or more commissures, or is due to dysplasia of one or more aortic cusps. These congenital malformations of the aortic valve may not be initially obstructive but may become stenotic later in life due to leaflet thickening, poor relative growth and-or calcification. It is not until the congenitally malformed aortic valve is or becomes stenotic that this term should be used.		Congenital valvar aortic stenosis; Congenital valvular aortic stenosis; Congenital aortic valve stricture; Congenital stenosis of aortic valve; Congenital aortic valve stenosis	AS
249	232	09.15.07	Congenital aortic regurgitation	A congenital cardiovascular malformation of the aortic valve allowing backward flow into the ventricle.	Congenital aortic regurgitation/insufficiency is rare as an isolated entity. Aortic insufficiency is more commonly seen with other associated cardiovascular anomalies.	Congenital aortic incompetence; Congenital aortic insufficiency; AR- Congenital aortic regurgitation; Congenital aorta valve insufficiency; Congenital aortic regurgitation; Congenital insufficiency of aortic valve	Congenital AI; Congenital AR
250	233	09.15.22	Bicuspid aortic valve	A congenital cardiovascular malformation where the aortic valve has two commissures and two separate leaflets (cusps) because of fusion or absence of one of the commissures		Bicommisural aortic valve	BAV
251	234	09.15.21	Unicuspid aortic valve	A congenital cardiovascular malformation in which the aortic valve has a single commissure and a single or functionally single leaflet (cusp)		Unicommissural aortic valve
252	235***	09.15.30	Aortic valvar prolapse	A congenital cardiovascular malformation of the aortic valve in which part or all of one or more of the aortic valve leaflets is on the ventricular side of the plane of the inferior aspect of the attachments of the aortic valve leaflets.		Aortic valve prolapse
253	236	09.15.06	Aortic valvar atresia	A congenital cardiovascular malformation in which there is no orifice of the aortic valve.	Aortic valve atresia will most often be coded under the hypoplastic left heart syndrome/complex diagnostic codes since it most often occurs as part of a spectrum of cardiovascular malformations. However, there is a small subset of patients with aortic valve atresia who have a well developed left ventricle and mitral valve and a large ventricular septal defect (nonrestrictive or restrictive).	Aortic valve atresia; Atresia of the aortic valve; Aorta valvular atresia; Aortic valvular atresia; Congenital aortic: atresia; Congenital atresia of aortic valve; Imperforate aortic valve	AA
254	237	09.15.17	Aortic anular hypoplasia	A congenital cardiovascular malformation of the aortic valve in which its “anulus” is hypoplastic (incomplete development or underdevelopment so that it is abnormally small [below the lower limit of normal adjusted for body size]).		Hypoplasia of the aortic anulus
255	238	09.15.09	Dysplasia of aortic valve	A congenital cardiovascular malformation where the aortic valve leaflets are markedly thickened with restricted mobility, characterized by the presence of myxomatous tissue.		Aortic valve dysplasia; Aortic valvar dysplasia
256	239	07.09.50	Congenital subaortic stenosis	A congenital cardiovascular malformation associated with narrowing within the outflow tract supporting the aortic valve.	This term should be used for congenital subaortic stenosis due to lesions such as deviation of the outlet septum (seen in coarctation of the aorta and interrupted aortic arch), or a restrictive ventricular septal defect (bulboventricular foramen) in single ventricle complexes. This term should not be used for subaortic stenosis due to a fibromuscular shelf or tunnel, as specific terms exist for these lesions. For subaortic stenosis due to an atrioventricular valve in the setting of normal ventriculo-arterial connections, the term “Congenital left ventricular outflow tract obstruction due to an atrioventricular valve” should be used.	Congenital subvalvular aortic stenosis; Subvalvular aortic stenosis, Congenital; Congenital subvalvar aortic stenosis
257	240	07.09.03	Subaortic stenosis due to fibromuscular shelf	A cardiovascular malformation in which there is subaortic stenosis due to a discrete fibrous and/or muscular ridge.		Discrete LVOTO
258	241	07.09.16	Subaortic stenosis due to fibromuscular tunnel	A cardiovascular malformation in which there is a long-segment fibrous and/or muscular subaortic stenosis.		Diffuse LVOTO
259	242	09.16.18	Congenital supravalvar aortic stenosis	A congenital cardiovascular malformation with narrowing of the aorta at the level of the sinotubular junction which may extend into the ascending aorta.	Congenital supravalvar aortic stenosis is described as three forms: an hourglass deformity, a fibrous membrane, and a diffuse narrowing of the ascending aorta. Supravalvar aortic stenosis may involve the coronary artery ostia, and the aortic leaflets may be tethered. The coronary arteries can become tortuous and dilated due to elevated pressures and early atherosclerosis may ensue.	Stenosis at or above the sinotubular junction; Ascending aorta stenosis; Ascending aorta stricture
260	243	09.18.01	Aneurysm of aortic sinus of Valsalva	A congenital cardiovascular malformation in which there is dilation of one or more aortic sinus of Valsalva.	The sinus of Valsalva is defined as that portion of the aortic root between the aortic root anulus and the sinotubular junction. Sinus of Valsalva aneurysm most commonly originates from the right sinus, less commonly from the non-coronary sinus, and rarely from the left sinus (<5%). The aneurysm may rupture into an adjacent chamber or site (right atrium, right ventricle, left atrium, left ventricle, pulmonary artery, pericardium) and in this case should be coded specifically (“Ruptured aortic sinus of Valsalva aneurysm”). This is to be distinguished from aortic root dilation associated with connective tissue disorders and aortopathies.	Aortic sinus of Valsalva aneurysm
261	244	09.17.01	Aortoventricular tunnel	A congenital cardiovascular malformation in which there is a paravalvar communication between the aorta and a ventricle.		Aortico-ventricular tunnel; Ventriculo-aortic tunnel; Aorto-ventricular tunnel
262	R ⁺	09.17.02	Aorto-left ventricular tunnel	A congenital cardiovascular malformation in which there is a paravalvar communication between the ascending aorta and the left ventricle.
263	S ⁺	09.17.04	Aorto-right ventricular tunnel	A congenital cardiovascular malformation in which there is a paravalvar communication between the ascending aorta and the right ventricle
264	245	09.04.28	Congenital anomaly of great arteries including arterial duct	A congenital cardiovascular malformation of the great arteries (aorta, pulmonary trunk [main pulmonary artery], branch pulmonary arteries) or the arterial duct (ductus arteriosus).	This term excludes the truncal root in “Common arterial trunk” (truncus arteriosus).
265	246	09.04.07	Congenital aortopulmonary window	A congenital cardiovascular malformation in which there is side-to-side continuity of the lumens of the ascending aorta and pulmonary trunk in association with separate aortic and pulmonary valves or their atretic remnants.	Side-to-side continuity of the lumens of the aorta and pulmonary arterial tree, which is distinguished from common arterial trunk (truncus arteriosus) by the presence of two arterial valves or their atretic remnants, and involvement of the pulmonary trunk (main pulmonary artery).	Aortopulmonary window; Aortic septal defect; Aortopulmonary septal defect; Aorticopulmonary window; Aorticopulmonary fenestration; Aorticopulmonary septal defect
266	247	09.07.16	Congenital anomaly of pulmonary arterial tree	A congenital cardiovascular malformation of the pulmonary trunk (main pulmonary artery) and/or branch pulmonary arteries (right, left, and ramifications).
267	248	09.10.36	Congenital dilation of pulmonary arterial tree	A congenital cardiovascular malformation in which there is an enlargement of the luminal diameter of the pulmonary trunk (main pulmonary artery) and/or branch pulmonary arteries (above the upper limit of normal adjusted for body size).	Includes idiopathic isolated PA dilation
268	249	09.07.19	Congenital pulmonary trunk anomaly	A congenital cardiovascular malformation of the pulmonary arterial trunk (main pulmonary artery).		Congenital main pulmonary artery anomaly
269	250	09.07.20	Congenital pulmonary trunk hypoplasia	A congenital cardiovascular malformation of the pulmonary trunk (main pulmonary artery) in which there is incomplete development or underdevelopment resulting in diffuse luminal narrowing (below the lower limit of normal adjusted for body size).	The stenosis or hypoplasia may be isolated or associated with other cardiovascular lesions. Since the narrowing is distal to the pulmonary valve, it may also be known as supravalvar pulmonary stenosis.	Main pulmonary artery hypoplasia
270	251	09.07.05	Absent or atretic pulmonary trunk	A congenital cardiovascular malformation where the pulmonary trunk (main pulmonary artery) is not present or has luminal occlusion, excluding common arterial trunk.		Pulmonary artery atresia; Atretic pulmonary trunk; Pulmonary trunk absent; Absent main pulmonary artery; Atretic main pulmonary artery; Absent or atretic main pulmonary artery
271	252	09.10.41	Congenital pulmonary arterial branch anomaly	A congenital cardiovascular malformation of a pulmonary arterial branch.		Congenital abnormality of pulmonary artery
272	253	09.10.27	Congenital pulmonary arterial branch stenosis
273	254	09.10.28	Congenital right pulmonary arterial stenosis	A congenital cardiovascular malformation in which there is discrete narrowing of the luminal diameter of one or more segments of the right pulmonary artery (below the lower limit of normal adjusted for body size).
274	255	09.10.29	Congenital left pulmonary arterial stenosis	A congenital cardiovascular malformation in which there is discrete narrowing of the luminal diameter of one or more segments of the left pulmonary artery (below the lower limit of normal adjusted for body size).
275	256	09.10.71	Congenital pulmonary arterial branch hypoplasia	A congenital cardiovascular malformation in which there is diffuse luminal narrowing of a pulmonary arterial branch (below the lower limit of normal adjusted for body size).		Hypoplasia of pulmonary artery; Congenital hypoplasia of pulmonary artery; Pulmonary artery hypoplasia
276	257	09.10.72	Congenital right pulmonary arterial hypoplasia	A congenital cardiovascular malformation in which there is diffuse luminal narrowing of the right pulmonary artery (below the lower limit of normal adjusted for body size).
277	258	09.10.73	Congenital left pulmonary arterial hypoplasia	A congenital cardiovascular malformation in which there is diffuse luminal narrowing of the left pulmonary artery (below the lower limit of normal adjusted for body size).
278	T ⁺	09.10.21	Absent or atretic right or left pulmonary artery	A congenital cardiovascular malformation in which the right and/or left pulmonary artery is not present or has luminal occlusion.		Atretic right or left pulmonary artery; Absent right or left pulmonary artery; Agenesis of pulmonary artery; Pulmonary arterial agenesis
279	259	09.10.75	Absent or atretic right pulmonary artery	A congenital cardiovascular malformation in which the right pulmonary artery is not present or has luminal occlusion.		Atretic right pulmonary artery; Absent right pulmonary artery
280	260	09.10.77	Absent or atretic left pulmonary artery	A congenital cardiovascular malformation in which the left pulmonary artery is not present or has luminal occlusion.		Atretic left pulmonary artery; Absent left pulmonary artery
281	261	09.10.37	Congenital central pulmonary arterial stenosis or hypoplasia proximal to hilar bifurcation	A congenital cardiovascular malformation of a pulmonary artery, proximal to its first branch, in which there is luminal narrowing (below the lower limit of normal adjusted for body size).	The stenosis or hypoplasia may be isolated or associated with other cardiovascular lesions. Coarctation of the pulmonary artery is related to abnormal extension of the arterial duct (ductus arteriosus) into a pulmonary arterial branch, more frequently the left branch. This is to be distinguished from narrowing or hypoplasia of the pulmonary trunk (main pulmonary artery).	Central pulmonary arterial stenosis; Central pulmonary stenosis; Proximal pulmonary arterial stenosis; Proximal pulmonary stenosis
282	262	09.10.38	Congenital peripheral pulmonary arterial stenosis or hypoplasia at or beyond hilar bifurcation	A congenital cardiovascular malformation of a pulmonary artery, distal to its first branch, in which there is luminal narrowing (below the lower limit of normal adjusted for body size).	Peripheral pulmonary artery narrowing or hypoplasia at or beyond the hilar bifurcation. The stenosis or hypoplasia may be isolated or associated with other cardiovascular lesions.	Peripheral pulmonary stenosis; Distal peripheral pulmonary stenosis; Peripheral pulmonary arterial stenosis; Distal peripheral pulmonary arterial stenosis
283	263	09.10.30	Congenitally discontinuous, non-confluent right and left pulmonary arteries	A congenital cardiovascular malformation in which there is absence of luminal continuity between the right and left branch pulmonary arteries.	The discontinuous branch pulmonary artery is typically supplied by a patent arterial duct (ductus arteriosus) or an aortopulmonary collateral.
284	264	09.09.08	Pulmonary artery origin from ascending aorta	A congenital cardiovascular malformation in which one branch pulmonary artery arises from the ascending aorta and the other branch pulmonary artery arises from the pulmonary trunk (main pulmonary artery).	One pulmonary artery arises from the ascending aorta and the other pulmonary artery arises from the right ventricle. This does include origin of the right or left pulmonary artery from the innominate (brachiocephalic) artery or the aortic arch via a patent arterial duct (ductus arteriosus) or collateral artery.		Hemitruncus
285	265	09.09.03	Right pulmonary artery from ascending aorta	A congenital cardiovascular malformation in which the right pulmonary artery arises from the ascending aorta and the left pulmonary artery arises from the pulmonary trunk (main pulmonary artery).			Hemitruncus
286	266	09.09.05	Left pulmonary artery from ascending aorta	A congenital cardiovascular malformation in which the left pulmonary artery arises from the ascending aorta and the right pulmonary artery arises from the pulmonary trunk (main pulmonary artery).			Hemitruncus
287	267	09.09.11	Pulmonary artery from arterial duct	A congenital cardiovascular malformation in which the pulmonary arteries are non-confluent and one or both arise from an arterial duct (ductus arteriosus).	When both arise from AD then code under discontinuous	Pulmonary artery from ductus arteriosus
288	268	09.09.02	Right pulmonary artery from arterial duct	A congenital cardiovascular malformation in which the pulmonary arteries are non-confluent and the right pulmonary artery arises from an arterial duct (ductus arteriosus).		Right pulmonary artery from ductus arteriosus
289	269	09.09.04	Left pulmonary artery from arterial duct	A congenital cardiovascular malformation in which the pulmonary arteries are non-confluent and the left pulmonary artery arises from an arterial duct (ductus arteriosus).		Left pulmonary artery from ductus arteriosus
290	270	07.09.34	Congenital anomaly of aorta or its branches	A congenital cardiovascular malformation of the aorta and/or its branches.
291	271	09.16.06	Congenital anomaly of ascending aorta	A congenital cardiovascular malformation of the aorta between the sinotubular junction and the origin of its first branch.
292	272	09.16.02	Hypoplasia of ascending aortic	A congenital cardiovascular malformation in which the luminal diameter of the aorta between its sinotubular junction and the origin of the innominate (brachiocephalic) artery is narrowed (below the lower limit of normal adjusted for body size).		Ascending aortic hypoplasia
293	273	09.16.19	Congenital ascending aortic aneurysm or dilation	A congenital cardiovascular malformation in which the luminal diameter of the aorta between its sinotubular junction and the origin of the innominate (brachiocephalic) artery is dilated (above the upper limit of normal adjusted for body size).
294	274	09.28.10	Congenital anomaly of aortic arch	A congenital cardiovascular malformation of the aorta between the origin of the innominate artery and the insertion of the arterial duct (ductus arteriosus).
295	275	09.29.11	Hypoplasia of aortic arch	A congenital cardiovascular malformation in which there is diffuse luminal narrowing of the aortic arch (below the lower limit of normal adjusted for body size).	Hypoplasia of the aortic arch is hypoplasia of the proximal or distal transverse arch or the aortic isthmus. The isthmus (arch between the left subclavian and insertion of the patent ductus arteriosus/ ligamentum arteriosum) is hypoplastic if its diameter is less than 40% of the diameter of the ascending aorta. The proximal transverse arch (arch between the innominate and left carotid arteries) and distal transverse arch (arch between the left carotid and left subclavian arteries) are hypoplastic if their diameters are less than 60% and 50%, respectively, of the diameter of the ascending aorta.	Hypoplastic aortic arch; Arch hypoplasia; Aortic hypoplasia; Aortic arch hypoplasia
296	277	09.29.31	Interrupted aortic arch	A congenital cardiovascular malformation in which there is an absence of luminal continuity between the ascending and descending aorta.	This term includes luminal atresia with discontinuity between the aortic segments and also luminal atresia with fibrous continuity between the aortic segments. Interrupted aortic arch is defined as the loss of luminal continuity between the ascending and descending aorta. In most cases, blood flow to the descending thoracic aorta is through a patent arterial duct, and there is a large ventricular septal defect. Arch interruption is further defined by site of interruption. In type A, interruption is distal to the left subclavian artery; in type B, interruption is between the left carotid and left subclavian arteries; and in type C, interruption occurs between the innominate and left carotid arteries.	Aortic arch interruption; Aortic interruption; Interruption of the aortic arch; Aortic atresia	IAA
297	278	09.29.32	Interrupted aortic arch distal to subclavian artery, type A	A congenital cardiovascular malformation of the aorta in which there is an absence of luminal continuity distal to the subclavian artery ipsilateral to the arch.		Aortic arch interruption Type A; Aortic interruption Type A; Interruption of the aortic arch Type A	IAA Type A
298	279	09.29.33	Interrupted aortic arch between subclavian and common carotid arteries, type B	A congenital cardiovascular malformation of the aorta in which there is an absence of luminal continuity of the aorta between the carotid and subclavian arteries.		Aortic arch interruption Type B; Aortic interruption Type B; Interruption of the aortic arch Type B	IAA Type B
299	280	09.29.34	Interrupted aortic arch between carotid arteries, type C	A congenital cardiovascular malformation of the aorta in which there is an absence of luminal continuity of the aorta between the carotid arteries.		Aortic arch interruption Type C; Aortic interruption Type C; Interruption of the aortic arch Type C	IAA Type C
300	281	09.28.15	Right aortic arch	A congenital cardiovascular malformation of the great vessels in which the aortic arch crosses to the right of the trachea.		Right arch; Right-sided arch; Right-sided aortic arch; Persistent right arch; Persistent right-sided arch; Persistent right-sided aortic arch
301	282	09.28.22	Left aortic arch	A congenital cardiovascular finding of the great vessels in which the aortic arch crosses to the left of the trachea.	To be coded only when this represents an abnormal finding, such as in situs inversus.	Left arch; Left-sided arch; Left-sided aortic arch
302	283	09.28.06	Cervical aortic arch	A congenital cardiovascular malformation in which the aortic arch is located superior to the clavicle, and is most commonly right-sided.		Cervical arch
303	X ⁺	09.30.22	Aortic diverticulum of Kommerell	A congenital cardiovascular malformation consisting of an aneurysmally dilated proximal portion of an aberrant subclavian artery or aberrant innominate (brachiocephalic) artery as it arises from the descending aorta.
304	Y ⁺	09.28.08	Persistent fifth aortic arch	A congenital cardiovascular malformation in which there is an accessory artery originating from the ascending aorta proximal to the ostium of the innominate (brachiocephalic) artery which connects to the descending aorta or near the confluence of the right and left pulmonary arteries.	When the arterial connection is between the ascending and descending aorta, it is often associated with coarctation of the aorta. This malformation may be distinguished from a double aortic arch by the lack of arch vessels arising from it and by establishing that the accessory vessel and the aortic arch lie on the same side of the tracheoesophageal axis. Although this malformation has been termed a “persistent fifth aortic arch,” the use of this term is in dispute.
305	276	09.29.01	Coarctation of aorta	A congenital cardiovascular malformation in which there is a discrete luminal narrowing of the junction between the aortic arch and the descending aorta.	Coarctation of the aorta generally indicates a narrowing of the descending thoracic aorta just distal to the left subclavian artery. However, the term may also be accurately used to refer to a region of narrowing anywhere in the thoracic or abdominal aorta.	Aortic coarctation
306	U ⁺	09.29.02	Preductal coarctation of aorta	A congenital cardiovascular malformation in which there is narrowing of the aortic lumen proximal to the insertion of the arterial duct (ductus arteriosus) or ligament (ligamentum arteriosum).
307	V ⁺	09.29.04	Postductal coarctation of aorta	A congenital cardiovascular malformation in which there is narrowing of the aortic lumen distal to the insertion of the arterial duct (ductus arteriosus) or ligament (ligamentum arteriosum).
308	W ⁺	09.29.03	Juxtaductal (paraductal) coarctation of aorta	A congenital cardiovascular malformation in which there is narrowing of the aortic lumen opposite the level of insertion of the arterial duct (ductus arteriosus) or ligament (ligamentum arteriosum).
309	284	09.30.17	Congenital anomaly of aortic arch branch	A congenital cardiovascular malformation of one or more branches of the aortic arch (innominate, carotid, or subclavian arteries).
310	285	09.30.02	Aberrant origin of right subclavian artery	A congenital cardiovascular malformation in which the right subclavian artery arises distal to the left subclavian artery in the setting of a left aortic arch.	Dysphagia lusoria (or Bayford-Autenrieth dysphagia) is an abnormal condition characterized by difficulty in swallowing caused by an aberrant right subclavian artery. It was discovered by David Bayford in 1761 and first reported in a paper by the same in 1787	Aberrant right subclavian artery; Arteria lusoria; Dysphagia lusoria; Aberrant right subclavian artery syndrome	ARSA, ARSCA
311	286	09.30.04	Aberrant origin of left subclavian artery	A congenital cardiovascular malformation in which the left subclavian artery arises distal to the right subclavian artery in the setting of a right arch.		Aberrant left subclavian artery; Retroesophageal left subclavian artery	ALSA, ALSCA, RELSCA
312	287	09.30.16	Isolation of an aortic arch branch	A congenital cardiovascular malformation in which the only connection to a brachiocephalic vessel is via an arterial duct (ductus arteriosus) or its ligament.
313	Z ⁺	09.30.11	Isolation of innominate artery	A congenital cardiovascular malformation in which the innominate (brachiocephalic) artery has no direct connection to the aorta.		Isolation of brachiocephalic artery; Isolation of brachiocephalic trunk
314	AA ⁺	09.30.14	Isolation of left subclavian artery	A congenital cardiovascular malformation in which the left subclavian artery has no direct connection to the aorta.
315	AB ⁺	09.30.15	Isolation of right subclavian artery	A congenital cardiovascular malformation in which the right subclavian artery has no direct connection to the aorta.
316	AC ⁺	09.30.12	Isolation of left common carotid artery	A congenital cardiovascular malformation in which the left common carotid artery has no direct connection to the aorta.
317	AD ⁺	09.30.13	Isolation of right common carotid artery	A congenital cardiovascular malformation in which in which the right common carotid artery has no direct connection to the aorta.
318	AE ⁺	09.30.34	Aberrant origin of innominate artery	A congenital cardiovascular malformation in which an innominate (brachiocephalic) artery arises from an abnormally distal position on the aortic arch, or from a diverticulum of Kommerell, or from a pulmonary artery.		Aberrant origin of brachiocephalic artery; Aberrant origin of brachiocephalic trunk
319	AF ⁺	09.30.31	Common origin of the innominate artery and left common carotid artery	A congenital cardiovascular malformation in which the innominate (brachiocephalic) artery and left common carotid artery arise from a single aortic orifice in the setting of a left aortic arch.	This term as well as “Left common carotid arising from innominate artery” have been used synonymously (but incorrectly) with the term “Bovine aortic arch,” despite the fact that neither resemble a true bovine arch.
320	AG ⁺	09.30.28	Separate origins of internal and external carotid arteries	A congenital cardiovascular malformation in which there is an absence of a common carotid artery and the internal and external carotid arteries connect separately to the aorta.
321	288	09.28.47	Congenital anomaly of descending thoracic or abdominal aorta	A congenital cardiovascular malformation of the aorta distal to the aortic arch.		Congenital anomaly of thoracoabdominal aorta
322	289	09.29.44	Descending thoracic or abdominal aortic coarctation	A congenital cardiovascular malformation in which there is discrete luminal narrowing of the descending thoracic or abdominal aorta.		Middle aortic syndrome	MAS
323	AH ⁺	09.29.05	Coarctation of the descending thoracic aorta	A congenital cardiovascular malformation in which there is discrete luminal narrowing of the descending thoracic aorta.
324	AI ⁺	09.29.06	Coarctation of the abdominal aorta	A congenital cardiovascular malformation in which there is discrete luminal narrowing of the abdominal aorta.
325	290	09.31.40	Tracheoesophageal compressive syndrome	A congenital cardiovascular malformation which causes compression of the trachea and/or the esophagus.
326	291	09.30.23	Innominate artery compression syndrome	A congenital cardiovascular malformation in which there is anterior compression of the trachea by the innominate artery.	This syndrome is a true compression of the trachea by the abnormally positioned innominate artery. The innominate artery can appear to “compress” the trachea in the presence of tracheomalacia, but in the absence of an abnormal origin and course of the innominate artery, this apparent “compression” most likely is a consequence of the tracheomalacia itself rather than actual compression by the innominate artery.	Innominate artery compressive syndrome; Brachiocephalic artery compression syndrome; Brachiocephalic artery compressive syndrome; Innominate artery compression of the trachea; Brachiocephalic artery compression of the trachea; Aberrant innominate artery; Aberrant brachiocephalic artery; Anomalous origin of the innominate artery; Anomalous origin of the brachiocephalic artery
327	AJ ⁺	09.30.27	Retro-esophageal origin of aberrant innominate artery	A congenital cardiovascular malformation in which the innominate (brachiocephalic) artery passes from right-to-left or from left-to-right posterior to the esophagus.
328	292	09.31.00	Vascular Ring	A congenital cardiovascular malformation in which one or more of the following encircle the trachea and esophagus: the aorta and its major branches, the pulmonary trunk and its major branches, and the arterial duct (ductus arteriosus) or their vascular remnant(s).	The term vascular ring refers to a group of congenital vascular anomalies that encircle and compress the esophagus and/or trachea. The compression may be from a complete anatomic ring (double aortic arch or right aortic arch with a left ligamentum) or from a compressive effect of an aberrant vessel (innominate artery compression syndrome).
329	293	09.28.09	Double aortic arch	A congenital cardiovascular malformation in which the right and left aortic arches (patent or atretic) encircle the trachea and esophagus.		Encircling double aortic arch	DAA
330	294	09.31.35	Vascular ring of right aortic arch and left arterial duct or ligament	A congenital cardiovascular malformation in which continuity of the right aortic arch, pulmonary trunk, and left arterial duct (or ligament) encircles the trachea and esophagus.	This diagnosis may or may not include the presence of a diverticulum of Kommerell.
331	295	09.31.34	Vascular ring of left aortic arch and right arterial duct or ligament	A congenital cardiovascular malformation in which continuity of the left aortic arch, pulmonary trunk, and right arterial duct (or ligament) encircles the trachea and esophagus.	This diagnosis may or may not include the presence of a diverticulum of Kommerell.
332	296	09.09.06	Anomalous origin of left pulmonary artery from right pulmonary artery	A congenital cardiovascular malformation in which the left pulmonary artery originates from the right pulmonary artery and passes between the trachea and esophagus, and is often associated with tracheobronchial anomalies such as tracheomalacia, stenosis, or complete tracheal rings.		Pulmonary arterial sling
333	297	09.27.05	Congenital arterial duct anomaly	A congenital cardiovascular malformation of the arterial duct (ductus arteriosus) or its fibrous remnant (ligamentum arteriosum).	The described anomalies include an anomalous course, abnormal laterality or duplication, persistent patency or premature closure, and aneurysm formation.	Congenital ductus arteriosus anomaly	Congenital PDA anomaly
334	298	09.27.21	Patent arterial duct	A congenital cardiovascular finding in which the arterial duct (ductus arteriosus) is open beyond the normal age of spontaneous closure.	A patent arterial duct (ductus arteriosus) is a vascular arterial connection between the thoracic aorta and the pulmonary artery. Most commonly, a patent arterial duct has its origin from the descending thoracic aorta, just distal and opposite the origin of the left subclavian artery. The insertion of the ductus is most commonly into the very proximal left pulmonary artery at its junction with the main pulmonary artery. Origination and insertion sites can be variable, however.	Patent ductus arteriosus; Persistent ductus Botalli; Patent ductus Botalli; Open ductus arteriosus; Persistent ductus arteriosus; Ductus arteriosus nonclosure; Patent ductus arteriosus- persisting type; PDA- patent ductus arteriosus; Persistent ductus arteriosus (Botalli)	PDA; PAD
335	AK ⁺	09.27.03	Absent arterial duct	A congenital cardiovascular malformation in which the arterial duct (ductus arteriosus) or ligament (ligamentum arteriosum) is not present.		Absent ductus arteriosus; Ductus arteriosus agenesis	Absent PDA; Absent PAD
336	AL ⁺	09.27.04	Congenital aneurysm of arterial duct	A congenital cardiovascular malformation where there is fusiform or saccular dilatation of the arterial duct (ductus arteriosus).		Aneurysm of ductus arteriosus	Aneurysm of PDA; Aneurysm of PAD
337	AM ⁺	09.27.41	Anomalous origin of arterial duct	A congenital cardiovascular malformation in which there is an arterial duct (ductus arteriosus) that is not in its expected position between the aorta or arch vessels and the pulmonary arterial circulation.	While in the setting of a left aortic arch the arterial duct is expected to arise from the concavity of the aortic arch, in the case of a right aortic arch it can normally be expected to arise from the base of the innominate (brachiocephalic) artery.	Anomalous origin of ductus arteriosus	Anomalous origin of PDA; Anomalous origin of PAD
338	AN ⁺	09.27.82	Anomalous origin of arterial ligament	A congenital cardiovascular malformation in which there is a remnant of the arterial duct (ductus arteriosus) that is not in its expected position between the aorta or arch vessels and the pulmonary arterial circulation.	While in the setting of a left aortic arch the arterial ligament is expected to be seen stretching from the concavity of the aortic arch to the pulmonary artery confluence, in the case of a right aortic arch one end of the ligament can normally be found at the base of the innominate (brachiocephalic) artery.
339	AO ⁺	14.10.51	Fetal arterial duct narrowing-closure	A congenital cardiovascular malformation in which there is prenatal partial or complete closure of the arterial duct (ductus arteriosus).		Fetal ductus arteriosus narrowing-closure	Fetal PDA narrowing-closure; Fetal PAD narrowing-closure
340	299	09.08.18	Systemic-to-pulmonary collateral arteries	A congenital cardiovascular malformation in which the blood supply to the lungs is derived completely or in part from collateral vessels that arise from the aorta or its branches.	At least part of the pulmonary blood flow is derived from systemic-to-pulmonary collateral arteries that are highly variable in number, and that usually arise from the descending thoracic aorta, but uncommonly may originate from the aortic arch or the subclavian, carotid, or even the coronary arteries. There may or may not be native pulmonary arteries which, if present, may be hypoplastic, and either confluent or non-confluent. This term is intended to exclude patients with systemic to pulmonary artery collaterals and “Tetralogy of Fallot with pulmonary atresia” since they are coded using the term “Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries.”	Major systemic-to-pulmonary collateral arteries; Major systemic-to-pulmonary collateral artery; Systemic-to-pulmonary collateral vessels; Systemic-to-pulmonary collateral flow; Aortopulmonary collateral arteries	APCAs, MAPCAs
341	300	09.46.03	Congenital anomaly of coronary artery	A congenital cardiovascular malformation of a coronary artery.	This includes absence of a coronary, anomalous origin or course, dilation or stenosis, and fistulas. Congenital anomalies of the coronary venous system should not be included here but rather under “Congenital anomaly of mediastinal systemic vein.”	Congenital malformations of coronary vessels; Malformation of coronary vessels
342	301	09.41.01	Anomalous origin of coronary artery from pulmonary arterial tree	A congenital cardiovascular malformation in which a coronary artery originates from the pulmonary trunk or one of its branches.	Although the most common of these malformations involves the left coronary artery arising from the pulmonary trunk (main pulmonary artery) rather than from the aorta, occasionally the right coronary artery, the circumflex, or both coronary arteries may arise from any of the central pulmonary arteries.
343	302	09.41.03	Anomalous origin of left coronary artery from pulmonary artery	A congenital cardiovascular malformation in which the left coronary artery originates from the pulmonary trunk or one of its branches.		Anomalous origin of left coronary artery from pulmonary trunk (ALCAPT)	ALCAPA; ALCAPT
344	303	09.42.00	Anomalous aortic origin or course of coronary artery	A congenital cardiovascular malformation in which the origin and/or course of a coronary artery is abnormal.	This is where coronary “anomalies” in the presence of discordant ventriculo-arterial connections should be coded.
345	304	09.42.21	Anomalous aortic origin of coronary artery with ventriculo-arterial concordance	A congenital cardiovascular malformation in the setting of ventriculo-arterial concordance in which a coronary artery arises from the aorta at a location other than its expected sinus.	Anomalous aortic origins of the coronary arteries include a spectrum of anatomic variations of the normal coronary artery origins. Coronary artery anomalies of aortic origin to be coded under this diagnostic field include anomalies of take-off (high take-off), origin (sinus), branching, and number. An anomalous course of the coronary artery vessels is also significant, particularly those coronary arteries that arise or course between the great vessels. This term is used for patients with concordant ventriculo-arterial connections.	AAOCA; Anomalous aortic origin of coronary artery	AAOCA
346	305	09.46.26	Right coronary artery from left aortic sinus with ventriculo-arterial concordance	A congenital cardiovascular malformation in the setting of ventriculo-arterial concordance in which the right coronary artery arises from, or immediately above, the left sinus of Valsalva.	This term applies to patients with concordant ventriculo-arterial connections.
347	306	09.46.21	Left coronary artery from right aortic sinus with ventriculo-arterial concordance	A congenital cardiovascular malformation in the setting of ventriculo-arterial concordance in which the left coronary artery arises from, or imm ediately above, the right sinus of Valsalva.	This term applies to patients with concordant ventriculo-arterial connections.
348	307	09.43.04	Anterior descending from right coronary artery across right ventricular outflow tract	A congenital cardiovascular malformation in which the left circumflex coronary artery arises normally and the anterior descending coronary artery arises from the proximal right coronary artery and courses across the right ventricular outflow tract.		Anterior interventricular artery from right coronary artery across right ventricular outflow tract; Left anterior descending (LAD) from right coronary artery across right ventricular outflow tract
349	308	09.43.05	Intramural proximal coronary arterial course	A congenital cardiovascular malformation in which the proximal coronary artery courses within and parallel to the wall of the aorta before it emerges to assume its epicardial course.		Intramural coronary artery
350	309	09.43.13	Single coronary artery supplying all of heart	A congenital cardiovascular malformation in which a solitary coronary artery supplies the myocardium.
351	310	09.43.12	Myocardial bridging of coronary artery	A congenital cardiovascular malformation in which a usually epicardial coronary arterial segment is located within the ventricular myocardium, making this segment susceptible to compression during systole.		Myocardial bridge	MB
352	311	09.44.05	Congenital coronary arterial orifice stenosis	A congenital cardiovascular malformation in which the orifice of a coronary artery is narrowed.		Congenital coronary ostial stenosis
353	312	09.44.19	Congenital coronary arterial orifice atresia	A congenital cardiovascular malformation in which the orifice of a coronary artery is not patent.	This excludes single coronary artery anomalies	Congenital coronary ostial atresia	COSA
354	313	09.45.16	Congenital coronary arterial fistula	A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with a cardiac chamber or with any segment of the pulmonary circulation.	This communication may be simple and direct or may be tortuous and dilated. In order of frequency the involved coronary artery is the right, the left and, rarely, both coronary arteries. Occasionally multiple fistulas are present.	Coronary fistula
355	314	09.45.10	Congenital coronary arterial fistula to right ventricle	A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with the right ventricle.
356	315	09.45.22	Congenital coronary arterial fistula to left ventricle	A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with the left ventricle.
357	316	09.46.14	Congenital coronary arterial aneurysm	A congenital cardiovascular malformation in which there is one or more localized dilation(s) of a coronary vessel.	Coronary artery aneurysms are usually seen in two forms, saccular (shaped like a sack, with a change in caliber over a very short distance), and fusiform (shaped like a spindle, tapering towards each end), and both may be single or multiple.
358	AP ⁺	09.42.09	Accessory coronary artery	A congenital cardiac malformation in which there is an additional coronary artery that duplicates the blood supply of an existing and named coronary artery.
359	AQ ⁺	09.46.44	Congenital absence of coronary artery	A congenital cardiac malformation in which a right, left main, interventricular, or circumflex coronary artery is not present in its anticipated epicardial course.
360	AR ⁺	09.46.19	Coronary arterial hypoplasia	A congenital cardiovascular malformation in which one or more coronary arteries have an abnormally reduced length or lumen diameter.
361	317	10.01.05	Congenital pericardial anomaly	A congenital malformation in which there is a structural and/or functional abnormality of the pericardium.	This term can include complete or partial absence of pericardium, pericardial cysts, antenatal pericardial effusion, and congenital tumors of the serous pericardium.
362	AS ⁺	10.01.02	Complete agenesis of pericardium	A congenital cardiac malformation in which the fibroserous pericardium is completely absent.
363	AT ⁺	10.01.01	Partial agenesis of pericardium	A congenital cardiac malformation in which the fibroserous pericardium is partly absent.
364	AU ⁺	10.01.03	Pleuropericardial cyst	A congenital finding in which there is a closed sac typically found at the pleuropericardial angle, having a distinct membrane and division compared to the nearby tissue.	It may contain air, fluid, or semi-solid material. Congenital non-malignant pleuropericardial cysts include pericardial teratoma, cystic lymphangioma, bronchogenic, and celomic cysts.
365	AV ⁺	10.03.53	Congenital cardiac tumor	A congenital malformation consisting of growth of abnormal tissue within the heart.
366	318***	09.19.05	Pulmonary arteriovenous fistula	A congenital cardiovascular malformation in which there is an abnormal, direct connection between a pulmonary artery and pulmonary vein or left atrium without an intervening capillary bed.		Pulmonary arteriovenous malformation; Pulmonary arteriovenous aneurysm
367	AW ⁺	02.02.03	Bifid apex of heart	A congenital cardiac finding in which there is a notch or cleft on the epicardial surface of the heart at the level of the distal interventricular groove that divides the apex in two, so that the apex of the left ventricle lies on one side of the cleft while the apex of the right ventricle lies on the other.	A bifid cardiac apex can be associated with congenital malformations but may also be found in otherwise normal hearts.

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Tags: Christo I. Tchervenkov, Jeffrey P. Jacobs, Kirklin/Barratt-Boyes Cardiac Surgery

Apr 21, 2026 | Posted by drzezo in CARDIAC SURGERY | Comments Off

Thoracic Key

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Nomenclature for congenital heart disease

Introduction

Historical considerations

Principles of nomenclature

Clinical versus administrative nomenclature

The meaning of the words right and left

Structural differences between the systems of nomenclature

The international pediatric and congenital cardiac code (IPCCC)

Eleventh revision of the international classification of diseases (ICD-11)

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Thoracic Key

Fastest Thoracic Insight Engine

Nomenclature for congenital heart disease

Introduction

Historical considerations

Principles of nomenclature

Clinical versus administrative nomenclature

The meaning of the words right and left

Structural differences between the systems of nomenclature

The international pediatric and congenital cardiac code (IPCCC)

Eleventh revision of the international classification of diseases (ICD-11)

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