Abstract
Background
Management and outcomes amongst children with congenital heart defects (CHD) are relatively heterogeneous. Traditionally, the highest-risk conditions and procedures have received the most attention, with less focus on outcome for less severe CHDs.
Objectives
Evaluate the management of children born with certain less severe CHDs across Europe.
Methods
This population-based linkage cohort study included children diagnosed with ventricular septal defect (VSD), atrial septal defect (ASD), pulmonary valve stenosis (PS), and patent arterial duct (PDA) from nine European regions. Data on surgery/intervention and survival from 1995 to the age of 10 years or until the end of 2014 were collected through electronic linkage to mortality and hospital databases. Kaplan-Meier survival estimates and meta-analyses were used to analyze the proportion of children undergoing surgery/intervention and survival rates.
Results
The study included 20,608 children with the less severe CHDs. Surgical correction rates for the anomaly within the first year of life varied significantly, particularly for VSD. The median age for initial surgery/intervention was consistent for VSD but varied for other conditions. Most conditions were corrected in a single procedure, except for PS, which required a median of 1.7 (95 % CI; 1.4–2.0) interventions. Postoperative mortality was highest for neonates undergoing VSD surgery/intervention, 8.7 (95 % CI; 4.0–15.8) deaths per 100 surgeries. The overall 5-year survival rate for all conditions was approximately 98 %.
Conclusion
There was considerable variation in the management of less severe CHDs across Europe, particularly in the timing of surgical interventions. Despite these differences, the overall 5-year survival rate is comparable and high.
Highlights
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Wide European variation in surgery timing for minor congenital heart defects
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Despite variation in timing, no observable differences in mortality
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Single intervention sufficed for most, but pulmonary valve stenosis often required more.
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Neonatal postoperative mortality highest for ventricular septal defect surgeries
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Overall 5-year survival rates for all conditions exceeded 98 %.
1
Introduction
Congenital heart defects represent the most common type of congenital anomalies [ ]. These anomalies range from minor defects, which may require minimal or no intervention, to severe malformations that necessitate complex surgical correction shortly after birth [ ]. The management of and outcomes amongst children with congenital heart defects is relatively heterogeneous, something that has been attributed to differences in both case-mix between hospitals and dissimilarities with respect to clinical management amongst institutions [ , ]. Traditionally, the highest-risk conditions and procedures have received most attention [ , , ]. Interestingly, a recent study from the United States found not only significant variation in mortality and morbidity but also similar variation for both high and low risk children [ ]: the authors reported a ≈ 3-fold variation in mortality and morbidity rates across hospitals for both low-risk and high-risk cardiac procedures in infants and children.
This study, as part of the EUROlinkCAT project [ ], evaluated timing of the first cardiac surgical intervention, the number of cardiac surgical interventions, postoperative mortality, and survival in children born with less severe congenital heart disease across nine European regions from six countries for the first 10 years of life. We aimed to provide a clearer picture of the clinical management of less severe congenital heart disease and its outcomes to support future guidelines and improve care for this large patient population.
2
Materials and methods
The European Surveillance of Congenital Anomalies (EUROCAT) registry provided comprehensive data on congenital anomalies across Europe since the start of 1995. Established to facilitate the epidemiological surveillance of congenital anomalies, EUROCAT aims to detect and monitor trends, facilitate research to identify causes of congenital anomalies, and ultimately prevent congenital anomalies. This study is part of the broader EUROlinkCAT project, which gathered comprehensive data concerning the morbidity and mortality of children diagnosed with congenital anomalies. Briefly, EUROlinkCAT did this by linking children recorded in the EUROCAT registries with national or regional population healthcare databases with information on mortality, hospital stays (incl. surgery), prescription, and education databases [ ].
2.1
Study cohort
The study included children recorded in the EUROCAT registries diagnosed with specific ICD-10 CHD codes. During the study, Finland and Italy utilized ICD-9 codes, which were subsequently aligned with the ICD-10 system for consistency (refer to [ , ] for a detailed mapping). The study excluded cases of patent arterial duct and peripheral pulmonary branch stenosis in preterm infants (<37 weeks gestation) and persistent foramen ovale across all gestational ages. The inclusion criteria were narrowed to children with isolated congenital heart disease, excluding those with associated non-cardiac anomalies and/or genetic conditions. A child can potentially have more than one CHD diagnosis. As both ventricular septal defect and atrial septal defect are frequently associated with other congenital heart diseases, we created two specific categories excluding cases of severe congenital heart disease (see below).
2.2
Included diagnosis
Ventricular septal defect (Q21.0), atrial septal defect (Q21.1), pulmonary valve stenosis (Q22.1), and patent arterial duct (Q25.0) if gestation age > 37 weeks. Ventricular septal defect, excluding severe congenital heart disease: all types of ventricular septal defect either isolated or associated with other congenital heart disease diagnoses, but excluding severe congenital heart disease. Atrial septal defect, excluding severe congenital heart disease: all types of atrial septal defect either isolated or associated with other congenital heart disease diagnoses, but excluding severe congenital heart disease.
2.3
Excluded diagnosis
Severe congenital heart disease classification includes a range of diagnoses known for their high perinatal mortality rates, as identified in prior research [ , ]. These include all diagnoses of common arterial truncus, double-outlet right ventricle, transposition of the great arteries, single ventricle, atrioventricular septal defect, tetralogy of Fallot, pulmonary atresia, tricuspid atresia or stenosis, Ebstein anomaly, hypoplastic right heart, aortic valve atresia or stenosis, mitral valve anomalies, hypoplastic left heart, coarctation of the aorta, interrupted aortic arch, and total anomalous pulmonary venous return.
2.4
Data collection
Survival data and records of surgical interventions up to the age of ten or until the end of 2014, whichever was sooner, were compiled through electronic links to mortality and hospital records, as outlined in previous publications [ ].
2.5
Surgical data coding
The coding of surgical procedures adhered to the respective national health system’s standards, with variations across countries in the use of ICD-9-CM, OPCS-4, and NCSP coding systems. The analysis included corrective procedures but excluded diagnostic procedures (for details, please see reference [ ]), i.e., catheter closure of a VSD will be included, whereas a diagnostic right heart catheterization will be excluded. In the paper, we have used the word surgery to encompass both surgical and catheter based intervention for a cardiac defect.
2.6
Statistical approach
The study applied Kaplan-Meier survival analysis to estimate the proportion of children undergoing surgery, adjusting for censoring due to death, emigration, or study endpoint. Confidence intervals for these estimates were calculated using Stata (version 16), with pooled estimates derived from random effects inverse-variance meta-analyses. For details on statistical methods, please refer to [ ].
2.7
Surgical interventions and outcomes
The distribution of the number of procedures and the age at first surgery were skewed and are therefore reported as medians and interquartile ranges. Meta-analytic techniques were applied to aggregate data across registries, as previously described [ ].
2.8
Confidentiality of data
In compliance with registry policies, the release of data involving small numbers (<5) was restricted for some registries.
2.9
Mortality and survival analysis
The study classified postoperative mortality and survival analysis according to specific age groups for each CHD category. Due to limited data in older age groups, the focus was primarily on the first five years of life.
2.10
Ethical approval
The EUROCAT network registries operate in accordance with established ethical approvals and protocols, ensuring the systematic collection, surveillance, and secure transmission of anonymized data to the central EUROCAT database. Each registry, adhering to national regulations, provided evidence of ethical compliance, which was documented within the EUROlinkCAT ethical framework. Ethical approval for the central data repository at Ulster University was granted (reference: FCNUR-21-060). Importantly, institutional review board approval was not required due to the study’s observational design. Local registries obtain parental consent for the inclusion of infants with congenital anomalies, as stipulated by respective national laws. Further details can be found in the published paper on ethics and legal requirements for data linkage [ ].
3
Results
3.1
Population characteristics
We obtained data from nine EUROCAT registries from six countries for children born between 1995 and 2014. Data were available for 20,608 children born with either ventricular septal defect, atrial septal defect, pulmonary valve stenosis, or patent arterial duct ( Table 1 ).
| CHD type | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Region | Birth years | PDA in term infants | ASD, excluding sCHD | VSD, excluding sCHD | PS | ||||
| Number | Percentage surgery in 1st year | Number | Percentage surgery in 1st year | Number | Percentage surgery in 1st year | Number | Percentage surgery in 1st year | ||
| Denmark, Funen | 1995–2014 | 22 | 23 (10–46) | 102 | 12 (7–20) | 389 | 10 (7–13) | 54 | 25 (15–38) |
| Finland | 1997–2014 | 14 | 21 (7–53) | 1827 | 12 (11–14) | 9040 | 5 (5–6) | 685 | 25 (22–28) |
| Italy, Tuscany | 2005–2014 | 33 | 12 (5–29) | 146 | 10 (6–16) | 966 | 7 (5–8) | 54 | 52 (39–66) |
| Italy, Emilia Romagna | 2008–2014 | 36 | 17 (8–34) | 260 | 20 (16–25) | 866 | 14 (12–16) | 53 | 75 (63–86) |
| Spain, Valencian Region | 2010–2014 | 138 | 7 (4–13) | 192 | 9 (6–15) | 618 | 12 (10–15) | 64 | 57 (45–70) |
| UK, Wales | 1998–2014 | 208 | 11 (7–16) | 697 | 15 (13–18) | 1706 | 11 (9–12) | 434 | 27 (23−31) |
| UK, Thames Valley | 2005–2013 | 5 | 20 (3–80) | 209 | 19 (15–26) | 256 | 25 (20−30) | 53 | 42 (30–57) |
| UK,Wessex | 2004–2014 | a | 55 | 35 (24–49) | 152 | 54 (46–63) | 63 | 68 (56–79) | |
| UK, East Midlands and South Yorkshire | 2003–2012 | 174 | 25 (20–33) | 278 | 28 (23–34) | 581 | 32 (28–36) | 156 | 70 (63–77) |
| Total | 630 | 3766 | 14,574 | 1616 | |||||
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