Type of ASD
Mechanism of ASD
Occurrence of the lesion/clinical outcome
Associated lesions
ASD secundum (ASD II)
Failure in pulling down of the septum primum and deficient septum secundum
The most common type of ASD: 70 % of all ASDs are ASD II; 65–70 % of ASD II are female; about half of them are closed spontaneously; chance of spontaneous closure is more in smaller-size defects and in less than 1 year of age; increasing age and larger defects, less chance for spontaneous closure
Mitral valve prolapse
Mitral valve stenosis
Pulmonary stenosis
ASD primum (ASD I)
If the ostium primum is not closed by the septum primum, ASD I results; ASD I is located close to the atrioventricular valves and is often associated with an atrioventricular septal defect discussed in Chap. 18 – AV Septal Defects
50 % of ASD I patients are female; usually, surgical treatment is often mandatory for closure of ASD I, because there is very little chance for spontaneous closure
Cleft mitral valve (always)
Inlet ventricular septal defect
Septal aneurysms
Sinus venosus type ASD (SVASD)
SVASD is an unusual type of ASD, with two common locations:
If located superiorly, it is near the entry of SVC
If located inferiorly, it is near the entry of IVC
It is often associated with anomalous attachment of venae cavae and pulmonary veins
Often, due to these anatomic locations, it is discussed under partial anomalous pulmonary venous drainage
40–50 % of SVASD patients are female. There is usually no spontaneous closure; surgical treatment is often mandatory
Partial anomalous venous return
Overriding superior vena cava
Coronary sinus septal defect (CSSD)
CSSD occurs if the delicate tissue separating the coronary sinus from the left atrium is not completed; the result is blood shunting through the defect and the orifice of the coronary sinus
Uncommon type of ASD
Unroofed coronary sinus
Left superior vena cava persistence
Partial/total anomalous venous return
Persistent foramen ovale (PFO)
The pathologic defect is the same as the pathology if fetal circulation persists (persistent fetal circulation, PFC) which is associated with increased pressure in the right side which overcomes the left side
PFO, as an independent lesion, is present in as much as 25 % of general population; so, its occurrence should be considered independently from defects in the interatrial septum
Similar to PFO
Pathophysiology of ASD
Due to ASD, during the early course of the disease, oxygenated blood flows from left to right, leading to the following events (Oliver et al. 2002; Azarbal et al. 2005; Tobis and Azarbal 2005; Warnes et al. 2008; Geva et al. 2014; Zvaigzne et al. 2014):
In nearly all of the ASD patients, there are varying degrees of interatrial shunt; if the pressure is higher in one side, the blood would be shunted from that side to the either side.
Often, there is a left-to-right shunt in ASD patients; the severity of the shunt and its direction depends on two main factors: size of the defect in the interatrial septum and left and right atrial pressures and the relationship between them.
In ASD secundum, if the defect is less than 10 mm, the amount of shunt is usually negligible, and the severity of right heart overload and pulmonary hypertension is very low.
If the defect is large enough to induce considerable shunt flow, then, the lungs are overflowed due to recirculation of oxygenated blood to the lungs.
Increased blood volume load to the lungs often leads to enlargement of the right heart chambers (right atrium and ventricle) often associated with impaired function of the right atrium; also, the pulmonary and right heart vascular system (both arteries and veins) are enlarged, due to pulmonary overflow.
Over time, pulmonary overflow, pulmonary vascular bed remodeling, ventricular remodeling, and trophic changes in the right and left ventricles lead to pulmonary hypertension with different severities; the incidence of right heart failure and pulmonary vascular disease is more in female patients compared with male patients and, also, in untreated adults.
Meanwhile, decreased blood flow through the left heart leads to shrinkage or compromised growth of the left ventricle and aorta; the final result could be diminished systemic output and, finally, left ventricular systolic dysfunction.
If pulmonary hypertension persists and, also, the process of shrinkage in the aorta and left ventricle continues, the final poor event, which is Eisenmenger syndrome, ensues presented as increased pulmonary pressure over the systemic pressure accompanied with different degrees of right heart failure; a full discussion of right heart failure could be found in Chap. 33 – Right Heart Failure.
Often, in the course of the disease, exercise intolerance occurs due to impaired hemodynamics, though it is not a common event during the early stages of the disease; however, with advanced age, intolerance increases insidiously.
Often, enlargement of the right heart, especially the right atrium, is associated with a range of arrhythmias which are discussed more in Chap. 37 – Arrhythmias and Their Management.
For prevention of the disastrous final outcomes, treatment is necessary: smaller ASDs impose less burden on the heart and might be closed spontaneously; however, larger ASDs cause significant burden on the heart leading to unwanted consequences which mandate treatment.
Some neurologic complications might occur during the process of the disease due to the abnormal shunts; for example, paradoxical emboli or rare desaturation episodes may lead to aura, migraine headaches, or even transient ischemic events in the central nervous system.
Diagnosis of ASD
In ASD patients, the process of diagnostic workup should aim mainly the following:
Presence of ASD
Size of ASD
Location of ASD
The effect of ASD shunt on the left and right ventricular function
The effect of ASD shunt on pulmonary circulation
Any possible associated lesions
Clinical Diagnosis of ASD
Clinical presentation and the course of the disease
The majority of the patients are asymptomatic during neonatal and early childhood. Any of the following findings like slow weight gain, tachypnea, or recurrent respiratory infections during infancy should raise suspicion. Most of them are acyanotic; however, very rarely, mild transient cyanosis may happen in the newborn which is due to right-to-left shunt. In physical examination, the precordium is hyperdynamic. Fixed splitting of the second heart sound is heard through respiratory cycles; the severity of pulmonary component of second heart sound (P2) corresponds with the severity of pulmonary hypertension only if it is present. So, the diagnosis in this time domain is usually an incidental finding by echo during routine clinical or paraclinical assessment like looking for the origin of an incidental heart murmur or anything abnormal found in chest X-ray or other studies. Untreated adults have more symptoms and signs, especially related to potential pulmonary hypertension. More explanation regarding adult patients with ASD could be found in Chap. 14 – Anesthetic Management of Adults with Congenital Heart Disease (Andrews et al. 2002; Lammers et al. 2005; Geva et al. 2014; Zvaigzne et al. 2014).
In a considerable number of patients with ASD secundum, the chance of spontaneous closure is high; in fact, the chance of spontaneous closure is higher in those patients with smaller-size defects and in less than 1 year of age; but, increasing age and larger defects are not in favor of spontaneous defect closure. On the other site, sinus venosus ASD and ASD primum nearly always need surgical treatment and have significant hemodynamic consequences. Interestingly, more than one-fourth of all adult patients with congenital heart defects are ASD cases, and among them, about 75 % are ASD secundum (Warnes et al. 2008; Vasquez and Lasala 2013).
Exercise intolerance
This is an uncommon finding in young ASD patients. However, with increasing age, in those who have been untreated, the frequency of exercise intolerance increases surreptitiously due to aggravation of pulmonary vascular function. If ASD secundum remains unrepaired, exercise capacity will decrease as much as 50–60 % of the predicted values (Geva et al. 2014).
Pulmonary hypertension
It is not a common finding in neonates and young children; but may be much more frequent in untreated adults. Increasing age and female gender are two main predisposing factors for the occurrence of pulmonary hypertension in untreated ASD; Down syndrome, sleep apnea, and pulmonary vascular embolic events are other risk factors. Eisenmenger syndrome is seen in 5–10 % of untreated adults (Rosas and Attie 2007; Warnes et al. 2008).
Electrocardiography
The main ECG findings include:
Tall P wave due to right atrial enlargement; inverted P waves in inferior leads suggest sinus venosus ASD.
First-degree AV block may be seen; also, right bundle branch block (usually incomplete form) may be seen especially in untreated adults.
Right axis deviation – if leftward or left superior QRS axis deviation is seen, one should sought for ASD primum.
Rhythms other than sinus rhythm are not common; however, atrial fibrillation or flutter may occur in adult patients with prolonged disease leading to right atrium enlargement, often occurring after 40 years.
Hypertrophy of the right ventricle (presented in ECG by RSR’ pattern in right precordial leads) may be seen due to pulmonary hypertension (Webb and Gatzoulis 2006; Lam and Friedman 2011; Geva et al. 2014; Zvaigzne et al. 2014) (Fig. 18.1).
Fig. 18.1
Atrial septal defect. The RSR’ pattern in lead V1 and right axis deviation are typical characteristics (Courtesy of Dr Majid Haghjoo and Dr Mohammadrafie Khorgami)
CXR
CXR is often abnormal in ASD; however, normal CXR does not rule out ASD. These are the most common findings in CXR of ASD patients:
Cardiomegaly, which is mainly due to dilation of right-sided chambers, best seen in lateral views; however, in ASD primum, dilation of left-sided chambers may lead to cardiomegaly which may be better seen in lateral views.
Pulmonary artery trunk and its perfusion domain are enlarged and plethoric; however, a discordance between the main pulmonary artery body and lung fields, leading to a normal appearance of lung fields in the presence of enlarged pulmonary artery trunk, may be indicative of pulmonary vascular obstructive lesion.
Imaging Techniques in Diagnosis of ASD
Diagnosis of ASD should be done and/or confirmed through imaging techniques in such a way to demonstrate shunting across the interatrial septal defect; also, any possible right ventricle overload or associated diseases should be detected and diagnosed. These techniques include mainly echocardiography and, also, cardiac CT, cardiac MR, and catheterization (Warnes et al. 2008).
Echocardiography
There is no doubt that two-dimensional transthoracic echocardiography with Doppler is considered as the cornerstone for evaluation of ASD, though not all ASDs can be visualized with transthoracic echocardiography. Echocardiography is the most common diagnostic modality used for all types of ASD both during primary assessment of the defect and during follow-up visits (Kharouf et al. 2008). The main elements that should be considered in a comprehensive echocardiographic evaluation for ASD mainly include:
Visualization of ASD, with definite characterization of its size (as much as possible)
Determining the direction of interatrial flow
Right heart examination
Pulmonary artery pressure measurement
Assessment and estimation of the pulmonary/systemic flow ratio
Assessment of the associated abnormalities
Initial assessment of ASD during echocardiography should specifically include the following steps:
- 1.
Assessment of the ASD defect including location, size, number, shunting, gradient (peak/mean), and rims
- 2.
Right ventricle size and function
- 3.
Right ventricle systolic pressure
- 4.
Pulmonary artery pressure
- 5.
Septal motion/curvature
- 6.
Pulmonary veins
- 7.
Pulmonary valve
- 8.
Possible mitral valve prolapse (MVP)
- 9.
Left ventricle size and function
- 10.
Left atrium size
- 11.
Possibility of aortic regurgitation and pulmonary insufficiency
Post-intervention assessment of ASD should focus on the following steps:
- 1.
Any possible residual leak
- 2.
Right ventricle size/function
- 3.
Septal motion/curvature
- 4.
LV size/function
- 5.
Possible mitral valve prolapse (MVP)
- 6.
Pulmonary valve
- 7.
Right ventricle systolic pressure
- 8.
Pulmonary artery pressure
- 9.
Pericardial effusion
Common views for detection of ASD:
Apical four-chamber view.
Parasternal short axis view.
Subcostal views.
Also, note that not all ASDs can be detected with transthoracic echocardiography.
Other Imaging Studies
Cardiac MR
It allows both anatomic assessment and, also, the hemodynamic effects of interatrial defects, and it has a great application in sinus venosus ASD. Also, cardiac MR is considered the gold standard in the assessment of RV volume and function and, also, the pulmonary artery and veins. Cardiac MR is the most accurate and the fastest data acquisition modality. However, this technology is not usually used before treatment of ASD secundum and has limited application before treatment of ASD primum (Webb and Gatzoulis 2006; Kharouf et al. 2008; Warnes et al. 2008; Geva et al. 2014).
Cardiac CT
Especially when using high-resolution cardiac CT, many great data could be acquired; however, the data gained from cardiac CT are not significantly more than the data gained from cardiac MR; also, the risk of radiation in cardiac CT should be considered.
Catheterization studies
It is not usually indicated to use cardiac catheterization in ASD unless one of the following three (Warnes et al. 2008; Geva et al. 2014):
In ASDs which are going to be closed with percutaneous devices, i.e., ASD secundum
To assess any associated anomaly not diagnosed with other noninvasive imaging modalities
For adult ASD to assessment of the coronary system
Treatment
Regardless of age, ASD closure leads to improvements in the course of the disease, both the patient symptoms and improvement in pulmonary vascular disease, pulmonary arterial pressure, ventricular remodeling, and chamber size. Time of repair depends mainly on the type and size of defect, age of the patient, associated symptoms, associated anomalies, and a number of other concomitant factors; however, if treatment is delayed, clinical parameters aggravate; so, the sooner treatment is done, the better the general outcome and life expectancy will be. Also, surgical closure, if performed before 25 years old, usually leads to normal life span. On the other side, if ASD leads to severe irreversible PAH and no evidence of a left-to-right shunt, there is no evidence in favor of ASD closure (Rosas and Attie 2007; Engelfriet et al. 2008; Warnes et al. 2008; Yalonetsky and Lorber 2009; Humenberger et al. 2011; Geva et al. 2014).
Percutaneous closure of ASD secundum with sufficient rims is the preferred approach; cardiac MR or cardiac catheterization is used for full assessment of ASD secundum and any potential associated anomaly before repair. However, surgical closure is the only treatment in ASD primum and sinus venosus ASDs. Also, surgical closure of ASD secundum is considered when surgical repair/replacement of a tricuspid valve is planned concomitantly or the anatomy of ASD secundum is not appropriate for deploying a percutaneous device (Warnes et al. 2008). In patients with a diagnosis of ASD primum or sinus venosus ASD (confirmed by echocardiography), no more sophisticated imaging modality is usually needed before surgery (Rigatelli et al. 2007; Kharouf et al. 2008; Warnes et al. 2008; Yalonetsky and Lorber 2009; Humenberger et al. 2011; Geva et al. 2014).
According to the “ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease,” ASD closure in adults (percutaneously or surgically) may be considered if the patient meets the following criteria (Level of Evidence: C):
Net left-to-right shunting.
Pulmonary artery pressure two-thirds of the systemic pressure or less than that.
Pulmonary vascular resistance less than two-thirds of the systemic vascular resistance or it responds to pulmonary vasodilator therapy or test occlusion of the defect (Warnes et al. 2008).
Anesthesia for ASD Treatment
Anesthesia for surgical treatment
Using anesthetic drugs with both hemodynamic stability and, also, the capacity to do fast-track extubation seems logic. Also, it is recommended to have an arterial line, a central venous line, and TEE monitoring. Usually, pulmonary artery catheter is not recommended, since it does not increase any data more than TEE. Antibiotic prophylaxis should be considered; Chap. 4 describes fully the antibiotic regime.
Anesthesia for interventional treatment
(device closure of ASD): although sedation could be used, often general anesthesia and tracheal intubation are preferred since TEE is almost always used for defining the correct deployment of the device and ruling out any residual defect.
Ventricular Septal Defect
Introduction
Ventricular septal defect usually named “VSD” is among the most common congenital heart diseases; in fact, it is the most common congenital defect at birth. If those defects that are part of other complex congenital heart disease are taken into account, VSD includes up to 40 % of all congenital heart diseases (Hoffman 1995; Roguin et al. 1995; Hoffman et al. 2004; Penny and Vick 2011; Jortveit et al. 2016). It involves the interventricular septum (IVS) and is often an isolated defect; however, it might be associated with other congenital disease(s) or as a component part of a complex congenital heart disease like:
The conotruncal defects (tetralogy of Fallot, transposition of great arteries, congenitally corrected transposition, DORV, DOLV)Stay updated, free articles. Join our Telegram channel
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