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Congenital heart defects (CHDs) are structural abnormalities of the heart or blood vessels that occur while cardiac structures are being formed in utero [...]

Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic hemodynamic abnormalities resulting from multiple defects in patients with cardiac malposition should be the focus of treatment. At the time of the first presentation, treating the pathophysiologic aberrations caused by the defect complex, whether it is by improving the pulmonary blood flow or restricting it, is the first step. Some patients with simpler or single defects are amenable to surgical or transcatheter therapy and should be treated accordingly. Other associated defects should also be treated appropriately. Biventricular or univentricular repair dependent on the patient’s cardiac structure should be planned. Complications in-between Fontan stages and after conclusion of Fontan surgery may occur and should be promptly diagnosed and addressed accordingly. Several other cardiac abnormalities unrelated to the initially identified heart defects may manifest in adulthood, and they should also be treated.

The last five decades have witnessed an inordinate number of advances in the diagnosis and management of congenital heart defects (CHDs), as reviewed elsewhere [...]

A significant number of patients with dextrocardia and other cardiac malpositions have other congenital heart defects (CHDs). The incidence of CHDs in subjects with cardiac malpositions is significantly greater than that in normal children, and the prevalence varies with the associated visceroatrial situs. The most useful approach to diagnosis is segmental analysis. Firstly, dextroposition should be excluded. In segmental analysis, the visceroatrial situs, ventricular location, status of atrioventricular connections, the great artery relationship, and conotruncal relationship are determined with the use of electrocardiogram (ECG), chest X-ray, and echocardiographic studies, and, when necessary, other imaging studies, including angiography. Following identification of the afore-mentioned segments, the associated defects in the atrial and ventricular septae, valvar and vascular stenosis or atresia may be determined by a review of the historical information, physical examination, and analysis of chest roentgenogram, ECG, and echocardiographic studies. Along the way, a pictorial rendition of the terminology and diagnosis of cardiac malpositions is undertaken.

Double-inlet left ventricle (DILV) is most frequent among univentricular atrioventricular connections. In DILV, there is a single functioning ventricle, most commonly with left ventricular structure. This chamber receives both atrioventricular valves and is connected to an outlet chamber with morphologic features of the right ventricle. The great vessels are often transposed, and pulmonary stenosis is seen in two-thirds of patients. The anatomy and pathophysiology can be defined by echo-Doppler studies with a rare need for other imaging studies. The management is mostly related to the nature of associated heart defects and the degree of pathophysiological abnormality. When the infants present initially, treatment to address the hemodynamic issues is undertaken. Subsequently, these babies need staged total cavo-pulmonary connection, i.e., the Fontan procedure which is undertaken in three stages; these stages are described in this review. The existence of inter-stage mortality and post-Fontan complications is recognized and was reviewed. The paper concludes that DILV can be successfully diagnosed with echo-Doppler studies and this heart anomaly can be effectively treated with the currently prevailing medical, catheter interventional, and surgical treatment practices.

The last five decades have witnessed an inordinate number of advances in the diagnosis and management of congenital heart defects (CHDs), as reviewed elsewhere [...]

Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended.

In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.

This paper describes various statistical methods used by the author during multiple studies conducted by the author. Initially, the data were scrutinized to ensure normal distribution, and expressed as mean ± standard deviation (SD) or standard error of mean (SEM) for normally distributed variables. Medians and ranges were given for the data with skewed distribution. Two tailed, paired t tests or independent sample t tests (analysis of variance) were used for normally distributed data, while non-parametric chi-square or similar other tests were utilized for data with skewed distribution. Statistical significance was set at a p value of < 0.05. Bonferroni correction was applied when the study involves multiple comparisons. A number of other statistical methods used during these studies were also discussed. Finally, special methods used in evaluating aortic remodeling subsequent to balloon angioplasty of native aortic coarctation were reviewed.

Several devices have been designed and tried over the years to percutaneously close atrial septal defects (ASDs). Most of the devices were first experimented in animal models with subsequent clinical testing in human subjects. Some devices were discontinued or withdrawn from further clinical use for varied reasons and other devices received Food and Drug Administration (FDA) approval with consequent continued usage. The outcomes of both discontinued and currently used devices was presented in some detail. The results of device implantation are generally good when appropriate care and precautions are undertaken. At this time, Amplatzer Septal Occluder is most frequently utilized device for occlusion of secundum ASD around the world.

The author’s contribution up to 1990 was reviewed in part I and the echo contributions from 1991 to 2020 will be reviewed in part II. These include defining the relationship between the quantity of shunt across the atrial septal defect (ASD) and the diameter of ASD by echo and angio on the one side and the stretched diameter of the ASD on the other; echocardiographic assessment of balloon-stretched diameter of secundum ASDs; development of echocardiographic predictors of accomplishment of percutaneous closure of ASDs with the buttoned device, highlighting limitations of echocardiography in comprehensive assessment of mixed type of total anomalous pulmonary venous connection; description of follow-up echocardiographic results of transcatheter closure of ASD with buttoned device; review of ultrasound studies; depiction of collaborative echocardiographic and Doppler studies; echocardiographic appraisal of the outcome of balloon pulmonary valvuloplasty; editorials; ventricular septal aneurysm causing pulmonary outflow tract obstruction in the morphologic left ventricle in corrected transposition of the great arteries; dependability of echocardiographic assessment of angiographic minimal diameter of the ductus; occurrence of supravalvular pulmonary artery stenosis after a Nuss procedure; echocardiographic assessment of neonates who were suspected of having heart disease; role of echocardiographic studies in the appraisal of patent ductus arteriosus in the premature babies; and the role of pressure recovery in explaining differences between simultaneously measured Doppler and cardiac catheterization pressure gradients across outflow tract stenotic lesions.

The author has undertaken multiple echocardiographic studies during his academic career; most of these were published in peer-reviewed journals. These studies include an evaluation of the role of echocardiography in the estimation of left-to-right shunt in isolated ventricular septal defects, an examination of the utility of contrast echocardiography in the diagnosis of anomalous connection of the right superior vena cava to the left atrium, a description of pitfalls in M-mode echocardiographic assessment of the aortic root in left ventricular hypoplasia syndromes, reviews of echocardiographic evaluation of left ventricular function, study of the role of contrast echocardiography in the evaluation of hypoxemia following open heart surgery, a quantification of left ventricular muscle mass by m-mode echocardiography in children, an examination of race and sex related differences in echocardiographic measurements in children, study of cardiac size and function in patients with sickle cell disease, an examination of afterload reduction in the management of primary myocardial disease, study of the utility of echo-Doppler studies in the evaluation of the results of balloon pulmonary valvuloplasty, study of the usefulness of Doppler in the prediction of pressure gradients in valvar pulmonary stenosis, a review of Doppler echocardiography in noninvasive diagnoses of heart disease, echo-Doppler studies of the evaluation of the results of balloon angioplasty of aortic coarctation, study of the value of Doppler in the prediction of pressure gradients across coarctation of the aorta, and a characterization of foramen ovale and transatrial Doppler velocity patterns in the normal fetus.

In this review management of the most common cyanotic congenital heart defects (CHDs) was discussed; the management of acyanotic CHD was reviewed in Part I of this series. While the need for intervention in acyanotic CHD is by and large determined by the severity of the lesion, most cyanotic CHDs require intervention, mostly by surgery. Different types of tetralogy of Fallot require different types of total surgical corrective procedures, and some may require initial palliation, mainly by modified Blalock–Taussig shunts. Babies with transposition of the great arteries with an intact ventricular septum as well as those with ventricular septal defects (VSD) need an arterial switch (Jatene) procedure while those with both VSD and pulmonary stenosis should be addressed by Rastelli procedure. These procedures may need to be preceded by prostaglandin infusion and/or balloon atrial septostomy in some babies. Infants with tricuspid atresia require initial palliation either with a modified Blalock–Taussig shunt or banding of the pulmonary artery and subsequent staged Fontan (bidirectional Glenn and fenestrated Fontan with extra-cardiac conduit). Neonates with total anomalous pulmonary venous connection are managed by anastomosis of the common pulmonary vein with the left atrium either electively in non-obstructed types or as an emergency procedure in the obstructed types. Babies with truncus arteriosus are treated by surgical closure of VSD along with right ventricle to pulmonary artery conduit. The other defects, namely, hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, double-outlet right ventricle, double-inlet left ventricle and univentricular hearts largely require multistage surgical correction. The currently existing medical, trans-catheter and surgical techniques to manage cyanotic CHD are safe and effective and can be performed at a relatively low risk.

Since the description of surgery for patent ductus arteriosus in late 1930s, an innumerable number of advances have taken place in the management of congenital heart defects (CHDs). In this review the current status of treatment of seven of the most common acyanotic CHDs was reviewed. The discussion included indications for, and timing of, intervention and methods of intervention. The indications are, by and large, determined by the severity of the lesion. Pressure gradients in obstructive lesions and the magnitude of the shunt in left-to-right shunt lesions are used to assess the severity of the lesion. The timing of intervention is different for each lesion and largely dependent upon when the criteria for indications for intervention were met. Appropriate medical management is necessary in most patients. Trans-catheter methods are preferable in some defects while surgery is a better option in some other defects. The currently available medical, trans-catheter, and surgical methods to treat acyanotic CHD are feasible, safe, and effective.

In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation. [ABSTRACT FROM AUTHOR]