Myocardial Diseases Cardiomyopathies include various diseases of the myocardium, often chronic or progressive, and associated with cardiac dysfunction. These are rare but serious disorders; only 25% of children survive more than 5 years after the onset of symptoms. Although our…
A large number of syndromes, dysplasias, and chromosomal anomalies are associated with congenital or acquired cardiac and vascular disease. This chapter discusses the cardiovascular features of some of the more commonly encountered lesions. A more extensive list is provided in…
The author would like to acknowledge Frandics Chan, MD, PhD, for his contributions to prior editions of this chapter. Coronary artery disease in children can be acquired, but the majority of anomalies are congenital. Among acquired coronary abnormalities, Kawasaki disease…
This chapter covers congenital anomalies of the great arteries that result in a vascular ring or sling. Overall, anomalies of the aortic arch and the cervical vessels are relatively common, with an estimated prevalence of 0.5% to 3%. They can…
Conotruncal anomalies are a group of congenital heart defects involving the outflow tracts of the heart. The conotruncal anomalies include tetralogy of Fallot (TOF), transposition of the great arteries (TGA), double-outlet ventricles, truncus arteriosus, and interrupted aortic arch (IAA) type…
Imaging of the Right Heart The algorithms used for left ventricular assessment cannot always be applied to the right ventricle (RV), which differs in morphology, shape, and function. The RV has a complex geometrical shape with a thinner and more…
Hypoplastic Left Heart Syndrome Overview. Hypoplastic left heart syndrome (HLHS) is a spectrum of disease characterized by underdevelopment of the left ventricle with obstruction or atresia of ventricular inflow and outflow. HLHS accounts for approximately 2% to 3% of all…
Atrial Septal Defect Overview. An atrial septal defect (ASD) is a defect in the atrial septum that allows communication between the right atrium (RA) and left atrium (LA). Isolated ASDs account for 6% to 10% of all congenital heart disease.…
Partial Anomalous Pulmonary Venous Connection and Scimitar Syndrome Overview. Partial anomalous pulmonary venous connection (PAPVC) is present when one or more pulmonary veins drain into a systemic vein. Because a single anomalous connection may be unrecognized, the incidence is difficult…
Introduction Congenital abnormalities of the heart are the most common birth defects, occurring in approximately 8 of every 1,000 newborns. Approximately 25% of those in whom an abnormality is identified have critical congenital heart disease (CHD) with risk for significant…