This chapter discusses the management of coronary artery anomalies in patients without other congenital heart defects. Most coronary artery anomalies in number, origin, and distribution are of intellectual interest only. However, a few are clinically significant because they may result…
Congenital aortic valve disease is one of the more commonly encountered congenital cardiac defects, occurring in 3% to 6% of children born with congenital heart disease. Congenital aortic valve disease primarily manifests as aortic stenosis and tends to be a…
Interrupted aortic arch (IAA) is a rare genetic disorder of the cardiovascular system, present in approximately two cases per 100,000 live births and comprising 1.5% of all cases of congenital heart disease. Its hallmark feature is a lack of luminal…
Truncus Arteriosus Truncus arteriosus is a relatively rare congenital heart defect with a single vascular trunk arising from the heart, giving origin to the true pulmonary arteries, aorta, coronary arteries, and brachiocephalic vessels. The lesion accounts for approximately 0.4% to…
Pulmonary Atresia with Ventricular Septal Defect Pulmonary atresia with ventricular septal defect (PA/VSD) is a congenital cardiac malformation characterized by discontinuity of blood flow from the right ventricle to the pulmonary arteries, a ventricular septal defect (VSD) resulting from anterior…
History Stensen, in 1672, described for the first time the anatomic features of what is now termed tetralogy of Fallot (TOF). In 1888, Etienne-Louis Arthur Fallot published his findings describing the four features of the congenital cardiac anomaly that bears…
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart defect occurring at a rate of 4 to 10 per 100,000 live births. This malformation is characterized by a variably sized right ventricle that has no exit, failing…
Ventricular Septal Defect A ventricular septal defect (VSD) is a hole between the left and right ventricles. A VSD may occur as an isolated anomaly or with a wide variety of intracardiac anomalies, such as tetralogy of Fallot or transposition…
Atrioventricular (AV) canal defects include a spectrum of lesions in which the common etiology appears to be abnormal development of the endocardial cushions, resulting in a defect in the AV septum and AV valves. This group of lesions forms approximately…
Etiology Normal embryologic development of the pulmonary venous system involves creation of a connection between the left atrium and the pulmonary venous plexus, and subsequent regression of systemic-to-pulmonary venous connections. Inappropriate connection of the pulmonary venous system to the systemic…