Ebstein Anomaly
Definition and Morphology Ebstein anomaly (EA) is a rare malformation of the tricuspid valve (TV) and right ventricle (RV). The anatomic and pathophysiologic characteristics are variable and lead to a…
Vascular Rings, Pulmonary Slings, and Other Vascular Abnormalities
The aortic arch, branch pulmonary arteries, and the ductus arteriosus have a close spatial relationship to the airways and esophagus. Abnormalities of the position or course of these vascular structures…
Atrioventricular Septal Defects
Definition and Morphology Definitions Atrioventricular septal defects (AVSDs) encompass a spectrum of cardiac anomalies. The hallmark feature is a five-leaflet atrioventricular (AV) valve with a common AV annulus that guards…
Valvular Aortic Stenosis
Left ventricular outflow tract obstruction accounts for 5% to 10% of all congenital heart defects and may be due to stenosis at a valvular, subvalvular, or supravalvular level. The obstruction…
Aortic Coarctation and Interrupted Aortic Arch
Coarctation of the Aorta Definition and Morphology Coarctation of the aorta (CoA) is a complex cardiovascular disorder, and, as part of a generalized arteriopathy, a lifelong disease that persists after…
Pacemakers and Internal Cardioverter Defibrillators in Adult Congenital Heart Disease
Implantable Cardiac Devices and Congenital Heart Disease Survival of children with congenital heart disease (CHD) continues to improve in lockstep with advances in surgical and medical therapy, and more than…
Ventricular Septal Defect
Definition and Morphology Ventricular septal defects (VSDs) are openings in the ventricular septum and occur both in isolation and in conjunction with other cardiac defects. The classification of VSDs is…
Atrial Septal Defect (Interatrial Communication)
Definition and Morphology An atrial septal defect (ASD) is a direct communication between the cavities of the atrial chambers that permits shunting of blood. In the normal heart the true…
Infective Endocarditis
In 1885, Sir William Osler summarized beautifully the main aspects of clinical infective endocarditis (IE) in the Gulstonian Lectures delivered at the Royal College of Physicians of London. Despite all…
Patent Ductus Arteriosus and Aortopulmonary Window
Patent Ductus Arteriosus Embryology The patent ductus arteriosus (PDA) is a remnant of the distal left sixth aortic arch, which connects the proximal descending aorta to the main pulmonary artery…
