Absent Pulmonary Valve Syndrome
Definition and Morphology Absent pulmonary valve syndrome is a rare congenital cardiac malformation that was first described anatomically by Chevers in 1847. The first clinical case report was published in…
Double-Chambered Right Ventricle
Definition and Morphology Double-chambered right ventricle (DCRV) is characterized by anomalous or hypertrophied muscle bundles, which cause a form of subvalvar right ventricular outflow tract (RVOT) obstruction, dividing the right…
Pulmonary Stenosis
Definition and Morphology Pulmonary valvular stenosis is usually an isolated congenital anomaly and occurs in 7% to 12% of patients with congenital heart disease. It can sometimes be associated with…
Tricuspid Regurgitation and Stenosis
Definitions and Morphology Isolated congenital anomalies of the tricuspid valve (TV) are rare structural malformations that involve one or more components of the TV apparatus and result more commonly in…
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…
