Section I Coarctation of the Aorta Definition Coarctation of the aorta is a congenital narrowing of the upper descending thoracic aorta adjacent to the site of attachment of the ductus arteriosus. The aortic lumen may be atretic in the most…
Congenital aortic stenosis is a cardiac anomaly in which narrowing at valvar, subvalvar, supravalvar, or combined (multiple) levels results in a systolic pressure gradient between the inflow portion of the left ventricle (LV) and the aorta beyond the obstruction. A…
Introduction Congenital anomalies of the coronary arteries represent a varied group of lesions. Taken together, they are relatively common, seen in 1% to 5% of the population, depending on the method of detection. Many lesions are incidental findings with little…
Definition Anomalous origin of a pulmonary artery from the ascending aorta is a condition in which the right pulmonary artery (RPA) or rarely, the left pulmonary artery (LPA), arises from the ascending aorta in the presence of separate aortic and…
Definition Aortopulmonary window (APW) is a round, oval, or sometimes spiral opening between the ascending aorta and pulmonary trunk, occurring as a congenital anomaly in hearts with separate aortic and pulmonary valves. This malformation has also been termed aortic septal…
Definition Truncus arteriosus (persistent truncus arteriosus, truncus arteriosus communis, common aorticopulmonary trunk) is a congenital cardiovascular malformation in which one great artery arising from the base of the heart by way of a single semilunar (truncal) valve gives origin to…
Definition Ebstein anomaly is a congenital defect of the tricuspid valve in which origins of the septal or posterior leaflets or both are displaced downward into the right ventricle, and the leaflets are variably deformed. Characteristically, the anterior leaflet is…
Section I Tricuspid Atresia and Single-Ventricle Physiology Definition Tricuspid Atresia Tricuspid atresia is a congenital cardiac malformation in which the right atrium, in the setting of ventricular D-loop, fails to open into a ventricle through an atrioventricular (AV) valve. Thus,…
Definition Pulmonary atresia and intact ventricular septum is a congenital malformation in which the pulmonary valve is atretic and no ventricular septal defect exists. It coexists with variable degrees of right ventricular (RV) and tricuspid valve hypoplasia, and variable degrees…
Definition Pulmonary stenosis and intact ventricular septum is a form of right ventricular outflow tract obstruction in which the stenosis can be valvar, infundibular, or both. Isolated infundibular stenosis is unusual. This chapter concerns primarily valvar pulmonary stenosis, with or…