Definition Double outlet right ventricle (DORV) is a congenital cardiac anomaly in which both great arteries arise wholly or in large part from the right ventricle. 1 It is a type of ventriculoarterial connection (see “Cardiac Connections” under Terminology and…
Definition Complete transposition of the great arteries (TGA) is a congenital cardiac anomaly in which the aorta arises entirely or largely from the right ventricle (RV) and in which the pulmonary trunk arises entirely or largely from the left ventricle…
Section I Vascular Ring Definition Vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus or both. Historical Note Double aortic arch was apparently first described…
Definition Congenital mitral valve disease is a developmental malformation of one or more of the components of the mitral valve apparatus, including that portion of left atrial wall immediately adjacent to the mitral anulus that produces stenosis or regurgitation or,…
Definition Hypoplastic left heart physiology is defined as inability of the left heart to sustain adequate cardiac output following birth because of underdevelopment of one or more left heart structures despite surgical or medical intervention. Box 49-1 emphasizes four important…
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…