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…
Section I Tetralogy of Fallot with Pulmonary Stenosis Definition Tetralogy of Fallot (TF) is a congenital cardiac malformation characterized by underdevelopment of the right ventricular (RV) infundibulum, with anterior and leftward displacement of the infundibular (conal, outlet) septum and its…
Definition Patent ductus arteriosus (PDA) is abnormal persistence of a patent lumen in the fetal ductus arteriosus, which usually connects the upper descending thoracic aorta with the proximal portion of the left pulmonary artery (LPA). When the aortic arch is…
Section I Unruptured and Ruptured Sinus of Valsalva Aneurysms Definition Congenital sinus of Valsalva aneurysms are thin-walled saccular or tubular outpouchings usually located in the right sinus or adjacent half of the noncoronary sinus. They generally have an intracardiac course…
Section I Primary Ventricular Septal Defect Definition Ventricular septal defect (VSD) is a hole or multiple holes in the interventricular septum. This chapter discusses VSDs that occur as the primary lesion, recognizing that hearts with primary VSDs may have minor…
Definition Atrioventricular (AV) septal defects are characterized by a deficiency or absence of septal tissue immediately above and below the normal level of the AV valves, including the region normally occupied by the AV septum, in hearts with two ventricles.…