Definition Atrial isomerism is a condition in which the right-sided and left-sided atria, normally morphologically different, are morphologically similar. 1 Thus, left atrial isomerism and right atrial isomerism can occur. Atrial isomerism is a specific phenotypic feature highly associated with…
Definition Anatomically corrected malposition is an anomaly in the position of the great arteries but not in cardiac connections. Thus, there is atrioventricular (AV) and ventriculoarterial (VA) concordant connection as in the normal heart, but the aortic origin lies to…
Definition Double inlet ventricle is a congenital cardiac malformation in which both atria connect to only one ventricular chamber by either two separate atrioventricular (AV) valves or a common AV valve. Closely related to double inlet ventricle are cardiac malformations…
Section I Congenitally Corrected Transposition of the Great Arteries Definition Congenitally corrected transposition of the great arteries is a congenital cardiac anomaly with ventriculoarterial discordant connection (transposition of the great arteries) and atrioventricular (AV) discordant connection, the right atrium connecting to…
Definition Double outlet left ventricle (DOLV) is a cardiac anomaly in which both great arteries arise from the left ventricle (LV). 1 The great arteries are assigned to one or the other ventricle by the rules described under Definition in…
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…