Definitions The term heterotaxy comes from the Greek heteros, meaning “other” and taxis, meaning “arrangement.” The nomenclature and definition of heterotaxy have been a matter of debate for years and…
In hearts with a univentricular atrioventricular connection both atria are connected to a single ventricle; this includes hearts with either atrioventricular valve (AVV) atresia or a double-inlet atrioventricular connection. Either…
Definition and Morphology The term transposition of the great arteries (TGA) describes the anatomic arrangement in which the aorta arises from the right ventricle (RV) and the pulmonary artery from…
General Overview Pulmonary atresia (PA), or absence of a communication between the right ventricle (RV) and the main pulmonary artery (MPA), exists in two forms based on the presence or…
Definition and Morphology In 1897, Victor Eisenmenger, an Austrian physician, first described both the clinical and pathologic features of irreversible pulmonary vascular disease in a 32-year-old man with a nonrestrictive…
Double-outlet right ventricle (DORV) is a “disease” that includes a family of anatomically related complex congenital heart lesions involving the right ventricular outflow tract (RVOT). There are several variations within…
Definition and Morphology It was Etienne-Louis Arthur Fallot who, in a series of papers in 1888, separated the malformation we now describe with his name from other anatomic lesions responsible…
Definition and Morphology Pulmonary atresia with intact ventricular septum (PAIVS) was first described by John Hunter in 1783. It is a rare congenital cardiac malformation with considerable morphologic heterogeneity, and…
Definition and Morphology The nomenclature for single ventricle has been a subject of debate for years. Double-inlet ventricle is commonly defined as the morphologic arrangement in which more than 50%…
Definition and Morphology Congenitally corrected transposition of the great arteries (CCTGA) was described in 1875 by von Rokitansky and is characterized by atrioventricular (AV) and ventriculo-arterial (VA) discordance. CCTGA may…
