Kawasaki Disease
Definition/Morphology Tomisaku Kawasaki first reported Kawasaki disease (KD) in 1967. At first, KD was thought to be an acute and self-limiting febrile disorder; however, the first nationwide survey in Japan…
Heterotaxy and Isomerism of the Atrial Appendages
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…
Atrioventricular Valve Atresia
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…
Transposition of the Great Arteries
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…
Pulmonary Atresia With Ventricular Septal Defect
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…
Eisenmenger Syndrome
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
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…
Tetralogy of Fallot
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…
Pulmonary Atresia With Intact Ventricular Septum
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…
Double-Inlet Ventricle
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%…
