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%…
Congenitally Corrected Transposition of the Great Arteries
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…
Absent Pulmonary Valve Syndrome
Definition and Morphology Absent pulmonary valve syndrome is a rare congenital cardiac malformation that was first described anatomically by Chevers in 1847. The first clinical case report was published in…
Double-Chambered Right Ventricle
Definition and Morphology Double-chambered right ventricle (DCRV) is characterized by anomalous or hypertrophied muscle bundles, which cause a form of subvalvar right ventricular outflow tract (RVOT) obstruction, dividing the right…
Pulmonary Stenosis
Definition and Morphology Pulmonary valvular stenosis is usually an isolated congenital anomaly and occurs in 7% to 12% of patients with congenital heart disease. It can sometimes be associated with…
Tricuspid Regurgitation and Stenosis
Definitions and Morphology Isolated congenital anomalies of the tricuspid valve (TV) are rare structural malformations that involve one or more components of the TV apparatus and result more commonly in…
