Subtypes

Pulmonary atresia with intact ventricular septum

Transposition of great vessels with VSD and pulmonary atresia

Tricuspid atresia with pulmonary atresia

Tricuspid stenosis with pulmonary atresia

May Cause

Congenital heart disease with decreased pulmonary vascularity

Main pulmonary artery obstruction

Marked cyanosis in first week of life

Right atrial enlargement

Right ventricular enlargement

May Be Caused by

Robinow syndrome

Werner syndrome