Uhl's anomaly with rudimentary pulmonary valve leaflets: a clinical, hemodynamic, angiographic, and pathologic study.

An acyanotic 4-year-old male child who failed to thrive presented with signs of persistent right heart failure and no cardiac murmurs. There was radiologic evidence of gross cardiomegaly with unremarkable lung fields. Cardiac catheterization and cineangiographic features helped in arriving at the diagnosis of parchment right ventricle with normal tricuspid valve (Uhl's anomaly), a rare condition seldom diagnosed during life. The patient was subjected to surgery, and Glenn's operation was performed, following which he died of a low output state and bradyarrhythmia. Autopsy revealed partial parchment right ventricle with rudimentary pulmonary valve leaflets, a combination not described previously.