Congenital Absence of the Pulmonary Valve

Abstract

The autopsy on a 32-year-old man revealed an absence of the pulmonary valve, a ventricular septal defect, and a single coronary artery. Congenital absence of the pulmonary valve has not been previously reported. The diagnosis of the accompanying pulmonary incompetence is discussed in the light of the catheterization studies and clinical findings. The pathologic abnormalities as found at postmortem examination are described and the embryologic basis for the several congenital malformations found in this unusual case are discussed.