Congenital Absence of the Pulmonary Valve

A group of cases of tetralogy of Fallot with congenital absence of the pulmonary valve are presented, and it is indicated that this abnormality presents a characteristic clinical, physiologic, and pathologic syndrome. It is postulated that the aneurysmal dilatation of the pulmonary artery may result either from a congenital weakness at the base of the pulmonary artery or from the hemodynamic effects of a ventricular septal defect and infundibular stenosis in the presence of pulmonary regurgitation. The absence of the pulmonary valve has not been a deterrent to successful corrective surgery aimed at the basic pathology of tetralogy of Fallot in three cases.