Congenital absence of the pulmonary valve: report of two cases with review of the literature.

Congenital absence of the pulmonary valve is an uncommon combination of anomalies. Recently, we encountered two such cases, one associated with tetralogy of Fallot (case 1), the other with ventricular septal defect (case 2). Clinically, our cases had minimum symptoms. The congenital cardiac anomalies were diagnosed with physical examination, chest x-ray film, echocardiography, and confirmed with cineangiography. These procedures disclosed a rudimentary pulmonary valve, pulmonary regurgitation and aneurysmal dilatation of the main and right pulmonary artery. In case 2, the right lower pulmonary artery occlusion and left hydronephrosis were also demonstrated by magnetic resonance imaging. The two cases underwent surgical repair to correct the tetralogy of Fallot and the ventricular septal defect respectively. Our experience in diagnosing these cardiac anomalies is reported.