[Agenesis of the pulmonary valves. Experience over 20 years]

Absent pulmonary valve is a rare cardiac malformation, usually associated with a tetralogy of Fallot. It features aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory distress. The experience of our group over a 20 year period (20 patients) is reported. The diagnosis was usually made in the neonatal period in the presence of cardiomegaly and a systolic and diastolic murmur at the left sternal border. The diagnosis was made at antenatal echocardiography in 2 cases. Ten patients were not operated and 4 of them died in the neonatal period of respiratory distress and 2 others of extracardiac causes. Three children are on the waiting list for surgical correction and the remaining child is asymptomatic. The other 10 cases were operated with a minimum closure of the ventricular septal defect and widening of the pulmonary tract. The latter procedure was performed with an infundibulo-pulmonary patch (6 cases) and insertion of a pulmonary valve (4 patients) (monocusp patch in 3 cases, pulmonary heterograft in one case). Eight patients underwent a reduction procedure of the pulmonary arteries to relieve branchial compression. Of those who were operated, two died during follow-up: the first of complete atrioventricular block and the second of infectious endocarditis of the stenosed pulmonary heterograft. These results show that reduction procedures of the pulmonary arteries are promising approaches which should improve the future prognosis of young patients with respiratory distress. The need for insertion of a pulmonary valve remains debatable.