ABSENT PULMONARY VALVE - EXPERIENCE OVER 20 YEARS

Absent pulmonary valve is a rare cardiac malformation, usually associa ted with a tetralogy of Fallot. It features aneurysmal dilatation of t he pulmonary arteries which may compress the bronchial tree and lead t o respiratory distress. The experience of our group over a 20 year per iod (20 patients) is reported. The diagnosis was usually made in the n eonatal period in the presence of cardiomegaly and a systolic and dias tolic murmur at the left sternal border. The diagnosis was made at ant enatal echocardiography in 2 cases. Ten patients were not operated and 4 of them died in the neonatal period of respiratory distress and 2 o thers of extracardiac causes. Three children are on the waiting list f or surgical correction and the remaining child is asymptomatic. The ot her 10 cases were operated with a minimum closure of the ventricular s eptal defect and widening of the pulmonary tract. The latter procedure was performed with an infundibulo-pulmonary patch (6 cases) and inser tion of a pulmonary vave (4 patients) (monocusp patch in 3 cases, pulm onary heterograft in one case). Eight patients underwent a reduction p rocedure of the pulmonary arteries to relieve branchial compression. O f those who were operated, two died during follow-up: the first of com plete atrioventricular block and the second of infectious endocarditis of the stenosed pulmonary heterograft. These results show that reduct ion procedures of the pulmonary arteries are promising approaches whic h should improve the future prognosis of young patients with respirato ry distress. The need for insertion of a pulmonary valve remains debat able.