[Stenosis of the pulmonary veins in total anomalous pulmonary venous drainage. Associated or iatrogenic abnormality?]

Abstract

Obstruction of total anomalous pulmonary venous drainage (TAPVD) is sometimes observed in neonates, usually at the site where the collector drains into the caval system. Stenosis of the pulmonary veins themselves before joining the collector is less common. This was observed in 6 cases of intra- and supracardiac TAPVD; the prognosis is usually very bad due to postoperative pulmonary hypertension (PHT) which is difficult to treat. Three of our cases had TAPVD into the coronary sinus with obstruction presenting at birth. Conventional surgery did not reduce the PHT of the first 2 children because the congenital stenosis of the pulmonary veins was not corrected. In the first case, the pulmonary veins resembled fibrous cords and in the second case, the collector was stenosed at its junction with the coronary sinus. These two children died despite surgery. The third child, however, was cured because the obstruction was diagnosed preoperatively and successfully treated when the TAPVD was corrected. In the other three cases, TAPVD to the superior vena caval system was not obstructed but the pulmonary veins retracted progressively after surgery causing PHT and right ventricular failure. After unsuccessful percutaneous dilatation, reoperation revealed obstruction due to exuberant scar tissue with retraction at the site of anastomosis and (or) reconstruction of the pulmonary veins by incision or enlargement with a pericardial patch. One of these children survived after pneumonectomy of the obstructed lung which relieved reflex PHT of the contralateral lung.(ABSTRACT TRUNCATED AT 250 WORDS)