Total anomalous drainage of the pulmonary veins into the coronary sinus is a complicated congenital heart disease which takes a particularly severe course at early age. Its diagnosis is based on the discovery of overfilling of pulmonary circulation on radiography in a cyanotic patient, atrial septal defect and distended coronary sinus on echocardiography, and, in some cases, anomalous drainage of the pulmonary veins into the coronary sinus. The final diagnosis may be established during selective pulmonary arteriography. Operations were performed on 12 patients with this pathological condition under extracorporeal circulation and hypothermia with good results. In patients over one year of age the operation consisted in widening the atrial septal defect with resection of the part of the interatrial septum between the defect and the superior border of the coronary sinus and closure of the newly formed defect with a graft and transposition of the coronary sinus and the abnormally draining pulmonary veins into the left atrium. In patients under one year of age the interatrial septum was not resected.

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