Correction of total anomalous pulmonary venous connection below the diaphragm

Six infants with total anomalous pulmonary venous connection below the diaphragm had correction by modification of conventional surgical technique. Catheterization revealed the confluence of the pulmonary veins draining into a descending vein below the diaphragm. Symptoms of pulmonary venous hypertension and low cardiac output were typical. All had repair with circulatory arrest (average time, 32 minutes). Mobilization of the pulmonary veins and the descending vein is important. The descending vein was transected at the diaphragm. Its anterior surface was incised through the confluence of the pulmonary veins, thus creating an open Y incision. This large Y-shaped vein was anastomosed to the left atrium and carried obliquely to the tip of the left atrial appendage. The anastomosis was fashioned so that the long limb of the Y rotated anteriorly and superiorly to substantially enlarge the left atrium, making the total diameter of the anastomosis larger than the mitral valve orifice. This simplified the surgical repair and allowed direct suture closure of the atrial septal defect in all patients, as the left atrial size was at least doubled. No postoperative complications occurred, and the patients were discharged an average of 4.2 days postoperatively. Restudy at an average of 3.5 years revealed normal pressures and normal architecture by angiography. Use of the descending vein as an integral part of the reconstruction and enlargement of the left atrium was the major technical factor leading to a successful outcome in these patients and eliminating a patch or transposition of the atrial septum.