Recurrent Pulmonary Venous Stenosis after Repair of Mixed-Type Total Anomalous Pulmonary Venous Connection

Abstract

A 10-day-old male neonate underwent repair of mixed-type total anomalous pulmonary venous connection. The left upper pulmonary vein connected to the left innominate vein by way of a vertical vein. The other veins converged to form a common pulmonary vein and drained to the coronary sinus. As the common pulmonary vein was not stenotic, normal coronary sinus unroofing was undertaken and the postoperative course was uneventful. Five months later pulmonary vein stenosis(PVS)occurred at the junction of the common pulmonary vein and coronary sinus. At reoperation the common pulmonary vein was deeply incised to the point near the pulmonary venous orifice, and the stenotic tissue was resected. Although he was discharged from the hospital on the 10th postoperative day, PVS recurred at age 9 months and a second reoperation was undertaken. This time, the common pulmonary vein was excised and the anterior wall of each pulmonary vein was incised to drain independently and directly to the left atrium without causing turbulence. The left upper pulmonary vein was anastomosed to the left atrial appendage. Pulmonary angiography 18 months after the second reoperation revealed the pulmonary venous pathway to be nonstenotic.