Concomitant valvotomy and subclavian-main pulmonary artery shunt in neonates with pulmonary atresia and intact ventricular septum.

Our current approach to the management of neonates with pulmonary atresia and intact ventricular septum is to perform a transarterial pulmonary valvotomy through a left anterolateral thoracotomy followed by a polytetrafluoroethylene shunt between the left subclavian artery and the pulmonary trunk at the site of the pulmonary arteriotomy. From October, 1983, to December, 1985, 7 consecutive neonates with pulmonary atresia and intact ventricular septum were managed in this fashion. Mean age was 5.1 days (5 patients, less than 48 hours old), and mean weight was 3.3 kg (range, 2.5-4.3 kg). Right ventricular morphology was type I (tripartite) in 4 patients, type II (absent trabecular portion) in 2, and type III (absent trabecular and infundibular portions) in 1. The mean right ventricular to left ventricular peak systolic pressure ratio was 1.5. One patient who initially had valvotomy alone required a left subclavian-pulmonary trunk shunt the next day for hypoxemia. All other patients had a valvotomy and shunt during the same procedure. There were no operative or hospital deaths. Follow-up of 3.5 to 34 months (mean, 17.5 months) confirmed shunt patency in all patients. Three of 4 patients undergoing postoperative catheterization have shown good right ventricular growth; 2 have undergone successful repair at 10 and 23 months. There have been 3 late deaths at 3.5, 4, and 8 months. Two other patients are doing well and are awaiting postoperative catheterization. This procedure permits synchronous valvotomy and shunting without the need for cardiopulmonary bypass in these critically ill neonates.(ABSTRACT TRUNCATED AT 250 WORDS)