Critical pulmonary valve stenosis with intact ventricular septum.

This study presents our experience in the surgical management of the newborn with critical pulmonary stenosis and intact ventricular septum. During the last decade we have seen 11 such patients, with a mean age of 2.3 days (maximum 6 days) and a mean weight of 3.6 kilograms (maximum 4.5 kilograms). The primary operation was a valvotomy in 7 patients (one of whom also had a systemic-pulmonary shunt), a commissurotomy with cardiopulmonary bypass in 3 patients, and a shunt alone in 1 patient. One infant died on the second postoperative day and another died following reoperation at 4 months. The other 9 patients were all alive and progressing satisfactorily when last seen. Five of these patients were beyond their third postoperative year and the longest survivor was at 7.9 years. Four of the long-term survivors had undergone reoperation. Our choice of operation remains transventricular valvotomy, although an open procedure utilizing cardiopulmonary bypass presents an attractive alternative. A systemic-pulmonary shunt as an addition to either of these operations is unnecessary. The perioperative use of prostaglandin E brings about substantial improvement in the early results.