Valvotomy for pulmonary atresia with intact ventricular septum. A disciplined approach to achieve a functioning right ventricle.

During the past 20 years, a consistent policy in applying early valvotomy has resulted in a unique opportunity to appraise the long-term results of this approach in pulmonary atresia with intact ventricular septum. Since 1964, 27 of 35 patients with pulmonary atresia with intact ventricular septum had type 1 or 2 right ventricle, 25 of these had early valvotomy, seven with and 18 without concomitant shunt. The remaining two patients with type 2 right ventricle and the eight patients with type 3 right ventricle received a shunt alone. Overall operative mortality was 34%; for those patients weighing more than 3 kg and those operated upon since 1977, it was 18% and 16%, respectively. There were 17 survivors of early valvotomy: 11 had valvotomy alone and six had valvotomy with shunt; 12 had type 1 right ventricle and five had type 2 right ventricle. Survival rates (+/- standard error) for these 17 patients were 85% +/- 10% and 68% +/- 17% at 5 and 10 years, respectively. The probability of reoperation was 100% by 6 years of age; outflow patch reconstruction was employed in all patients in whom reoperation has been performed. Aggressive follow-up and early recatheterization were essential features of management. Delayed reconstruction after shunt alone was unsuccessful in three patients. Primary valvotomy without shunt is the operation of choice for patients with pulmonary atresia with intact ventricular septum and type 1 right ventricle. Concomitant shunt may be required for some patients with type 1 and most with type 2 right ventricle, selected preoperatively by angiography or after valvotomy by clinical necessity. Delayed right ventricular reconstruction after shunt alone is not an acceptable approach when an outflow tract is present.