Surgical management of critical pulmonary stenosis in the neonate

Experience with 36 consecutive neonates less than 1 month of age with critical pulmonary stenosis (PS) with intact ventricular septum was analyzed to define the role of pulmonary valvotomy and of a concomitant systemic-pulmonary shunt as well as the impact of prostaglandin E1 (PGE1) therapy in the management of this disorder. Operative procedures included pulmonary valvotomy as an isolated procedure (Group 1, N = 22), pulmonary valvotomy plus a systemic-pulmonary shunt (Group 2, N = 8), pulmonary valvotomy with PGE1 therapy (Group 3, N = 5), and one miscellaneous procedure. The hospital mortality (+/- 70% confidence limits [CL]) by treatment group was as follows: Group 1, 54% (CL, 41-67%) (12/22); Group 2, 25% (CL, 9-50%) (2/8); and Group 3, 0 (CL, 0-32%); Group 1 versus Group 2 plus Group 3 (p less than or equal to 0.05). Patients managed with a shunt or perioperative administration of PGE1 experienced a significant improvement in early survival. Late postoperative angiography demonstrated exemplary right ventricular growth in the majority of patients, although important residual abnormalities of the outflow tract necessitating operative repair were frequently present (5-year and 10-year actuarial freedom from reoperation, 73 +/- 10% and 42 +/- 16%, respectively [+/- standard error of the mean]). This review illustrates the limitations of pulmonary valvotomy as an isolated therapeutic method in neonates with critical PS. Provision of a systemic extracardiac source of pulmonary blood flow, accomplished by a systemic-pulmonary shunt or PGE1 infusion continued postoperatively, is the most important determinant of early survival in this disorder.