Surgical management of pulmonary atresia, and critical pulmonary stenosis with intact ventricular septum.

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum has included 20 patients with pulmonary atresia and 3 patients with critical pulmonary stenosis. Group 1 consisted of 7 infants who were treated by transarterial pulmonary valvotomy. Four of them were lost shortly after surgery. Group 2 included 11 neonates and one infant who were treated by systemic-pulmonary shunt with 2 operative death. Group 3 included 4 infants who underwent primary prosthetic enlargement of the right ventricular outflow tract. Two of the 4 were lost in the early postoperative period. Eight of 13 patients in groups 1 and 2 who survived the initial palliative procedures underwent additional operations to enlarge the right ventricular outflow tract, and 5 patients survived. However, adequate right ventricular and tricuspid valve growth was obtained in only patients in whom the prosthetic enlargement of the right ventricle was performed in early infancy. These findings strongly suggest the necessity to decompress the right ventricle completely, as early as possible. In conclusion, 1) transarterial pulmonary valvotomy seldom provided satisfactory decompression of the right ventricle, and operative risk was high. 2) systemic-pulmonary shunt to re-establish pulmonary blood flow improved neonatal survival, although this procedure may not provide a satisfactory long term palliation. 3) primary or secondary operative enlargement of the right ventricle in early infancy provided decompression of the right ventricle and accelerated substantial right ventricular growth. For patients without an infundibulum or for those in whom attempts to induce tricuspid valve growth had failed, a Fontan type procedure seemed to be the procedure of choice.