Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair?

Thirteen patients with complex of tetralogy of Fallot and absent pulmonary valve were treated. Three infants presented with severe cardiorespiratory distress (Group I) necessitating surgical intervention in two and intensive medical treatment in the third; there were no late survivals. The remaining 10 patients were operated upon electively at an older age (Group II); in six the ventricular septal defect was closed and the right ventricular outflow obstruction relieved (Group IIA), and in the other four a valve was inserted at the pulmonary anulus at the time of intracardiac repair (Group IIB). There was no hospital mortality in Group II. Five of the six patients in Group IIA remained symptomatic postoperatively, and three needed a subsequent pulmonary valve insertion. All patients in Group IIB were symptom free following operation; one patient began having a progressive decrease in exercise performance, decrease in pulmonary artery size, and cardiothoracic ratio were more marked in those patients who had primary insertion of a pulmonary valve. The clinical results and postoperative assessment of cardiac function tend to indicate that pulmonary valve insertion should be performed at the time of the intracardiac repair.