Surgical management of tetralogy of Fallot with absent pulmonary valve in infancy

: Between November, 1987 and May, 1990, definitive operation was performed in 7 infants for tetralogy of Fallot with absent pulmonary valve. Preoperative respiratory failure was serious in 5 patients and semi-emergency operation was done. Surgical procedure consisted of closure of ventricular septal defect, reconstruction of right ventricular outflow tract using heterograft valved pericardial roll and plication of aneurysmally dilated pulmonary artery with or without pulmonary artery suspension in 5 infants with severe respiratory distress and heart failure, operated on 26 days to 5 months of age. In the other two less symptomatic infants, operated on at 13 months of age, reconstruction of right ventricular outflow tract was performed by transannular bovine pericardial patch with monocusp. There were no operative death and six infants are doing well 6 months to 3 years postoperatively, but one patient died of prolonged respiratory infection and respiratory failure one year after operation. Postoperative pulmonary arteriogram revealed a 42 to 61 (mean 54) percent reduction in right and left pulmonary artery size in 3 patients who underwent extensive plication of pulmonary artery. We suggest that definitive plication with or without suspension of pulmonary artery, together with closure of ventricular septal defect and reconstruction of right ventricular outflow tract is supposed to eliminate air way obstruction.