Gomes, Manuel M.R.

The results of complete repair of double-outlet right ventricle with pulmonic stenosis in 22 Mayo Clinic patients were analyzed. The overall mortality rate was 32%. Since 1960, the mortality rate has been reduced to 16%. Anomalies of the distribution of the right coronary artery, a second ventricular septal defect of the atrioventricular canal type, and significant residual pulmonic stenosis were associated with a higher mortality rate. The difficulties in the diagnosis of this uncommon condition are emphasized. Complete repair is best carried out after the patient is 4 years old. A systemic-to-pulmonary arterial shunt is the procedure of choice for those who are younger than this. In the occasional patient with anomalous distribution of the right coronary artery which precludes adequate relief of the pulmonic stenosis, use of an aortic homograft from the right ventricle to the pulmonary artery should be considered.

The results of complete repair of double-outlet right ventricle without pulmonic stenosis in 18 Mayo Clinic patients are analyzed; the over-all operative mortality rate was 22%. Pulmonary vascular obstructive disease and a second ventricular septal defect of the atrioventricular canal type were associated with a higher mortality rate. The difficulties in the diagnosis of this uncommon condition are emphasized. Patients with double-outlet right ventricle without pulmonic stenosis should have complete repair early in life before the onset of severe pulmonary vascular obstructive disease-the only specific contraindication to operation. Pulmonary artery banding may be preferable as a first-stage palliative procedure in infants less than six months old who do not respond to medical therapy.