Believing early repair to offer major benefits, we have repaired tetralogy of Fallot with pulmonary atresia in five infants aged less than six months. The ventricular septal defect was closed via a right atriotomy. The right ventriculotomy was 30±3% of the right ventricular length. The posterior wall of the right ventricular outflow tract was created by anastomosing directly the pulmonary trunk to the right ventriculotomy, or either by interposition of the left atrial appendage or an autologous pericardia! flap. The pathway was then roofed over with an equine pericardia! patch. All patients survived and are now doing well from 18 to 41 (31±11) months after the repair, although one patient required reoperation for relief of stenosis at the site of an anastomosis between the pulmonary arteries and the interposed left appendage. In the other patients, postoperative sequential echocardiography has shown no obstruction in the right ventricular outflow tract, nor significant pulmonary or tricuspid regurgitation. We conclude that primary repair in early infancy is an excellent option for surgical treatment of tetralogy of Fallot with pulmonary atresia.