Outcome after repair of tetralogy of fallot with absent pulmonary valve

Background. Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is as sociated with pulmonary artery dilatation and airway compression. Methods. Since January 1, 1984, 28 patients with TOF/APV have undergone com plete repair (median age 11 days, range 1 day to 16 years). Results. Thirteen patients were ventilated for respiratory failure preopera tively and extracorporeal membrane oxygenation was used in 3. Twenty-six pa tients underwent pulmonary artery plication (11 anterior, 15 anterior/poste rior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intub ated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved a fter repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71 %) at 10 years. In a multivariable analysis, only preoperative intubation w as associated with a worse outcome (p = 0.04). Conclusions. Long-term outcome for patients with TOF/APV who survive the in itial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression. (Ann Thora c Surg 1994;67:1391-6) (C) 1999 by The Society of Thoracic Surgeons.