Surgical repair of patients with tetralogy of Fallot and unilateral absence of pulmonary artery

Background. Patients with tetralogy of Fallot and unilateral absence of pulmonary artery are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 29-year experience in the treatment of those patients. Methods. Between May 1966 and February 1995, 2,511 patients underwent correction of tetralogy of Fallot in our department, 24 of those patients with unilateral absence of pulmonary artery (20 had absence of the left pulmonary artery, 4 had absence of the right pulmonary artery). Valved conduits were used in 9 patients, right ventricular patches were used in 4 patients, and transannular patches with a monocusp that was made of the patient's pericardium were used in 11 patients. Results. There were two operative deaths; both were in patients with hypoplasia of the left ventricle. All survivors had good early and late results. Conclusions. A right ventricular patch should be used in patients with tetralogy of Fallot and infundibular stenosis; a transannular patch with a monocusp should be used in patients with tetralogy of Fallot and stenosis of the left or right pulmonary artery's origin as well as the pulmonary trunk. A homograft valved conduit is suitable for patients with anomalous coronary artery or pulmonary atresia.