Tetralogy of Fallot with absent pulmonary valve. Early and late results of surgical treatment.

From 1957 through September, 1983, 35 patients with tetralogy of Fallot and absent pulmonary valve underwent operation. Two subgroups of patients were recognized: minimally symptomatic (Group A, n = 21) and markedly symptomatic (Group B, n = 14). Group B patients were symptomatic at an earlier age and were younger at operation. Ages ranged from 1 day to 42 years (mean = 7.8 +/- 7.4 SD). Repair consisted of closure of the ventricular septal defect and relief of the right ventricle-pulmonary artery pressure gradient (nine patients received a tissue valve). In five patients, partial resection and/or plication of the aneurysmal pulmonary arteries also was performed. Operative mortality was 4.8% in Group A and 35.7% in Group B. In up to 25 years of follow-up, there was one late death in Group A (an early case with complete heart block); the remaining 19 patients are asymptomatic. In Group B, there were four late deaths, three related to cardiopulmonary insufficiency. These results indicate pulmonary valve insufficiency is tolerated well in minimally symptomatic patients. For markedly symptomatic patients, results may be improved if repair includes establishment of pulmonary valvular competence and reduction of size and length of aneurysmal pulmonary arteries.