Surgical treatment of tetralogy of Fallot with pulmonary atresia.

Thirteen (16 percent) of 80 patients with tetralogy of Fallot and pulmonary atresia undergoing corrective operations between Jan. 1, 1967, and Jan. 1, 1978, died in the hospital. The hospital mortality rate was 13 percent (10 deaths) among the 77 patients with confluent right and left pulmonary arteries. The risk of operation was not significantly affected by age at operation or by use of a valved external conduit versus a transannular outflow patch. It was affected (p = 0.008) by the ratio of peak right ventricular to left ventricular pressure (PRV/LV) immediately after repair. This (PRV/LV) was determined primarily by size of left and right pulmonary arteries. An equation was developed relating postrepair PRV/LV to diameter of right and left pulmonary artery (normalized by dividing by size of descending thoracic aorta), body surface area, and possible arborization abnormalities and stenoses of the right and left pulmonary arteries. Cardiac performance after repair was better in those in whom a transannular patch was used rather than a valved external conduit. Important pulmonary dysfunction postoperatively occurred more often in patients with large "bronchial" arteries than in those without them, but was less when these were not ligated. Four (8 percent) of 48 traced hospital survivors died late postoperatively. Reoperations late postoperatively were required only in patients receiving valved external conduits. Forty (91 percent) of 44 living traced patients are asymptomatic.