Mortality and risk factors for late death in tetralogy of Fallot: the Japanese Nationwide Multicenter Survey.

OBJECTIVES We have compared mortality and risk factors for late deaths in patients with tetralogy of Fallot undergoing surgical repair in 1972 and 1982 in a Japanese multicentric study, examining in particular the impact of time of repair. BACKGROUND There is limited information on the effect that time of repair, and our constantly changing approach to it, has on late outcome in repaired tetralogy of Fallot. METHODS We analysed the Japanese registry of deaths occurring after surgical repair of tetralogy of Fallot. We studied two postoperative 1-year cohorts of survivors of surgery performed in 12 centers. Of the patients, 122, aged 29+/-12 years, had undergone repair in 1972, their age at repair being 9.6 years. An additional 186 patients, aged 23+/-8.7 years, had been repaired in 1982 at the age of 7.7 years. RESULTS Annual mortality, as judged per 100,000 population of patients with tetralogy of Fallot, declined from 0.387 in 1972 to 0.196 in 1982. Significant differences were deaths following surgery (27% vs. 13%, p < 0.001), patching of the subpulmonary outflow tract (48% vs. 89%, p < 0.001), and transjunctional patching (13% vs. 63%, p < 0.001). Late death was observed in 6 vs. 3 patients (9/308, 2.9%). The actuarial rate of survival calculated over 14 years was 97% vs. 98%. Reoperation was performed in 5 vs. 9 patients (14/308, 4.5%). Risk factors for late death were age at repair (p = 0.01), and history of reoperation (p < 0.001). Transjunctional patching (p = 0.01) proved to be associated with late mortality only in patients repaired in 1972. CONCLUSIONS Late survival was excellent, with a low incidence of reoperations in both groups of patients. The era of repair has a big influence on total and operative mortality, but has only a small impact on late mortality. Ongoing analysis of follow-up will possibly reveal subsequent changes with time.