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Background: Arrhythmia and late cardiac deaths are thought to be major complications in patients after right ventricle (RV) to pulmonary artery (PA) conduit repair, although the incidence and predictors of these complications remain unknown. The aim of this study was to clarify the incidence and risk factors for arrhythmia and late deaths in patients with the RV to PA conduit repair through a Japanese multicenter study., Methods: Three hundred fifty-one hospital survivors who underwent the RV to PA conduit repair before 1995 were studied., Results: Survival rate after repair was 92% at 10 years, 88% at 20 and 25 years, respectively. Late death was observed in 30 (8.5%) including 4 patients with sudden death (SD). Higher right ventricular pressure (p = 0.02), larger cardio-thoracic ratio after repair (p = 0.02) and higher incidence of brady- or tachy-arrhythmia and SD (9/30) were associated with late death. Six (1.7%) patients developed ventricular tachycardia or ventricular fibrillation (VT/Vf). There were 22 patients who had 23 new-onset supraventricular tachy-arrhythmia (SVT). Right ventricular hypertension (p = 0.04) was associated with VT/Vf or SD. Male sex (p < 0.01), absence of previously aorto-pulmonary shunt (p < 0.05), older age at repair (p < 0.01) or longer length of follow-up (p < 0.01) were associated with SVT., Conclusion: Arrhythmia and late sudden death are relatively common late after the RV to PA conduit repair. Our data support recent surgical strategies of earlier primary operation and timely reoperation for progressive right ventricular outflow stenosis that may reduce the incidence of late arrhythmias and SD.

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.