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Mortality and risk factors for late deaths in tetralogy of Fallot: the Japanese Nationwide Multicentric Survey
- Koichiro Niwa, Hiromichi Hamada, Makoto Nakazawa, Masaru Terai, Shigeru Tateno, Satoru Sugimoto, Hiroshi Watanabe, Arata Murakami, Mayumi Ohta, Akira Ishizawa, Toshiyuki Katoki, Katuhiko Mori, Seiyo Yasui, Youichi Kawahira, Teiji Akagi, Naho Haraguchi, Michael A Gatzoulis
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- Journal:
- Cardiology in the Young / Volume 12 / Issue 5 / October 2002
- Published online by Cambridge University Press:
- 15 August 2006, pp. 453-460
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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.
Electrocardiographic changes in patients with cardiac rhabdomyomas associated with tuberous sclerosis
- Junko Shiono, Hitoshi Horigome, Seiyo Yasui, Tomoyuki Miyamoto, Miho Takahashi-Igari, Nobuaki Iwasaki, Akira Matsui
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- Journal:
- Cardiology in the Young / Volume 13 / Issue 3 / June 2003
- Published online by Cambridge University Press:
- 24 May 2005, pp. 258-263
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Background: Cardiac rhabdomyomas associated with tuberous sclerosis induce various abnormalities in the electrocardiogram. Electrocardiographic evidence of ventricular hypertrophy may appear if the tumour is electrically active. To our knowledge, electrocardiographic evidence of ventricular hypertrophy has been reported only in association with congestive heart failure. Follow-up studies of changes in electrocardiographic findings are also lacking. Methods: We studied 21 consecutive patients with cardiac rhabdomyoma associated with tuberous sclerosis, 10 males and 11 females, aged from the date of birth to 9 years at diagnosis. The mean period of follow-up was 53 months. None of the patients developed congestive heart failure. We evaluated the electrocardiographic changes during the follow-up, and their association with echocardiographic findings. Results: Of the 21 patients, 12 showed one or more abnormalities on the electrocardiogram at presentation, with five demonstrating right or left ventricular hypertrophy. In all of these five cases, the tumours were mainly located in the respective ventricular cavity. In one patient with a giant tumour expanding exteriorly, there was marked left ventricular hypertrophy on the electrocardiogram. Followup studies showed spontaneous regression of the tumours in 12 of 19 patients, with abnormalities still present in only 7 patients. A gradual disappearance of left ventricular hypertrophy as seen on the electrocardiogram was noted in the patient with marked left ventricular hypertrophy at presentation in parallel with regression of the tumour. Conclusions: The presence of cardiac rhabdomyomas in patients with tuberous sclerosis might explain the ventricular hypertrophy seen on the electrocardiogram through its electrically active tissue without ventricular pressure overload or ventricular enlargement, although pre-excitation might affect the amplitude of the QRS complex. Even in cases with large tumours, nonetheless, the electric potential might not alter the surface electrocardiogram if the direction of growth of the tumour is towards the ventricular cavity. In many cases, electrocardiographic abnormalities tend to disappear, concomitant with regression of the tumours.