Clinical and Genetic Features of Japanese Patient with Congenital Long QT Syndrome

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概要

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• <B>Backgrounds:</B> Genotype-phenotype aspects in Japanese patients (pts) with congenital long QT syndrome (LQTS) have not been fully elucidated. <B>Methods and Results:</B> Genotyped LQTS patients from Japanese multicenter registry (402 LQT1, 381 LQT2, and 113 LQT3 pts) were investigated. Age at first cardiac event (CE) was significantly lower and corrected QT (QTc) interval was significantly shorter in LQT1 than in LQT2 and LQT3 (13±14, 19±15, 17±20 yo; P=0.0004, 475±46, 489±46, 482±58 ms; P=0.0003). Notched T wave (NTW) was more frequently observed in LQT2 (77%) than in LQT1 (9%) and LQT3 (10%). T wave alternance (TWA) was more frequently found in LQT3 (13%) compared with LQT1 (4%) and LQT2 (6%). Multivariate Cox-regression demonstrated that LQT1 and LQT2 pts with pore-site mutation and LQT3 pts with transmembrane mutation had significantly higher CE rate than those without (HR=1.52, 1.63, and 4.67, respectively). TWA (HR=2.37) in LQT1, longer QTc (HR=1.06 for QTc/10 ms) and NTW (HR=1.51) in LQT2, and longer QTc (HR=1.11), NTW (HR=3.26), and TWA (HR=2.51) in LQT3 were independent risk factors of CE. <B>Conclusions:</B> In addition to mutations located in transmembrane region, some electrocardiographic parameters can be available as predictors of cardiac events in 3 genotypes of Japanese LQTS.

著者

• KAMAKURA Shiro

  National Cardiovascular Center

• Horie Minoru

  Japanese LQTS Multicenter Registry by the Minister of Health, Lobour and Welfare, National Cardiovas

• Aizawa Yoshifusa

  Japanese LQTS Multicenter Registry by the Minister of Health, Lobour and Welfare, National Cardiovas

• SHIMIZU Wataru

  National Cardiovascular Center

• Makita Naomasa

  Japanese Brugada Multicenter Registry By The Minister Of Health Labour And Welfare National Cardiova

• Yamagishi Masakazu

  Japanese Brugada Multicenter Registry By The Minister Of Health Labour And Welfare National Cardiova

• Miyamoto Yoshihiro

  National Cardiovascular Center

• Ohe Tohru

  Japanese Brugada Multicenter Registry By The Minister Of Health Labour And Welfare National Cardiova

• Ogawa Satoshi

  Japanese Brugada Multicenter Registry By The Minister Of Health Labour And Welfare National Cardiova

• Ogawa Satoshi

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

• Makimoto Hisaki

  National Cerebral and Cardiovascular Center

• Makiyama Takeru

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

• Aiba Takeshi

  National Cerebral and Cardiovascular Center

• Makita Naomasa

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

• Ohe Tohru

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

• Horie Minoru

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

• Aizawa Yoshifusa

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

• Yamagishi Masakazu

  Japanese LQTS Multicenter Registry by the Minister of Health, Labour and Welfare

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