Pediatric Cohort With Long QT Syndrome : KCNH2 Mutation Carriers Present Late Onset But Severe Symptoms

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  • Ozawa Junichi
    Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences
  • Ohno Seiko
    Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science Center for Epidemiologic Research in Asia, Shiga University of Medical Science
  • Hisamatsu Takashi
    Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science Center for Epidemiologic Research in Asia, Shiga University of Medical Science Department of Public Health, Shiga University of Medical Science
  • Itoh Hideki
    Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science
  • Makiyama Takeru
    Department of Cardiovascular Medicine, Kyoto University Graduate School of Medicine
  • Suzuki Hiroshi
    Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences
  • Saitoh Akihiko
    Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences
  • Horie Minoru
    Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science

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タイトル別名
  • Pediatric Cohort With Long QT Syndrome　– <i>KCNH2</i> Mutation Carriers Present Late Onset But Severe Symptoms –

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Background:In children with long QT syndrome (LQTS), risk factors for cardiac events have been reported, but age-, gender- and genotype-related differences in prognosis remain unknown in Asian countries.Methods and Results:The study examined clinical prognosis at age between 1 and 20 years in 496 LQTS patients who were genotyped as either of LQT1–3 (male, n=206). Heterozygous mutations were observed in 3 major responsible genes:KCNQ1in271,KCNH2in 192, andSCN5Ain 33 patients. LQTS-associated events were classified into 3 categories: (1) syncope (n=133); (2) repetitive torsade de pointes (TdP, n=3); and (3) cardiopulmonary arrest (CPA, n=4). The risk of cardiac events was significantly lower in LQT1 girls than boys≤12 years (HR, 0.55), whereas LQT2 female patients ≥13 years had the higher risk of cardiac events than male patients (HR, 4.60). Patients in the repetitive TdP or CPA group included 1 LQT1 female patient, 1 LQT2 male patient, and 5 LQT2 female patients. All LQT2 patients in these groups had TdP repeatedly immediately after the antecedent event. In addition, all 5 female LQT2 patients in these groups had the event after or near puberty.Conclusions:Female LQT2 children might have repeated TdP shortly after prior events, especially after puberty. (Circ J 2016; 80: 696–702)

収録刊行物

  • Circulation Journal

    Circulation Journal 80 (3), 696-702, 2016

    一般社団法人 日本循環器学会

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