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Prognosis and prognostic factors in patients with hypertrophic cardiomyopathy in Japan: results from a nationwide study
  1. Ali Nasermoaddeli (moaddeli{at}med.u-toyama.ac.jp)
  1. Toyama University, Japan
    1. Katsuyuki Miura (miura{at}kanazawa-med.ac.jp)
    1. Kanazawa Medical University, Ishikawa, Japan
      1. Akira Matsumori
      1. Kyoto University Graduate School of Medicine, Kyoto, Japan
        1. Yoshiyuki Soyama
        1. Kanazawa Medical University, Ishikawa, Japan
          1. Yuko Morikawa
          1. Kanazawa Medical University, Ishikawa, Japan
            1. Akira Kitabatake
            1. Hokkaido University Graduate School of Medicine, Sapporo, Japan
              1. Yutaka Inaba
              1. Juntendo University School of Medicine, Tokyo, Japan
                1. Hideaki Nakagawa
                1. Kanazawa Medical University, Ishikawa, Japan

                  Abstract

                  Objective To investigate prognosis and prognostic factors in patients with hypertrophic cardiomyopathy (HCM) in Japan.

                  Design A nationwide epidemiological study.

                  Setting Hospitals selected randomly from among all hospitals in Japan.

                  Patients Clinical and epidemiological information for 2155 patients with HCM were collected in 1999. Main outcome measures: Patients were classified on the basis of baseline prognostic factors. Survival rates up to 5 years were calculated by Cox's proportional hazard model for 1605 patients.

                  Results During the follow-up period 241 deaths were recorded. The crude 5-year survival rate for the entire cohort was 86% (95% confidence interval [CI]: 84-88), and annual mortality ranged from 2.2% to 3.0%. A higher cardiothoracic ratio on chest X-ray (hazard ratio [HR] 1.61 [95%CI: 1.26-2.05] with 1 standard deviation [6.2%] increase), a lower left ventricular ejection fraction (HR 1.42 [95%CI: 1.20-1.69] with 1 standard deviation [13%] decrease), and the presence of left bundle branch block (HR 3.14; 95%CI: 1.28-7.71) were independently associated with a poorer prognosis, while the presence of apical hypertrophy at baseline (HR 0.58; 95%CI: 0.36-0.92) predicted a better chance of survival.

                  Conclusions The nationwide survey of patients with hypertrophic cardiomyopathy yielded important information on its prognosis and prognostic factors. These observations afford, for the first time, a measure of risk stratification in patients with HCM in Japan.

                  • Hypertrophic cardiomyopathy
                  • Japan
                  • Prognostic factors
                  • Survival rate

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