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  • Clinical Features of Heart ...
    Miyamoto, Yuya; Kubo, Toru; Ochi, Yuri; Baba, Yuichi; Hirota, Takayoshi; Yamasaki, Naohito; Kawai, Kazuya; Yamamoto, Katsuhito; Kondo, Fumiaki; Bando, Kanji; Yamada, Eisuke; Furuno, Takashi; Yabe, Toshikazu; Doi, Yoshinori L.; Kitaoka, Hiroaki

    Circulation Journal, 2022/06/07
    Journal Article

    Background: The clinical features of heart failure (HF) in patients with hypertrophic cardiomyopathy (HCM) in Japan have not been fully elucidated.Methods and Results: In 293 patients with HCM (median age at registration, 65 (57–72) years) in a prospective cardiomyopathy registration network in Kochi Prefecture (Kochi RYOMA study), HF events (HF death or hospitalization for HF) occurred in 35 patients (11.9%) (median age, 76 (69–80) years), including 11 HF deaths during a median follow-up of 6.1 years. The 5-year HF events rate was 9.6%. Atrial fibrillation, low percentage of fractional shortening, and high B-type natriuretic peptide level at registration were predictors of HF events. The combination of these 3 factors had a relatively high positive predictive value (55%) for HF events and none of them had a high negative predictive value (99%). There were 4 types of HF profile: left ventricular (LV) systolic dysfunction (40%), severe LV diastolic dysfunction (34%), LV outflow tract obstruction (LVOTO) (20%), and primary mitral regurgitation (MR) (6%). HF deaths occurred in patients with LV systolic dysfunction or LV diastolic dysfunction, but none of patients with LVOTO or primary MR due to additional invasive therapies.Conclusions: In a Japanese HCM cohort, HF was an important complication, requiring careful follow-up and appropriate treatment.