International Heart Journal
Online ISSN : 1349-3299
Print ISSN : 1349-2365
ISSN-L : 1349-2365
Clinical Studies
Clinical Characteristics and Long-Term Outcomes of Hypertrophic Cardiomyopathy
Results from the Aichi Hypertrophic Cardiomyopathy (AHC) Registry
Masataka YoshinagaDaiji YoshikawaHideki IshiiAkihiro HirashikiTakahiro OkumuraAki KubotaShinichi SakaiKen HaradaFuji SomuraTomofumi MizunoWakaya FujiwaraHiroatsu YokoiMutsuharu HayashiJunichi IshiiYukio OzakiToyoaki MuroharaYukihiko YoshidaTetsuya AmanoHideo Izawa
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2015 Volume 56 Issue 4 Pages 415-420


Hypertrophic cardiomyopathy (HCM) has various morphological and clinical features. A decade has passed since the previous survey of the epidemiological and clinical characteristics of Japanese HCM patients. The Aichi Hypertrophic Cardiomyopathy (AHC) Registry is based on a prospective multicenter observational study of HCM patients. The clinical characteristics of 42 ambulant HCM patients followed up for up to 5 years were investigated. The primary endpoint was major adverse cardiac events (MACE), defined as death, non-fatal stroke, admission due to congestive heart failure (CHF), or episodes of sustained ventricular tachycardia/fibrillation. The MACE-free survival during the 5-year follow-up period was 76% according to Kaplan–Meier analysis. HCM-related death occurred in 3 (7%) patients and SCD occurred in 2 (5%) patients. Additionally, 3 (7%) patients were admitted to the hospital due to CHF. Meanwhile, sustained VT was detected in one (2%) of the patients who received ICD implantation and subsequently terminated with antitachycardia pacing using an ICD. The patients with HCM exhibiting left ventricular outflow obstruction (HOCM) had a slightly lower MACE-free survival rate than those with neither HOCM nor dilated-HCM (dHCM) (71% versus 81%, log-rank P = 0.581). Furthermore, the patients with dHCM demonstrated a significantly lower MACE-free survival rate than those with neither HOCM nor dHCM (33% versus 81%, log-rank P = 0.029). In the AHC Registry targeting current Japanese HCM patients, we demonstrated that many HCM patients continue to suffer from MACE despite the development of various treatments for HCM.

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© 2015 by the International Heart Journal Association
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