title: Clinical Characteristics and Long-Term Outcomes of Hypertrophic Cardiomyopathy.
abstract: 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.
authors.affiliation: Department of Cardiology, Fujita Health University Banbuntane-Hotokukai Hospital.
authors.forename: Masataka
authors.initials: M
authors.lastname: Yoshinaga
authors.forename: Daiji
authors.initials: D
authors.lastname: Yoshikawa
authors.forename: Hideki
authors.initials: H
authors.lastname: Ishii
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hirashiki
authors.forename: Takahiro
authors.initials: T
authors.lastname: Okumura
authors.forename: Aki
authors.initials: A
authors.lastname: Kubota
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sakai
authors.forename: Ken
authors.initials: K
authors.lastname: Harada
authors.forename: Fuji
authors.initials: F
authors.lastname: Somura
authors.forename: Tomofumi
authors.initials: T
authors.lastname: Mizuno
authors.forename: Wakaya
authors.initials: W
authors.lastname: Fujiwara
authors.forename: Hiroatsu
authors.initials: H
authors.lastname: Yokoi
authors.forename: Mutsuharu
authors.initials: M
authors.lastname: Hayashi
authors.forename: Junichi
authors.initials: J
authors.lastname: Ishii
authors.forename: Yukio
authors.initials: Y
authors.lastname: Ozaki
authors.forename: Toyoaki
authors.initials: T
authors.lastname: Murohara
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Amano
authors.forename: Hideo
authors.initials: H
authors.lastname: Izawa
mesh_terms: D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D003643:Death; D004452:Echocardiography / Q000379:methods; D004554:Electric Countershock / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries / Q000706:statistics & numerical data; D020521:Stroke / Q000453:epidemiology / Q000209:etiology; D016019:Survival Analysis; D014693:Ventricular Fibrillation / Q000453:epidemiology / Q000209:etiology / Q000628:therapy