Heart failure & cardiomyopathies

Contemporary clinical characteristics and management patterns in hypertrophic cardiomyopathy: insights from baseline enrolment data in a nationwide prospective Japanese registry

HeartPublished multicentre, prospective study

Authors' conclusion

As the first large-scale, prospective HCM registry in Japan, this study provides valuable baseline data on the clinical characteristics and management of HCM. These findings will help address gaps between current practice and guideline recommendations, improving the care of patients with HCM.

Study context

Population
Patients with hypertrophic cardiomyopathy (HCM) enrolled at referral hospitals across Japan starting in 2016patients enrolled by December 2019
Participants
1,485 patients
Outcome
Age
Follow-up
At registration and diagnosis
Methodological detailsconsecutive patients, 24 referral hospitals across Japan, baseline enrolment data
  • Consecutive patients
  • 24 referral hospitals across Japan
  • Baseline enrolment data

Main finding

As reported in the abstract

Median age at registration

69 years

Median age at diagnosis

60 years

Men

54%

Observational data: associations, not effects.

Further findings

Class IIa primary-prevention defibrillator recommendation in the whole cohort, and defibrillator implantation among class IIa patients, by guideline · at registration

Class IIa recommendation, whole cohort

Defibrillator implantation in class IIa

Familial HCM

Familial HCM confirmed
18%

HCM phenotypes

Hypertrophic obstructive cardiomyopathy (HOCM)
36%
Mid-ventricular obstruction
8%
Apical HCM
14%
End-stage phase
4%

Atrial fibrillation · at registration

Atrial fibrillation
27%
Majority asymptomatic or with mild symptoms at registration

Prior adverse outcomes

Prior sustained ventricular tachycardia or fibrillation
6%
Heart failure requiring hospitalisation
11%
Embolic events
5%

Defibrillator implantation

Defibrillator implantation
11%

Management of patients with HOCM

Beta blockers, of patients with HOCM
90%
Cibenzoline, of patients with HOCM
51%
Septal reduction therapies, of patients with HOCM
22%
Surgical myectomy, of patients with HOCM
6%

Original publication

Contemporary clinical characteristics and management patterns in hypertrophic cardiomyopathy: insights from baseline enrolment data in a nationwide prospective Japanese registry. Heart. Published 4 March 2025. doi:10.1136/heartjnl-2024-324811

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