What is the clinical evidence from this study?

Study design: Observational. Population: Cardiomyopathies (n=4267). Intervention: Dilated cardiomyopathy (DCM) vs. Hypertrophic cardiomyopathy (HCM). Primary outcome: 1-year mortality (p=<0.0001).

January 1, 2002Open Access

Epidemiologic and Clinical Characteristics of Cardiomyopathies in Japan. Results From Nationwide Surveys.

Q: What are the key findings of this study?

In a nationwide survey in Japan, dilated cardiomyopathy was associated with a significantly higher 1-year mortality rate (5.6%) compared to hypertrophic cardiomyopathy (2.8%).

Key Result

In a nationwide survey in Japan, dilated cardiomyopathy was associated with a significantly higher 1-year mortality rate (5.6%) compared to hypertrophic cardiomyopathy (2.8%).

Study Design

Type

Observational (n=4,267)

Multicenter

Yes

Structured PICO

Population

Japanese patients with cardiomyopathies including idiopathic dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), arrhythmogenic right ventricular dysplasia (ARVD), mitochondrial disease, Fabry's disease of the heart, and prolonged Q-T interval syndrome.

Outcome

Prevalence, 1-year mortality, and causes of deathhard clinical

Nationwide surveys in Japan reveal that DCM has a higher 1-year mortality than HCM, with congestive heart failure and arrhythmias being the respective leading causes of death.

Main Result

Absolute Event Rate: 5.6% vs 2.8%

p-value: p=<0.0001

Limitations

Survey was limited to individuals who visited hospitals, potentially missing asymptomatic subjects and underestimating true prevalence.
Did not analyze the long-term survival of the participants.
Hospital-based sampling may have biased the prevalence measured in men versus women.

Abstract

Nationwide clinico-epidemiological surveys of cardiomyopathies in Japan were carried out. Disorders surveyed included idiopathic dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), arrhythmogenic right ventricular dysplasia (ARVD), mitochondrial disease, Fabry's disease of the heart and prolonged Q-T interval syndrome. The total number of patients was estimated at 17,700 for DCM, 21,900 for HCM, 300 for RCM, 520 for ARVD, 640 for mitochondrial disease, 150 for Fabry's disease of the heart, and 1,000 for prolonged Q-T interval syndrome. The prevalence of both DCM and HCM was higher in men than women: the male-to-female ratios were 2.6 and 2.3 for DCM and HCM, respectively. Detailed data on patients with DCM or HCM were collected by a follow-up survey. In 1 year more patients with DCM (5.6%) died than with HCM (2.8%): congestive heart failure (CHF) and arrhythmias were the leading causes of death for DCM and HCM, respectively. Angiotensin converting enzyme inhibitors (64.6%) and beta-adrenergic blockers (40.9%) are commonly used to treat the CHF complicating DCM and may be associated with the clinical improvement in a significant number of DCM patients. Thus, the nationwide surveys of Japanese patients have yielded important current epidemiological and clinical information on the characteristics of cardiomyopathies in Japan.

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