Elsevier

JACC: Heart Failure

Volume 1, Issue 6, December 2013, Pages 459-466
JACC: Heart Failure

Mini Focus Issue: Translational Research
Sarcomere Gene Mutations Are Associated With Increased Cardiovascular Events in Left Ventricular Hypertrophy: Results From Multicenter Registration in Japan

https://doi.org/10.1016/j.jchf.2013.08.007Get rights and content
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Objectives

This study investigated the occurrence of cardiovascular events in patients with hypertensive heart disease (HHD) or hypertrophic cardiomyopathy (HCM) with or without sarcomere gene mutations.

Background

Although HHD and HCM are associated with left ventricular hypertrophy (LVH), few data exist regarding the difference in prognosis between them.

Methods

We enrolled 256 patients with LVH (>13 mm) screened for sarcomere gene mutations. We divided them into 3 groups: the first had HHD without sarcomere gene mutations (group H), the second had sarcomere gene mutations (group G), and the third had neither sarcomere gene mutations nor HHD (group NG). We compared the occurrence of sudden cardiac death, ventricular tachycardia/fibrillation, admission for heart failure, and atrial fibrillation for 1 year.

Results

Group G (n = 78, 36 men; mean age, 53.4 years) experienced more total cardiovascular events than group H (n = 45, 32 men; mean age, 67.4 years) (p = 0.042) after adjustments for age and sex, although there was no significant difference in total cardiovascular events between groups H and NG (n = 98, 66 men; mean age, 62.0 years). With Kaplan-Meier analysis, group G exhibited a significantly higher incidence of admission for heart failure (p = 0.017) and atrial fibrillation (p = 0.045) than group H in those 50 years of age and older. Additionally, there was a significant difference in total cardiovascular events between groups G and NG (p = 0.021).

Conclusions

These results demonstrate that HCM with sarcomere gene mutations can be associated with increased cardiovascular events compared with HHD or HCM without sarcomere gene mutations.

Key Words

heart failure
left ventricular hypertrophy
prognosis
sarcomere gene mutations

Abbreviations and Acronyms

AF
atrial fibrillation
BNP
plasma B-type natriuretic peptide
BP
blood pressure
ECG
electrocardiographic
HCM
hypertrophic cardiomyopathy
HHD
hypertensive heart disease
ICD
implantable cardioverter-defibrillator
LVH
left ventricular hypertrophy
MR
mitral regurgitation
VF
ventricular fibrillation
VT
ventricular tachycardia

Cited by (0)

This work was supported by a research grant for cardiovascular diseases (20C-4) from the Ministry of Health, Welfare, and Labor of Japan. The authors have reported that they have no relationships relevant to the contents of this paper to disclose. Part of this work was presented at the Annual Scientific Sessions, American Heart Association, 2012, Los Angeles, California.