Sarcomere Gene Mutations Are Associated With Increased Cardiovascular Events in Left Ventricular Hypertrophy. Results From Multicenter Registration in Japan.

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.