Family history of sudden cardiac death (HR 1.83, CI 1.161-2.9) and positive genotype (HR 1.52, CI 1.01-2.31) were independently associated with sudden cardiac death in HCM.
Cohort (n=3,258)
Yes
Are genotype and family history of sudden cardiac death independent risk markers for sudden cardiac death in adult patients with hypertrophic cardiomyopathy?
Family history of sudden cardiac death and positive genotype are independent risk markers for sudden cardiac death in hypertrophic cardiomyopathy, suggesting genotype-negative patients with only a family history may not require ICD insertion.
Effect estimate: HR 1.83 (FHxSCD), HR 1.52 (genotype-positive) (95% CI 1.161-2.9 (FHxSCD), 1.01-2.31 (genotype-positive))
p-value: p=0.01 (FHxSCD), 0.047 (genotype-positive)
BACKGROUND There are limited data on genotype as a risk marker of sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). It remains unknown whether family history of SCD (FHxSCD) is a risk marker of SCD independently of genotype. OBJECTIVES The goal of this study was to assess the association of genotype and FHxSCD with SCD outcomes in HCM and investigate methods for incorporation of genotype into SCD risk stratification models. METHODS This historical cohort study used registries of 2 HCM referral centers. Association of FHxSCD and genotype with SCD was assessed by multivariable hazard regression analysis. RESULTS Of 3,258 patients, 896 (27.5%) were genotype-positive, and 332 (10.2%) had FHxSCD. During 4.9 years' median follow-up, 114 patients reached the SCD outcome. On multivariable analysis, the hazard ratios for SCD were 1.83 (95% CI: 1.161-2.9; P = 0.01) for FHxSCD and 1.52 (95% CI: 1.01-2.31; P = 0.047) for being genotype-positive. Among patients with FHxSCD but no other risk markers, the risk of SCD events was 6.4% at 5 years (95% CI: 2.7%-14.9%) in genotype-positive patients and 2.6% (95% CI: 0.6%-10.0%) in genotype-negative patients. CONCLUSIONS FHxSCD remained independently associated with SCD in adult patients with HCM even after adjusting for genotype. Among patients with FHxSCD but no other risk markers, genotype-positive patients were at high risk of SCD but genotype-negative patients were not. These data suggest that ICD insertion is not indicated for most genotype-negative patients with FHxSCD unless other risk markers are present.
Sakhnini et al. (Sun,) conducted a cohort in Hypertrophic cardiomyopathy (HCM) (n=3,258). Family history of sudden cardiac death (FHxSCD) and positive genotype vs. No family history of sudden cardiac death and negative genotype was evaluated on Sudden cardiac death (SCD) (HR 1.83 (FHxSCD), HR 1.52 (genotype-positive), 95% CI 1.161-2.9 (FHxSCD), 1.01-2.31 (genotype-positive), p=0.01 (FHxSCD), 0.047 (genotype-positive)). Family history of sudden cardiac death (HR 1.83, CI 1.161-2.9) and positive genotype (HR 1.52, CI 1.01-2.31) were independently associated with sudden cardiac death in HCM.