Heart failure & cardiomyopathies
Genotype and Family History as Risk Markers of Sudden Cardiac Death in Hypertrophic Cardiomyopathy
Authors' conclusion
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.
Study context
- Population
- Patients with hypertrophic cardiomyopathy (HCM) at 2 HCM referral centers896 (27.5%) genotype-positive, 332 (10.2%) with FHxSCD
- Participants
- 3,258 patients
- Exposure
- Association of FHxSCD and genotype with sudden cardiac death (SCD)
- FHxSCDn = 332
- Genotype-positiven = 896
- Outcome
- SCD outcome
- Follow-up
- 4.9 years' median
Methodological detailsregistries of 2 HCM referral centers, multivariable hazard regression analysis
- Registries of 2 HCM referral centers
- Multivariable hazard regression analysis
Main finding
As reported in the abstract
Hazard ratio (95% CI)P value
FHxSCD
1.83(1.161–2.9)
P = 0.01Genotype-positive
1.52(1.01–2.31)
P = 0.047- Patients reaching the SCD outcome
- 114
Observational data: associations, not effects.
Further findings
Percent
Genotype-positive patients with FHxSCD but no other risk markers
6.4%(95% CI 2.7%–14.9%)
Genotype-negative patients with FHxSCD but no other risk markers
2.6%(95% CI 0.6%–10.0%)
Original publication
Genotype and Family History as Risk Markers of Sudden Cardiac Death in Hypertrophic Cardiomyopathy. JACC Clin Electrophysiol. Published 5 March 2026. doi:10.
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