Heart failure & cardiomyopathies
Differences in Disease Trajectory, Comorbidities, and Mortality in Sarcomeric and Nonsarcomeric Hypertrophic Cardiomyopathy
Authors' conclusion
Sarcomeric HCM is associated with worse prognosis
Study context
- Population
- Genotyped patients with hypertrophic cardiomyopathy in the Sarcomeric Human Cardiomyopathy registry (SHaRe)median follow-up 5.3 years
- Participants
- 6,120 patients
- Exposure
- Sarcomeric vs nonsarcomeric hypertrophic cardiomyopathy (HCM)
- Sarcomeric HCMn = 3,082
Methodological detailsregistry-based (SHaRe)
- Registry-based (SHaRe)
Main finding
As reported in the abstract
Age-standardized incidence of CV events
ASI ratio
- AF
- 1.28 (CI 1.16–1.40)
- Left ventricular (LV) systolic dysfunction
- 1.31 (CI 1.15–1.48)
- Ventricular arrhythmias
- 1.37 (CI 1.17–1.52)
Observational data: associations, not effects.
Further findings
Age at diagnosis
P < 0.001
All-cause mortality
P = 0.20
| Outcome | HR (CI) | P value | |
|---|---|---|---|
| HCM-related mortality | 1.61 (1.18–2.20) | ||
| AF as disease modifier (both groups) — AF vs No AF · LV systolic dysfunction | 2.54 (2.07–3.11) | ||
| AF as disease modifier (both groups) — AF vs No AF · ventricular arrhythmias | 3.13 (2.36–4.20) | ||
| AF as disease modifier (both groups) — AF vs No AF · mortality | 1.94 (1.64–2.31) | ||
| 0.20.5125Hazard ratio (log scale) |
- All-cause mortality — Sarcomeric HCM died younger (mean)
- 7.8 years; P<0.001
- All-cause mortality — Life-years lost between ages 44 and 85 (model-based)
- 3.5
- AF as disease modifier (both groups) — Genotype interaction: larger impact of AF and LV systolic dysfunction in sarcomeric HCM; effect ratio up to (severe HF)
- 1.98
- AF as disease modifier (both groups) — Genotype interaction: effect ratio up to (mortality)
- 2.01
- AF as disease modifier (both groups): interaction both P<0.01
Original publication
Differences in Disease Trajectory, Comorbidities, and Mortality in Sarcomeric and Nonsarcomeric Hypertrophic Cardiomyopathy. Circulation. Published 9 March 2026. doi:10.
Summarised from the published abstract; every number is checked against it