Differences in Disease Trajectory, Comorbidities, and Mortality in Sarcomeric and Nonsarcomeric Hypertrophic Cardiomyopathy

doi:10.1161/circulationaha.125.076036

Heart failure & cardiomyopathiesCohort study

Differences in Disease Trajectory, Comorbidities, and Mortality in Sarcomeric and Nonsarcomeric Hypertrophic Cardiomyopathy

CirculationPublished 9 Mar 2026

Multicentre longitudinal cohort study
registry-based (SHaRe)

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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

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

Sarcomeric HCMmedian 38.1 years

Nonsarcomeric HCMmedian 54.3 years

All-cause mortality

P = 0.20

Sarcomeric HCM10.4%

Nonsarcomeric HCM9.4%

Outcome	Lower risk ←→ Higher risk	HR (CI)
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:10.1161/circulationaha.125.076036

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Summarised from the published abstract; every number is checked against it