Heart failure & cardiomyopathies
Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction: Insights From the SHaRe Registry
Authors' conclusion
HCM-LVSD affects ≈8% of patients with HCM. Although the natural history of HCM-LVSD was variable, 75% of patients experienced adverse events, including 35% experiencing a death equivalent an estimated median time of 8.4 years after developing systolic dysfunction.
Study context
- Population
- Patients with hypertrophic cardiomyopathy (HCM) and LV systolic dysfunction (LVEF <50%), SHaRe Registry, 11 high-volume HCM centres553 (8%) HCM-LVSD
- Participants
- 6,793 patients
- Outcome
- Composite of:
- All-cause death
- Cardiac transplantation
- LV assist device implantation
Methodological detailsmulticentre, international
- Multicentre
- International
Main finding
As reported in the abstract
Composite outcome in HCM-LVSD
- Met composite outcome
- 35%
- All-cause death
- 128
- Cardiac transplantation
- 55
- LV assist device implantation
- 9
- Clinically relevant events
- 75%
- Median time to composite outcome after recognition of HCM-LVSD
- 8.4 years
- Predictor: multiple pathogenic/likely pathogenic sarcomeric variants, HR
- 5.6 (2.3-13.5) (95% CI)
- Predictor: AF, HR
- 2.6 (1.7-3.5) (95% CI)
- Predictor: LVEF <35%, HR
- 2.0 (1.3-2.8) (95% CI)
Observational data: associations, not effects.
Further findings
Incident HCM-LVSD · 15 years
- Incidence of new HCM-LVSD
- ≈7.5%
- Predictor: greater LV cavity size, HR
- 1.1 (1.0-1.3) (95% CI)
- Predictor: wall thickness, HR
- 1.3 (1.1-1.4) (95% CI)
- Predictor: baseline LVEF 50% to 60%, HR
- 1.8 (1.2, 2.8)-2.8 (1.8-4.2) (95% CI)
- Predictor: late gadolinium enhancement on CMR, HR
- 2.3 (1.0-4.9) (95% CI)
- Predictor: pathogenic/likely pathogenic sarcomeric variant, HR
- 1.5 (1.0-2.1) (95% CI)
- Predictor: thin filament variant, HR
- 2.5 (1.2-5.1) (95% CI)
Original publication
Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction: Insights From the SHaRe Registry. Circulation. Published 31 March 2020. doi:10.
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