Heart failure & cardiomyopathiesDiagnostic or prognostic study
Laminopathies: natural history and risk prediction of heart failure
- Registry-based cohort study with prediction model derivation and external validation
- nationwide registry
- independent international validation cohort
- Fine-Gray competing risk model
Authors' conclusion
The first prediction model for severe HF events in adult laminopathies was developed
Study context
- Population
- Adults with LMNA gene variants (adult-onset laminopathies), French LMNA nationwide registryderivation cohort
- Participants
- 470 adults
- Comparison
- 0 risk factors vs 1 risk factor vs ≥2 risk factors
- Outcome
- HF-MACE:
- Heart failure (HF) hospitalization
- HF-related death
- Mechanical circulatory support
- Heart transplantation
Performance
As reported in the abstract
HF-MACE · median follow-up 7.1 years (IQR 3.4-12.1)
Each group vs 0 risk factorsAdjusted hazard ratio (95% CI)
Male sex
1.86(1.060–3.290)
LVEF <50%
2.18(1.080–4.400)
Missense variants in head and rod domains
2.91(1.110–7.630)
Complete left bundle branch block
2.99(1.400–6.400)
- HF-major adverse cardiac events (HF-MACE) events in derivation cohort
- 65
- C-index, derivation cohort
- 0.750 (95% CI 0.720-0.780)
- C-index, validation cohort
- 0.758 (95% CI 0.720-0.800)
Further findings
HF-MACE in patients with left ventricular ejection fraction (LVEF) <30% at baseline · 1 year
- 1-year incidence of HF-MACE
- 50%
- excluded from the risk score
Original publication
Laminopathies: natural history and risk prediction of heart failure. Eur Heart J. Published 6 March 2026. doi:10.1093/eurheartj/ehag104
Summarised from the published abstract; every number is checked against it