Heart failure & cardiomyopathiesCohort study
The Cardiovascular Magnetic Resonance Phenotype of Lamin Heart Disease
- Prospective cohort study
- multicentre
Authors' conclusion
The CMR phenotype of LMNA carriers with preserved left ventricular systolic function consists of longer T2, higher serum troponin levels, higher extracellular volume, and impaired strain. CMR-derived focal fibrosis and strain biomarkers are prognostic
Study context
- Population
- LMNA carriers, dilated cardiomyopathy (DCM) with wild-type LMNA and healthy volunteers undergoing phantom-calibrated cardiovascular magnetic resonance (CMR)
- Participants
- 187 carriers
- Exposure
- Lamin+EF, Lamin-EF and DCMwt vs healthy volunteers; LMNA carriers vs DCMwt
- Lamin+EFn = 29
- Lamin-EFn = 38
- DCMwtn = 73
- Healthy volunteersn = 47reference
Main finding
As reported in the abstract
CMR phenotype and serum biomarkers
Difference (95% CI)
Lamin+EF vs Healthy volunteersphantom-normalized T2 longer
10(2–20)
Lamin+EF vs Healthy volunteersECV higher
3%(1%–6%)
Lamin-EF vs DCMwtphantom-normalized T2 longer
16(1–31)
Lamin-EF vs DCMwtextracellular volume higher
5%(1%–9%)
Observational data: associations, not effects.
Further findings
| Outcome | HR (95% CI) | P value | |
|---|---|---|---|
| MACE predictors in lamin participants (Cox regression) — Each 1% increase in global late gadolinium enhancement | 1.15 (1.02–1.30) | P ≤ 0.025 | |
| MACE predictors in lamin participants (Cox regression) — Each 1% decrease in Procrustes trajectory sizes | 1.01 (1.01–1.02) | P ≤ 0.025 | |
| 0.512Hazard ratio (log scale) |
| Outcome | Lamin+EF | Lamin-EF | DCMwt | Healthy volunteers | Estimate | P value |
|---|---|---|---|---|---|---|
| MACE (CV death, life-threatening ventricular tachyarrhythmia, heart transplantation or AV block requiring pacing) · 4 years | Not reported in the abstract | Not reported in the abstract | 6% | Not reported in the abstract | P < 0.001 |
- Major adverse cardiovascular events (MACE) (CV death, life-threatening ventricular tachyarrhythmia, heart transplantation or AV block requiring pacing): Lamin 21%
Original publication
The Cardiovascular Magnetic Resonance Phenotype of Lamin Heart Disease. JACC Cardiovasc Imaging. Published 14 May 2025. doi:10.1016/j.jcmg.2025.01.004
Summarised from the published abstract; every number is checked against it