Heart failure & cardiomyopathies
Penetrance and Prognosis of MYH7 Variant-Associated Cardiomyopathies: Results From a Dutch Multicenter Cohort Study
Authors' conclusion
MYH7 variants can cause cardiomyopathies and MCEs at a young age.
Study context
- Population
- Carriers of (likely) pathogenic MYH7 variants30.1% index patients
- Participants
- 581 carriers
- Exposure
- Carriers of noncompaction cardiomyopathy (NCCM)-, dilated cardiomyopathy (DCM)- and hypertrophic cardiomyopathy (HCM)-associated MYH7 variants
- Outcome
- Penetrance and major cardiomyopathy-related events (MCEs)
Main finding
As reported in the abstract
Early penetrance and MCEs (age <12 years)
Early penetrance
Early MCEs
Observational data: associations, not effects.
Further findings
Prognostic factors for penetrance
| Adjusted HR (95% CI) | P value | ||
|---|---|---|---|
| Converter region variant carriers | 1.87 (1.15–3.04) | P = 0.012 | |
| Age ≤1 year in NCCM- or DCM-associated variant carriers | 21.17 (4.81–93.20) | P < 0.001 | |
| Family history of early MCEs | 2.45 (1.09–5.50) | P = 0.030 | |
| 0.050.21520Adjusted hazard ratio (log scale) |
Prognostic factors for MCE
| Adjusted HR (95% CI) | P value | ||
|---|---|---|---|
| Family history of early MCEs | 1.82 (1.15–2.87) | P = 0.010 | |
| Age ≤5 years in NCCM- or DCM-associated variant carriers | 38.82 (5.16–291.88) | P < 0.001 | |
| 0.050.21520Adjusted hazard ratio (log scale) |
Original publication
Penetrance and Prognosis of MYH7 Variant-Associated Cardiomyopathies: Results From a Dutch Multicenter Cohort Study. JACC Heart Fail. Published 9 August 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →