Heart failure & cardiomyopathies
Titin-related familial dilated cardiomyopathy: factors associated with disease onset
Main finding · DCM (lifetime risk / onset)Result — go to the resultOdds ratio 21.21 95% CI 14.80–30.39Authors' conclusion
Disease onset in TTNtv-associated familial DCM is dependent on individual patient context and is potentially modifiable by risk factor management and prophylactic therapeutic intervention.
Study context
- Population
- Families with TTNtv-related dilated cardiomyopathy (DCM)1,043 families
- Participants
- 3,158 participants
- Exposure
- TTNtv-positive vs TTNtv-negative subjects; clinical risk factor profiles
- Outcome
- DCM (lifetime risk and onset)
Methodological detailsinternational, multicentre, family-based
- International
- Multicentre
- Family-based
Main finding
DCM (lifetime risk / onset)
Odds ratio
21.21
95% CI 14.80–30.39
TTNtv-positive
OR 21.21 (95% CI 14.80–30.39)
As reported in the abstract
OR
- Presence of clinical risk factors associated with earlier DCM onset
- 3.41 (95% CI 2.06-5.64)
- Prior history of AF: two-fold increased odds of DCM
- 2.05 (95% CI 1.27-3.32)
- Beta-adrenergic receptor or renin-angiotensin system-blocking drugs prior to overt DCM: 87% reduced odds of DCM
- .13 (95% CI .08-.23)
Observational data: associations, not effects.
Further findings
- Prevalence of clinical risk factors increased with age; strength of DCM association greatest for young-onset (<30 years) disease
- OR 4.75 (95% CI 2.35-9.60)
Original publication
Titin-related familial dilated cardiomyopathy: factors associated with disease onset. Eur Heart J. Published 11 August 2025. doi:10. 1093/ eurheartj/ ehaf380
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