Heart failure & cardiomyopathiesCohort study

Titin-related familial dilated cardiomyopathy: factors associated with disease onset

Eur Heart JPublished

  • Retrospective cohort study
  • international
  • multicentre
  • family-based

Authors' 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)

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

Clinical risk factors and young-onset (<30 years) DCM

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

Summarised from the published abstract; every number is checked against it