Heart failure & cardiomyopathies

Family Screening and Disease Predictors in Gene-Elusive Dilated Cardiomyopathy

JACC Heart FailPublished Study of first-degree relatives of gene-elusive dilated cardiomyopathy (DCM) probands

Authors' conclusion

First-degree relatives of gene-elusive DCM probands have a relatively low but non-negligible risk of disease development. Familial clustering, electrocardiographic or echocardiographic abnormalities, and age of disease expression in the proband may help tailor surveillance intensity.

Study context

Population
First-degree relatives of gene-elusive (without pathogenic or likely pathogenic variants) dilated cardiomyopathy (DCM) probands who underwent baseline electrocardiography and echocardiographyrelatives (median age: 36 years, 48% male)
Participants
292 relatives
Outcome
Development of the disease, defined as DCM or isolated left ventricular (LV) systolic dysfunction (LV ejection fraction: <50%)
Follow-up
Median 52 months
Methodological details4 international referral centers, age-adjusted Cox models with family-clustered robust standard errors
  • 4 international referral centers
  • Age-adjusted Cox models with family-clustered robust standard errors

Main finding

As reported in the abstract

Age-adjusted
Hazard ratio (predictor of disease development) (95% CI)P value
Familial DCM
4.24(1.38–13.1)
P = 0.012
QRS duration >120 ms
7.24(2.25–23.3)
P < 0.001
Lower LV ejection fraction
3.73(2.46–5.65)
P < 0.001
LV dilatation
6.19(2.27–16.9)
P < 0.001
Additional relatives who developed disease
21
Incidence rate
17 per 1,000 person-years

Secondary outcomes

Affected at baseline

Relatives affected at baseline
14 (5%)

Correlation of age at diagnosis in probands with age at onset in relatives

R
0.80; 95% CI: 0.55-0.92; P < 0.001

Major adverse cardiac events (exploratory secondary outcome)

Patients with major adverse cardiac events
9 patients (4%)

Original publication

Family Screening and Disease Predictors in Gene-Elusive Dilated Cardiomyopathy. JACC Heart Fail. Published 23 October 2026. doi:10.1016/j.jchf.2026.103476

Composed by AI from the published abstract and checked against it. How Briefs are made →

Get them every week

New studies on this topic in My Week and in your Monday e-mail.

We e-mail you a sign-in link and a code. No password, no spam.