Heart failure & cardiomyopathiesCohort study

Individualized Family Screening for Arrhythmogenic Right Ventricular Cardiomyopathy

J Am Coll CardiolPublished

  • Prospective cohort study
  • registry-based
  • external replication cohort

Authors' conclusion

Symptomatic relatives, those 20 to 30 years of age, and those with borderline ARVC have a higher probability of developing definite ARVC.

Study context

Population
At-risk relatives without definite arrhythmogenic right ventricular cardiomyopathy (ARVC) by 2010 task force criteria, Netherlands Arrhythmogenic Cardiomyopathy Registry123 with follow-up
Participants
136 relatives
Exposure
Borderline ARVC vs possible ARVC
  • Borderline ARVCn = 43
  • Possible ARVCn = 93reference
Outcome
Development of definite ARVC (predictors and probability)

Main finding

As reported in the abstract

Probability of developing definite ARVC

1-year probability

3-year probability

  • Borderline ARVC vs Possible ARVC: P < 0.01
Definite ARVC developed after 8.1 years (IQR: 4.2-11.4 years)
41 (33%)
Symptomatic subjects had higher hazard of definite ARVC, independent of baseline phenotype
P = 0.014
Age 20 to 30 years had higher hazard of definite ARVC, independent of baseline phenotype
P = 0.002

Observational data: associations, not effects.

Further findings

External replication (Italian cohort)

Comparable results
P > 0.05

Original publication

Individualized Family Screening for Arrhythmogenic Right Ventricular Cardiomyopathy. J Am Coll Cardiol. Published 18 May 2023. doi:10.1016/j.jacc.2023.05.005

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