Heart failure & cardiomyopathiesCohort study

Monitoring of Myocardial Involvement in Early Arrhythmogenic Right Ventricular Cardiomyopathy Across the Age Spectrum

J Am Coll CardiolPublished

  • Longitudinal cohort study
  • computational modelling (Digital Twins)

Authors' conclusion

Disease progression was similar in all age groups, and sustained VA also occurred in patients aged >50 years without overt ARVC phenotype at first evaluation.

Study context

Population
Patients with early-stage arrhythmogenic right ventricular cardiomyopathy (ARVC) and genotype-positive relatives without overt structural disease and VA at first evaluation313 echocardiographic assessments
Participants
82 patients
Exposure
Age <30 years vs 30 to 50 years vs ≥50 years
Outcome
Myocardial disease progression by longitudinal biventricular deformation

Main finding

As reported in the abstract

Longitudinal strain progression · 6.7 ± 3.3 years of follow-up

LV global longitudinal strain deterioration, similar in all age groups (%-point per year)

0.1 (95% CI: 0.05-0.15)

RV lateral wall longitudinal strain worsening, all age groups (%-point per year)

0.6 (95% CI: 0.46-0.70)

Observational data: associations, not effects.

Further findings

Digital Twin RV lateral wall

Local differences in myocardial contractility, compliance and activation delay

Safety

VA during follow-up

Patients with VA during follow-up
6

Original publication

Monitoring of Myocardial Involvement in Early Arrhythmogenic Right Ventricular Cardiomyopathy Across the Age Spectrum. J Am Coll Cardiol. Published 29 August 2023. doi:10.1016/j.jacc.2023.05.065

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