Arrhythmias & electrophysiology

Clinical Presentation and Long-Term Outcomes of Late-Onset Symptomatic Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

JACC Clin ElectrophysiolPublished Retrospective cohort study

Authors' conclusion

Clinical onset of ARVC after age 40 years is usually less severe than early-onset disease, despite similar structural phenotype, and is associated with slower VTs and less VT storm, and appears to have a lower risk of progression to clinical heart failure.

Study context

Population
Probands with definite ARVC (2010 Task Force criteria) and symptomatic arrhythmias at initial presentation, 2004–2024
Participants
76 probands
Exposure
Late-onset vs early-onset symptomatic ARVC
  • E-ARVCn = 50reference
  • L-ARVCn = 26
Outcome
Presentation with cardiac arrest or syncope

Main finding

As reported in the abstract

P = 0.003

Observational data: associations, not effects.

Further findings

VT cycle length · mean follow-up

P < 0.001

Electrical storm · mean follow-up

P = 0.004

ICD therapies · mean follow-up

P < 0.001

HF · mean follow-up 11.1 years · lower incidence in L-ARVC; printed 46.0% vs 19.2%
P = 0.028

HF · mean follow-up 11.1 years

Lower incidence of HF in L-ARVC

VT ablation success

Similar success rates

Original publication

Clinical Presentation and Long-Term Outcomes of Late-Onset Symptomatic ARVC. JACC Clin Electrophysiol. Published 6 August 2025. doi:10.1016/j.jacep.2025.06.011

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