ARVC at 40+: Clinical Insights From a 20-Year Study

Hosts Mitsuaki Sawano, MD, Kentaro Ejiri, MD, and Satoshi Shoji, MD, welcome Dr. Shunsuke Uetake of Nippon Medical School Chiba Hokusoh Hospital to discuss the clinical presentation and long-term outcomes of late-onset symptomatic arrhythmogenic right ventricular cardiomyopathy (ARVC), based on a retrospective cohort study recently published in JACC: Clinical Electrophysiology. Dr. Uetake reviews findings from 76 probands with definite ARVC by 2010 Task Force criteria who presented with symptomatic ventricular arrhythmias between 2004 and 2024. Patients were divided into early-onset (≤40 years) and late-onset (>40 years) groups. Compared with early-onset disease, late-onset ARVC was associated with less severe initial presentation (palpitations more common, fewer syncope or cardiac arrest cases), slower ventricular tachycardia cycle length, and fewer arrhythmic events and heart failure progression during long-term follow-up, despite similar structural substrate on electroanatomical mapping. These results highlight that age of onset, even within the same genetic background, may shape the arrhythmic burden and clinical trajectory of ARVC, raising new questions about risk stratification and management in this rare but high-stakes cardiomyopathy.