Diagnostic and therapeutic strategies for arrhythmogenic right ventricular dysplasia/cardiomyopathy

This review tackles a rare but lethal disease — arrhythmogenic right ventricular cardiomyopathy — that has an outsized claim on our attention because it kills young, often athletic people, frequently without warning.

From the Johns Hopkins group that helped write the diagnostic criteria, it lays out the essentials: the condition is largely genetic (desmosomal mutations, PKP2 most common); intense exercise actively worsens it, making activity restriction a genuine therapy; risk stratification is hard, since most sudden-death victims were asymptomatic beforehand; and an implantable defibrillator is the only treatment that reliably prevents sudden death in high-risk patients, with catheter ablation palliative at best.

The practical takeaway is a clear suspicion-and-action framework: think of it in a young person with ventricular arrhythmia or a family history of sudden death, apply the Task Force Criteria, screen families, restrict exercise, and reserve ICDs for the genuinely high-risk.

We rate the evidence high for a review: from a world authority (Calkins) in a leading electrophysiology journal, comprehensive and practical, though narrative and weighted toward one center’s experience. Its clinical significance is high — a lethal, heritable condition where correct recognition and exercise restriction are literally lifesaving. It sits apart from the lipid-and-prevention core of this library, but speaks to the same theme: catching dangerous disease before it announces itself.