Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a characterised by fibrofatty replacement of the right and sometimes left ventricles, and which predisposes individuals to ventricular arrhythmias. Around half of ARVC cases are familial.

Evaluation begins with a full clinical and family history and physical examination along with echocardiography, and additional imaging with fluorodeoxyglucose PET-CT and cardiac MRI is crucial. Pharmaceutical therapy is aimed at targeting traditional neurohumeral mediators to achieve recovery of ejection fraction, and immunosuppressants are used to directly treat inflammation. Pharmaceutical treatment alone is successful in many patients, but some will require further invasive management with electrophysiological study and radiofrequency catheter ablation.