Arrhythmogenic right ventricular cardiomyopathy: contemporary challenges of diagnosis and management

Abstract

Arrhythmogenic right ventricular cardiomyopathy is a rare but potentially lethal condition that predominantly affects the right ventricle although a left or biventricular form is now recognised. The condition can present in a variety of ways from palpitations to aborted sudden cardiac death. It is characterised by the replacement of healthy myocardium with fatty infiltrates and is progressive in nature. There is no single test for this condition and diagnosis can be difficult – relying on a diagnostic criteria to aid diagnosis. This score system uses criteria based on ECG findings, evidence of arrhythmia, structural changes detected on echocardiogram and MRI, histopathology and family history. Treatment is initially medicine based, to suppress ventricular arrhythmias; however, patients may require device therapy, ablation or cardiac transplantation. There are recognised psychological implications for patients with arrhythmogenic right ventricular tachycardia and they may require support to come to terms with the changes that a diagnosis can bring.