Clinical management of arrhythmogenic cardiomyopathy relies on implantable cardioverter defibrillators to prevent sudden cardiac death, alongside medical therapy and catheter ablation for arrhythmias.
This review summarizes current strategies for managing arrhythmogenic cardiomyopathy, highlighting ICDs as the primary lifesaving therapy alongside medical and ablation strategies for symptom control.
Arrhythmogenic cardiomyopathy (ACM) is an inheritable heart muscle disease characterised pathologically by fibrofatty myocardial replacement and clinically by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Although, in its original description, the disease was believed to predominantly involve the right ventricle, biventricular and left-dominant variants, in which the myocardial lesions affect in parallel or even mostly the left ventricle, are nowadays commonly observed. The clinical management of these patients has two main purposes: the prevention of SCD and the control of arrhythmic and heart failure (HF) events. An implantable cardioverter defibrillator (ICD) is the only proven lifesaving treatment, despite significant morbidity because of device-related complications and inappropriate shocks. Selection of patients who can benefit the most from ICD therapy is one of the most challenging issues in clinical practice. Risk stratification in ACM patients is mostly based on arrhythmic burden and ventricular dysfunction severity, although other clinical features resulting from electrocardiogram and imaging modalities such as cardiac magnetic resonance may have a role. Medical therapy is crucial for treatment of VAs and the prevention of negative ventricular remodelling. In this regard, the efficacy of novel anti-HF molecules and drugs acting on the inflammatory pathway in patients with ACM is, to date, unknown. Catheter ablation represents an effective strategy to treat ventricular tachycardia relapses and recurrent ICD shocks. The present review will address the current strategies for prevention of SCD and treatment of VAs and HF in patients with ACM.
Migliore et al. (Tue,) conducted a review in Arrhythmogenic cardiomyopathy. Clinical management strategies (ICD, medical therapy, catheter ablation) was evaluated. Clinical management of arrhythmogenic cardiomyopathy relies on implantable cardioverter defibrillators to prevent sudden cardiac death, alongside medical therapy and catheter ablation for arrhythmias.