Overcoming challenges in the management of arrhythmogenic right ventricular cardiomyopathy.

Overcoming challenges in the management of arrhythmogenic right ventricular cardiomyopathy. Kardiol Pol. 2020 May 19;: Authors: Kubala M, Tschabrunn C, Marchlinski DF, Marchlinski FE Abstract Arrhythmogenic right ventricular cardiomyopathy (ARVC) appears in most patients to be an inherited disease characterized by fibrofatty replacement of myocytes extending from epicardium to endocardium in the right ventricle. The disease process results in life-threatening ventricular arrhythmias and ventricular dysfunction. In the absence of a gold standard diagnostic test and despite the progress in imaging techniques, ARVC is often misdiagnosed and earlier detection of the disease is challenging. Pre-procedural identification and localization of the substrate can be determined from the analysis of surface ECG and cardiac MRI. Typically, perivalvular arrhythmogenic substrate, defined by electroanatomic mapping, is present and can be isolated to the epicardium. Ablation targets are further identified with activation, entrainment and local electrogram abnormalities based on detailed electroanatomic mapping. Extensive combined endo/epicardial ablation performed in experienced centers is frequently required to prevent VT. Catheter ablation significantly reduces the recurrences of ventricular tachycardia (VT), appropriate ICD shocks and the use of antiarrhythmic drugs and cardiac transplant as a management strategy for refractory arrhythmias is rarel...
Source: Polish Heart Journal - Category: Cardiology Authors: Tags: Kardiol Pol Source Type: research