Current and state of the art on the electrophysiologic characteristics and catheter ablation of arrhythmogenic right ventricular dysplasia/cardiomyopathy

摘要: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited genetic disease caused by defective desmosomal proteins, and it has typical histopathological features characterized predominantly progressive fibro-fatty infiltration of the ventricle. Clinical presentations ARVD/C vary from syncope, heart failure (HF), tachyarrhythmias, sudden cardiac death (SCD). The 2010 modified Task Force criteria were established to facilitate recognition diagnosis ARVD/C. An implantable defibrillator (ICD) remains be cornerstone in prevention SCD patients fulfilling definite ARVD/C, especially among with hemodynamically unstable tachycardia (VT), fibrillation, aborted SCD. Further risk stratification clinically valuable management borderline or possible mutation carriers family members. However, given entity heterogeneous penetrance non-uniform phenotypes, standardization clinical practice guidelines for at-risk individuals will next frontier breakthrough. Antiarrhythmic drugs are prescribed frequently experiencing frequent tachyarrhythmias and/or appropriate ICD shocks. Amiodarone recommended drug choice. Radiofrequency catheter ablation (RFCA) been demonstrated effectively eliminate drug-refractory VT efficacy prognosis RFCA via endocardial approach alone was disappointing prior era epicardial approach. In recent years, proven that integration targeting critical isthmus eliminating abnormal electrograms within diseased substrates could yield higher acute success lower recurrence during long-term follow-up. Heart transplantation final option extensive disease, biventricular HF uncontrollable hemodynamic compromise, refractory despite aggressive medical therapies.