Jorge Romero1, Michael Grushko1, David F Briceño1, Andrea Natale2, Luigi Di Biase3. 1. Division of Cardiology at Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine, 111 East 210th Street, Bronx, NY, 10467, USA. 2. Texas Cardiac Arrhythmia Institute, Austin, Texas, USA. 3. Division of Cardiology at Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine, 111 East 210th Street, Bronx, NY, 10467, USA. dibbia@gmail.com.
Abstract
PURPOSE OF REVIEW: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable form of cardiomyopathy, typically with autosomal dominant transmission, shown to be a defect in the cardiac desmosomes, with distinct regional and histopathological features. Clinically, this can ultimately result in bi-ventricular failure and/or malignant ventricular tachycardia (VT) via reentrant circuits created by patchy scar formation. We sought to review the current treatment for ventricular arrhythmias in the setting of ARVC, with particular attention to radiofrequency (RF) ablation and its varied techniques, along with potential therapies in the ablation spectrum. RECENT FINDINGS: There is underwhelming data on the effectiveness of medical therapy for ARVC-related VT, including beta-blockers and antiarrhythmic medication. Primary and secondary prophylactic implantable-cardioverter defibrillator (ICD) implantation in higher-risk patients is recommended. More recently, RF ablation has been used for ARVC-related VT. Endocardial VT ablation in this setting can produce acute success, though recurrence rate is quite high, which may be explained by the more epicardial and patchy nature of the disease. Combined endocardial-epicardial ablation has since been shown to be feasible, safe, and with significantly better acute and long-term success, particularly when combined with scar dechanneling or homogenization of the scar. However, recurrence rates are not insignificant, and ablation does not eliminate the need for ICD placement. Medical therapy for ARVC-related VT is suboptimal. RF ablation techniques including endocardial and epicardial approaches appear to have the highest success rates for ARVC-related VT. Catheter ablation of VT in ARVC patients should be considered a potentially effective strategy for eliminating frequent VT episodes and ICD shocks rather than a curative therapeutic approach, until long-term efficacy has been consistently documented. Research into the optimal mapping and ablation techniques are promising and ongoing.
PURPOSE OF REVIEW: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable form of cardiomyopathy, typically with autosomal dominant transmission, shown to be a defect in the cardiac desmosomes, with distinct regional and histopathological features. Clinically, this can ultimately result in bi-ventricular failure and/or malignant ventricular tachycardia (VT) via reentrant circuits created by patchy scar formation. We sought to review the current treatment for ventricular arrhythmias in the setting of ARVC, with particular attention to radiofrequency (RF) ablation and its varied techniques, along with potential therapies in the ablation spectrum. RECENT FINDINGS: There is underwhelming data on the effectiveness of medical therapy for ARVC-related VT, including beta-blockers and antiarrhythmic medication. Primary and secondary prophylactic implantable-cardioverter defibrillator (ICD) implantation in higher-risk patients is recommended. More recently, RF ablation has been used for ARVC-related VT. Endocardial VT ablation in this setting can produce acute success, though recurrence rate is quite high, which may be explained by the more epicardial and patchy nature of the disease. Combined endocardial-epicardial ablation has since been shown to be feasible, safe, and with significantly better acute and long-term success, particularly when combined with scar dechanneling or homogenization of the scar. However, recurrence rates are not insignificant, and ablation does not eliminate the need for ICD placement. Medical therapy for ARVC-related VT is suboptimal. RF ablation techniques including endocardial and epicardial approaches appear to have the highest success rates for ARVC-related VT. Catheter ablation of VT in ARVC patients should be considered a potentially effective strategy for eliminating frequent VT episodes and ICD shocks rather than a curative therapeutic approach, until long-term efficacy has been consistently documented. Research into the optimal mapping and ablation techniques are promising and ongoing.
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