Angiotensin-Converting Enzyme Inhibitors/Angiotensin Receptor Blockers: Anti-arrhythmic Drug for Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Background: Current treatment guidelines for arrhythmogenic right ventricular cardiomyopathy (ARVC) mainly emphasize on prevention of ventricular arrhythmic events. Despite the progressive nature of ARVC, therapeutic options focusing on decelerating disease progression are scarce.Methods and Results: This retrospective observational cohort study included 311 patients [age, 39.1 ± 14.4 years; male, 233 (74.9%)] with a definite diagnosis of ARVC as determined by the 2010 Task Force Diagnostic Criteria. Among them, 113 patients (36.3%) received ACEI/ARB treatment. Disease progression was evaluated according to repeat transthoracic echocardiograms with a linear mixed model. Patients receiving ACEI/ARB treatment were associated with slower disease progression reflected by a gradual decrease in tricuspid annular plane systolic excursion than those not receiving ACEI/ARB treatment (0.37 vs. 0.61 mm per year decrease, P < 0.001) and slower dilation of right ventricular outflow tract (0.57 vs. 1.06 mm per year increased, P = 0.003). Cox proportional hazard regression models were used to evaluate the association between life-threatening ventricular tachycardia events and ACEI/ARB treatment. A reduced risk of life-threatening ventricular arrhythmia was associated with ACEI/ARB treatment compared to that without ACEI/ARB treatment (adjusted HR: 0.71, 95% CI: 0.52–0.96, P = 0.031).Conclusions: ACEI/ARB treatment is associated with slower disease progression and lower risk of life-threatening ventricular arrhythmia in patients with ARVC. Delaying disease progression may pave way for reducing life-threatening ventricular arrhythmia risk.

Arrhythmogenic Cardiomyopathy: Exercise Pitfalls, Role of Connexin-43, and Moving beyond Antiarrhythmics

“…Many studies have provided experimental and clinical evidence on the efficacy of ACEIs and/or ARBs [ 142 , 143 , 144 ]. More recently, a detailed retrospective observational cohort study including 311 patients diagnosed with ACM investigated whether ACEIs and/or ARBs blockers slow ACM progression and reduce the occurrence of VAs [ 145 ]. This was the first single-center observational study to provide insights into the effects of ACEIs and/or ARBs in patients with ACM during long-term follow-up.…”

Management Strategies in Arrhythmogenic Cardiomyopathy across the Spectrum of Ventricular Involvement

“…ACEi or ARB therapy was associated with slower reductions in TAPSE, RV dilation, and ventricular arrhythmias in one retrospective study [57]. Preemptive initiation of ACEi in mouse models of ARVC type 5 (an aggressive autosomal dominant form) led to reductions in myocardial fibrosis and mortality along with less severe reduction in LV dysfunction [58].…”