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.
Highlights
arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death/arrest (SCD/A) in young people and athletes [1]
Patients with hypertension and left ventricle (LV) heart failure tended to be treated with after their first discharge from our hospital (ACEI)/ARB, and bi-ventricular dysfunction was more frequently observed in the ACEI/ARB group than in the non-ACEI/ARB group (P < 0.001)
The abnormal wall stretch caused by cardiac dysfunction may contribute to arrhythmogenesis through a process known as mechanoelectrical feedback [20], which corroborates our findings that lower levels of tricuspid annular plane systolic excursion (TAPSE) and RVEF and higher levels of NT-proBNP are associated with increased risk of life-threatening ventricular arrhythmias (VA) events
Summary
ARVC is a leading cause of sudden cardiac death/arrest (SCD/A) in young people and athletes [1]. It is characterized by progressive replacement of the myocardium with fibrofatty tissue in the right ventricle (RV) and left ventricle (LV) in some cases [2]. Some evidence suggests that the progressive nature of ARVC and persistent deterioration of the RV is associated with an increased risk of VA events [9,10,11] These findings indicate that therapies focused on delaying disease progression may offer a new approach for reducing the VA risk; no validated drug therapy has been established to decelerate disease progression. Despite the progressive nature of ARVC, therapeutic options focusing on decelerating disease progression are scarce
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