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

2021 ◽  
Vol 8 ◽  
Author(s):  
Bin Tu ◽  
Lingmin Wu ◽  
Lihui Zheng ◽  
Shangyu Liu ◽  
Lishui Sheng ◽  
...  
Keyword(s):  
Ventricular Arrhythmia ◽  
Right Ventricular ◽  
Disease Progression ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Angiotensin Receptor Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Cardiomyopathy ◽  
Life Threatening

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.

scholarly journals Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Vol 10 (1) ◽  
pp. 26-32
Author(s):  
Ryan Wallace ◽  
Hugh Calkins
Keyword(s):  
Ventricular Arrhythmia ◽  
Risk Stratification ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Young Population ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Increased Risk ◽  
Fibrofatty Tissue

Arrhythmogenic right ventricular cardiomyopathy (ARVC), also called arrhythmogenic right ventricular dysplasia or arrhythmogenic cardiomyopathy, is a genetic disease characterised by progressive myocyte loss with replacement by fibrofatty tissue. This structural change leads to the prominent features of ARVC of ventricular arrhythmia and increased risk for sudden cardiac death (SCD). Emphasis should be placed on determining and stratifying the patient’s risk of ventricular arrhythmia and SCD. ICDs should be used to treat the former and prevent the latter, but ICDs are not benign interventions. ICDs come with their own complications in this overall young population of patients. This article reviews the literature regarding the factors that contribute to the assessment of risk stratification in ARVC patients.

Secondary prevention of sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2358-2361
Author(s):  
Katja Zeppenfeld ◽  
Sebastiaan R. D. Piers
Keyword(s):  
Sudden Cardiac Death ◽  
Ventricular Arrhythmia ◽  
Secondary Prevention ◽  
Right Ventricular ◽  
Cardiac Death ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Recurrence Rates ◽  
First Line Therapy

Sustained ventricular tachycardia (VT) and (aborted) sudden cardiac death are the presenting symptoms in 23–57% and 3–13% of patients who are diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC), respectively. Implantation of an implantable cardioverter defibrillator (ICD) is recommended in patients with a history of aborted sudden cardiac death, haemodynamically poorly tolerated VT, and unexplained syncope, and should be considered in patients with haemodynamically well-tolerated sustained VT. Appropriate ICD intervention rates of up to 15%/year are observed in patients implanted for secondary prevention, the majority triggered by rapid and thereby potentially fatal monomorphic VT. Although life-saving, ICD therapy does not prevent ventricular arrhythmias, and therapeutic options to control ventricular arrhythmia burden are required. Beta blockers and sotalol are typically applied as first-line therapy, the latter mainly based on a study with serial programmed stimulation testing. Amiodarone may be superior in selected patients but data are based on small cohorts - large, prospective, observational and randomized trials are lacking. Up to 97% of ventricular arrhythmia episodes in ARVC are monomorphic VT with scar-related reentry as the dominant underlying mechanism, often involving subepicardial scar layers. Catheter ablation can result in a significant reduction of the ventricular arrhythmia burden with VT recurrence rates of 10–15%/year if a combined endocardial–epicardial ablation approach is performed in experienced tertiary referral centres.

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