Arrhythmogenic right ventricular cardiomyopathy: asymptomatic to life threatening as illustrated by the cases of two sisters

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for ll%-22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.

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

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.769138 ◽

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.

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Arrhythmogenic left ventricular cardiomyopathy

BJR|case reports ◽

10.1259/bjrcr.20190079 ◽

2020 ◽

Vol 6 (1) ◽

pp. 20190079

Author(s):

Seyedeh Mojdeh Mirmomen ◽

Andrew Jay Bradley ◽

Andrew Ernest Arai ◽

Arlene Sirajuddin

Keyword(s):

Right Ventricular ◽

Cardiac Death ◽

Arrhythmogenic Right Ventricular Cardiomyopathy ◽

Ventricular Myocardium ◽

Left Ventricular ◽

Ventricular Cardiomyopathy ◽

Cardiotoxic Effect ◽

Life Threatening ◽

Muscle Disorder ◽

The Right

Arrhythmogenic ventricular cardiomyopathy (AVC) is a heritable heart muscle disorder characterized by fibrofatty infiltration of the myocardium. Intramyocardial fat deposition is considered arrhythmogenic and predisposes patients to life-threatening arrhythmias and sudden cardiac death. The classic subtype of AVC is characterized by fibrofatty replacement of the right ventricular myocardium (i.e. arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the left ventricle may be involved as well. Predominantly left ventricular involvement by AVC is exceedingly rare and lack of specific diagnostic criteria as well as its potential cardiotoxic effect make its diagnosis challenging and of high importance.

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