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Arrhythmogenic Right Ventricular Cardiomyopathy: Diagnosis, Risk Stratification, and Treatment
Fabienne Kreimer1, Ardan M Saguner, Ibrahim Akin
1University Hospital of Muenster, Department of Cardiology II - Rhythmology, Muenster; University Hospital of Zurich, Department of Cardiology, Zurich; University Medical Center Mannheim, 1st Medical Department, Mannheim; Heart and Diabetes Center Bad Oeynhausen, Erich & Hanna Klessmann Institute, Bad Oeynhausen; University Hospital St. Josef Hospital Bochum, Department of Cardiology and Rhythmology, Ruhr University Bochum, Bochum; Institute of Physiology, Department for Cellular and Translational Physiology and Institute for Research and Teaching (IFL), Molecular and Experimental Cardiology, Ruhr University Bochum, Bochum.
Insights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart condition that increases sudden cardiac death risk. Diagnosis and risk stratification are key, but primary defibrillator implantation decisions remain challenging.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart disease affecting 1:2000-1:5000 individuals.
- Characterized by fatty and connective tissue replacement of the heart muscle, ARVC carries a high risk of sudden cardiac death.
- This review synthesizes current knowledge on ARVC diagnosis, risk stratification, and management.
Purpose of the Study:
- To provide an overview of the current diagnostic assessment for ARVC.
- To outline strategies for risk stratification in ARVC patients.
- To review established and emerging treatment options for ARVC.
Main Methods:
- Literature search of PubMed using keywords "ARVC" and "arrhythmogenic right ventricular dysplasia".
- Evaluation of pertinent publications including guidelines, clinical registry studies, meta-analyses, and randomized controlled trials.
Main Results:
- Diagnosis utilizes ECG, echocardiography, MRI, and genetic testing.
- The ARVC risk calculator aids in assessing ventricular arrhythmia risk and guiding defibrillator implantation decisions.
- Treatment includes lifestyle modifications (sports restriction), pharmacotherapy (beta-blockers, antiarrhythmics), and catheter ablation for refractory cases.
Conclusions:
- ARVC significantly increases the risk of sudden cardiac death.
- Accurate risk assessment, especially for primary prophylactic defibrillator implantation, remains a clinical challenge.
- Further research, including randomized controlled trials, is needed to refine management strategies.
Background:
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart disease with a prevalence of 1 : 2000 to 1 : 5000. It is characterized by the progressive replacement of heart muscle tissue by fatty and connective tissue. It is associated with a high risk of sudden cardiac death. This review presents the current state of knowledge regarding the diagnostic assessment, risk stratification, and treatment of ARVC.
Methods:
This review is based on pertinent publications retrieved by a search in PubMed using the keywords "ARVC" and "arrhythmogenic right ventricular dysplasia". Guidelines, clinical registry studies, meta-analyses, and randomized controlled trials were evaluated.
Results:
The diagnosis is established with the aid of (long-term) ECG, echocardiography, magnetic resonance imaging, and genetic tests. The ARVC risk calculator is used to assess the risk of ventricular arrhythmia. Patients' participation in sports is restricted. Beta-blockers are recommended for patients with extrasystoles or ventricular tachycardia (grade I recommendation). If beta-blockers alone have an insufficient effect, amiodarone, flecainide or sotalol can be added (grade IIa). For patients with recurrent ventricular tachyarrhythmia, catheter ablation is an option (grade IIa). While there is a clear recommendation (grade I) for defibrillator implantation for patients who have survived sudden cardiac death, the ARVC risk calculator should be used for decisionmaking in patients for whom primary prophylactic implantation is considered (recommendation grade IIa).
Conclusion:
ARVC is associated with an increased risk of sudden cardiac death. Risk assessment remains challenging in the absence of randomized controlled trials, particularly with regard to the primary prophylactic implantation of a defibrillator.
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