Arrhythmogenic Right Ventricular Cardiomyopathy: Diagnosis, Risk Stratification, and Treatment

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.