Arrhythmogenic Right Ventricular Cardiomyopathy: A Comprehensive Review

Area of Science:

• Cardiology
• Genetics
• Pathophysiology

Background:

• Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart muscle disease.
• It's characterized by ventricular dilation, adiposity, and fibrosis, leading to arrhythmias and sudden cardiac death.
• Prevalence estimates vary due to genetic penetrance and underdiagnosis.

Purpose of the Study:

• To provide a comprehensive overview of ARVC.
• To discuss the genetics, pathophysiology, and clinical manifestations of ARVC.
• To review diagnostic criteria, electrophysiological and imaging findings, and current management strategies.

Main Methods:

• Review of current literature on ARVC genetics, pathophysiology, and clinical aspects.
• Analysis of diagnostic criteria, including electrophysiological and imaging modalities (ECG, cardiac MRI, echocardiography).
• Evaluation of current treatment options, including medications, devices, and ablation.

Main Results:

• ARVC diagnosis is evolving with new genetic discoveries and technological advancements.
• Diagnostic criteria have been revised to improve accuracy.
• Management strategies are multifaceted, involving medications, devices, and ablation techniques.

Conclusions:

• Understanding ARVC genetics and pathophysiology is key to diagnosis and management.
• Updated diagnostic criteria and advanced imaging improve detection rates.
• Effective management strategies aim to prevent arrhythmias and sudden cardiac death in ARVC patients.