Risk stratification in arrhythmogenic right ventricular cardiomyopathy

Area of Science:

• Cardiology
• Genetics
• Electrophysiology

Background:

• Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder causing fibrofatty replacement of the right ventricle.
• It is a primary cause of sudden cardiac death (SCD) in young individuals.
• Preventing SCD is the main therapeutic goal in ARVC management.

Purpose of the Study:

• To review the current strategies for SCD prevention in ARVC patients.
• To discuss the evolving role of implantable cardioverter-defibrillators (ICDs) in ARVC.
• To explore risk stratification methods for primary prevention in ARVC.

Main Methods:

• Review of existing literature on ARVC, SCD, and ICD therapy.
• Analysis of risk factors for SCD in ARVC patients.
• Discussion of current guidelines and emerging data on ICD implantation.

Main Results:

• ICD implantation is proven effective for secondary SCD prevention in ARVC.
• Primary prevention remains challenging due to risks of inappropriate ICD shocks and complications.
• ECG abnormalities, syncope, VT, and ventricular involvement are key predictors for risk stratification.

Conclusions:

• ICD therapy is the only proven life-saving option for ARVC patients at risk of SCD.
• Risk stratification for primary prevention is complex and often individualized.
• Further research is needed to optimize primary prevention strategies in ARVC.