Update on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C)

Area of Science:

• Cardiology
• Genetics
• Inherited cardiovascular diseases

Background:

• Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic heart condition causing dangerous arrhythmias and heart dysfunction.
• Current treatment strategies lack randomized trials, relying on clinical expertise and observational data.
• Management aims to prevent sudden cardiac death, slow disease progression, and support patients with a chronic illness.

Purpose of the Study:

• To outline current expert-based treatment and screening recommendations for ARVD/C.
• To emphasize the importance of accurate diagnosis and family-based screening protocols.
• To detail therapeutic interventions for managing arrhythmias and heart dysfunction in ARVD/C.

Main Methods:

• Diagnosis relies on established Task Force criteria.
• Family screening involves regular noninvasive cardiac testing for at-risk members.
• Treatment decisions are informed by clinical expertise, registry studies, and understanding of disease mechanisms.

Main Results:

• Recommendations include significant exercise reduction and discontinuation of competitive athletics for affected individuals.
• Implantable cardioverter-defibrillator (ICD) placement is generally advised for probands meeting criteria.
• Anti-arrhythmic drugs, catheter ablation (endocardial and epicardial), beta-blockers, and ACE inhibitors are key therapeutic modalities.

Conclusions:

• Accurate diagnosis and comprehensive family screening are foundational for ARVD/C management.
• A multi-faceted approach involving lifestyle modification, device therapy, pharmacotherapy, and catheter ablation is necessary.
• Ongoing genetic counseling and patient support are vital for families affected by ARVD/C.