[Arrhythmogenic right ventricular cardiomyopathy. Etiology, diagnosis and therapy]

Area of Science:

• Cardiology
• Genetics
• Electrophysiology

Context:

• Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary inherited heart muscle disease.
• It is characterized by myocardial atrophy and replacement with fatty and fibrous tissue, predominantly affecting the right ventricle.
• ARVC typically manifests in young adulthood, with a higher prevalence in males.

Purpose:

• To describe the key features, diagnostic findings, and management strategies for arrhythmogenic right ventricular cardiomyopathy.
• To highlight the association between ARVC and ventricular tachyarrhythmias, including sudden cardiac death.
• To outline therapeutic approaches aimed at managing arrhythmias and preventing sudden death in ARVC patients.

Summary:

• ARVC involves progressive degeneration of the right ventricular myocardium, leading to fibrofatty replacement.
• Clinical manifestations include ventricular tachyarrhythmias with a left bundle branch block pattern, often triggered by exercise, and ECG abnormalities like QRS prolongation.
• Sudden cardiac death can be the initial presentation. While left ventricular involvement can occur, it rarely causes biventricular failure.

Impact:

• Early identification and tailored treatment strategies can significantly improve the long-term prognosis for patients with ARVC.
• Management focuses on antiarrhythmic drug therapy, catheter ablation, and cardioverter-defibrillator implantation to mitigate risks.
• Understanding ARVC is crucial for preventing sudden cardiac death in affected individuals and families.