cardiovascular disorders
Arrhythmogenic Right Ventricular Cardiomyopathy
Key Points
Arrhythmogenic right ventricular cardiomyopathy is a genetic disorder in which myocytes are replaced by fibrofatty tissue, leading to arrhythmias and later right ventricular failure. The disorder progresses quicker in patients who engage in endurance exercise. Diagnosis is based on consensus criteria involving clinical and electrocardiographic factors, cardiac imaging, and genetic testing. First-degree family members have a significant risk of disease and require initial screening and regular testing. Treatment requires moderation of physical activity, beta blockade, and often an ICD. 