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Arrhythmogenic Right Ventricular Dysplasia (ARVD) is an autosomal dominant genetic disorder characterized by fatty infiltration of the right ventricular free wall. It predisposes patients to paroxysmal ventricular arrhythmias, sudden cardiac death, and biventricular failure. ARVD is the second most common cause of sudden cardiac death in young individuals, accounting for up to 10% of cases in patients under 65 years of age.
The diagnosis of ARVD is challenging and relies on a combination of clinical, electrocardiographic, and radiological features, as defined by the 2010 Task Force Criteria. Key ECG findings include T-wave inversion in the right precordial leads (V1 to V3), the presence of an epsilon wave, localized QRS widening in V1 to V3, and a prolonged S-wave upstroke. Epsilon wave is a highly specific finding for ARVD. Ventricular ectopy with a left bundle branch block morphology and episodes of sustained ventricular tachycardia with LBBB morphology, known as right ventricular outflow tract tachycardia, are also characteristic.
Echocardiography and cardiovascular MRI are valuable imaging modalities for assessing structural and functional abnormalities of the RV.
Treatment involves arrhythmia suppression with antiarrhythmic drugs, radiofrequency ablation, and placement of implantable cardioverter-defibrillator (ICD) in high-risk patients. Heart failure management and anticoagulation are also important considerations.