Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

ARVD

Thank you Dr. Layoun for an excellent presentation on arrhythmogenic right ventricular cardiomyopathy/dysplasia

Teaching Points:

  • ARVD is a genetic cardiomyopathy with mutations in desmoglein-2, desmoplakin, desmocollin-2, plakophilin, etc
  • Characterized by life-threatening ventricular arrhythmias (monomorphic VT)
  • ECG: QRS prolongation (in right precordial leads), RBBB morphology, Epsilon Wave (distinct wave between QRS and T waves, seen in precordial leads, V1 is the best spot)
  • Treatment: refrain from high intensity exercise, low dose beta-blocker, ICD for secondary prophylaxis in patients with history of VT or VF

Further Reading:
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review

Evidence Base Medicine:
Treatment of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): An International Task Force Consensus Statement