see also:

• syncope / near syncope
• patterns of ECGs
• 2005 Gene Reviews: Arrhythmogenic Right Ventricular Cardiomyopathy Overview

• genetic disease of desmosome dysfunction resulting in the progressive replacement of the RV myocardium by fatty fibrous tissue which slows cardiac impulse conduction through the RV
• may account for 3-5% of sudden cardiac death under the age of 65 years as it may cause exercise or catecholamine-induced LBBB pattern Right Ventricular Outflow Tract Tachycardia (RVOT) and ventricular fibrillation (VF), while survivors often develop RV failure

• Epsilon wave - a small positive deflection at the very end of the QRS complex
  • Fontaine bipolar precordial leads (F-ECG) are used to increase the sensitivity of epsilon wave detection
• T wave inversion in V1-3
• localized QRS widening and prolonged S-wave upstroke in V1-V3
• see https://litfl.com/arrhythmogenic-right-ventricular-dysplasia-arvd/

• a cyclic-AMP-mediated form of VT that is classically triggered by exercise or catecholamine excess
• ventricular tachycardia (VT) with a LBBB pattern
  • this needs to be differentiated from:
    • supraventricular tachycardias (SVT) with LBBB
    • supraventricular tachycardias (SVT) with WPW
    • see https://lifeinthefastlane.com/ecg-library/basics/vt_vs_svt/
• 2 main causes of RVOT:
  • Idiopathic RVOT these patients have structurally normal hearts, more common in women, generally benign
  • ARVD

• dilated hypokinetic RV
• dilated RV outflow tract

• patients with high risk features require urgent insertion of an implantable cardioverter-defibrillator (ICD)
• anticoagulation to prevent RV thrombus formation
• beta adrenergic blockers such as sotalol to reduce catecholamine-triggered VT/VF