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 Right Ventricular Outflow Tract Tachycardia (RVOT) and ventricular fibrillation (VF), while survivors often develop RV failure
ECG findings
Epsilon wave - a small positive deflection at the very end of the QRS complex
T wave inversion in V1-3
localized QRS widening and prolonged S-wave upstroke in V1-V3
Right Ventricular Outflow Tract Tachycardia (RVOT)
a cyclic-AMP-mediated form of VT that is classically triggered by exercise or catecholamine excess