Answer – Arrhythmogenic RV Dysplasia. This is the second most common cause of sudden cardiac death in young people.
- Physiology – RV myocardium is replaced by fibrofatty tissue that leads to conduction system abnormalities. Symptoms are caused by provoked ventricular beats or ventricular tachycardia.
- Symptoms – Consider this diagnosis in a young patient presenting with syncope, VT, CHF or new onset RV-Failure.
- ECG Findings – in general the ECG is not sensitive for this diagnosis, but there are some pathognomonic findings:
- Epsilon Waves (30% of cases) – low-amplitude electrical potentials occurring at the end of the QRS and the beginning of the ST segment. Highly specific for ARVC and reflect delayed RV activation.
- TWI in V1-V3 (54-85%)
- QRS prolongation V1-V3 (>110 ms)
- Echo – may demonstrate a dilated, hypokinetic RV with prominent apical trabeculae
- Cardiac MRI – evaluates for fibrofatty infiltration and thinning of the RV myocardium, RV aneurysms, RV dilatation, regional wall motion abnormalities.
- May require endocardial biopsy (notable for fibrofatty replacement of RV myocardium).
- Short-Term – use amiodarone, beta-blockers (sotalol preferred) to suppress ventricular arrhythmias.
- Long-Term – EP consult, ICD placement +/- radiofrequency ablation.