23 y M presenting to ED with episode of syncope while playing soccer this afternoon. Endorses preceding palpitations, but denies any associated CP/diaphoresis/n/v/sob. EKG is as follows:
What is the diagnosis?
Arrhythmogenic Right Ventricular Dysplasia (ARVD). This is a diagnosis that is often not considered in the young patient with syncope, but is actually the second most common cause of sudden cardiac death in patients under age 35 (responsible for 20% of sudden cardiac deaths in patients less than 35 yo). It is caused by fibro-fatty tissue replacement of the right ventricular myocardium. Patients typically present with syncope, palpitations or sudden cardiac death most commonly incited by exercise. It is inherited in an autosomal dominant fashion and therefore there is typically a family history of sudden cardiac death. Pt’s who survive go on to have right ventricular failure and possibly biventricular failure as well. On EKG ARVD presents with an epsilon wave (30% of time), TWI in V1-3 (85% of time), prolonged S wave in V1-3 (95% of time), and prolonged QRS (>110 ms) in V1-3. It can deteriorate to VT with left bundle morphology as well – this is usually what produces symptoms. Further workup is usually with echo, stress testing, MRI, and holter monitoring. If there are high risk features (syncope from arrest, hx of cardiac arrest in first degree family members) an urgent ICD placement is necessary.
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