Initial Presentation:
A 21-year-old physically fit male emergency medical technician trainee with recurrent pre-syncope presented to our emergency department for an episode of chest pain with near syncope in March 2021. He was in his usual state of health when he began to experience transient substernal chest pain while rigorously biking in cold weather with associated lightheadedness, diaphoresis, and blurred vision. He reported similar symptoms in the past with palpitations and dyspnea on exertion. He denied any other toxic habits and urine toxicology screen was negative. He notably reported a family history of ventricular tachycardia for which his mother (at age 40) had received an implantable cardiac defibrillator (ICD). He had seen a cardiologist prior where work up for thyroid disease and holter monitoring were unremarkable.
On initial admission, his peak Troponin I was minimal (Table 1). ECG showed normal sinus rhythm without any acute ST/T wave changes (Image 1). Transthoracic echocardiogram revealed normal diastolic filling pattern, right sided pressures, and LV systolic function with an Ejection Fraction (EF) 55-60% and no regional wall motion abnormalities. He had no events on telemetry. An exercise stress test was performed but stopped due to hypotension (BP 70/50), sinus tachycardia (190 bpm) and near syncope at Bruce stage 5 (maximal exercise). There were no significant arrhythmias or evidence of ischemia during exercise and very rare PVCs were noted in recovery. The patient was subsequently discharged with outpatient telemetry monitoring, which revealed infrequent ventricular ectopy/PVCs, with 2 episodes of brief PVC in couplets and triplets.
The patient underwent a CMR scan (initial scan- three months from initial presentation) which was a grossly normal study without evidence of myocardial fibrosis, infiltrative disease, and did not meet criteria for ARVC (Image 3).