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).