Abstract

HISTORY: A 16 year old female was found by a passer-by unconscious on the street. She was arousable and fully conscious by the time EMS arrived. She was taken to a local hospital and later transferred to a pediatric hospital for evaluation. She was discharged home the same day with instructions to follow up with cardiology. Six days later, she presented to PMD for complaints of persistent chest pain. Cardiac enzymes were mildly elevated. She returned to the pediatric hospital for re-evaluation. She was admitted with an abnormal ECG. Past medical history was notable for three epiosdes of sudden onset tachycardia. One epiosde occurred while walking down the stairs at home. The epiosde was sudden in onset lasting about 15 minutes with dizziness, lightheadedness and blurry vision during the episode. The other two episodes were similar but not as long. PHYSICAL EXAMINATION: HR 51 BPM; BP 105/67 mmHg CV: nl S1, S2 no murmur, rub, gallop Lungs: CTA Abd: benign DIFFERENTIAL DX: 1) Neurocardiogenic syncope 2) LQTS 3) SVT 4) CPVT 5) ARVD RESULTS: 1) Echocardiogram; normal anatomy, normal function 2) ECG (initial): sinus rhythm, LAD, T-wave inversion anterior and mid-precordial leads. Flat T-waves lateral precordial leads 3) ECG (admission): monomorphic ventricular tachycardia 4) MRI: mild-mod RV dilation with mildly dec. function; thinning of RV myocardium; delayed gadolinium enhancement consistent with fibrofatty infiltration DIAGNOSIS: 1) Arrhythmogenic right ventricular cardiomyopathy 2) Ventricular tachycardia OUTCOME: 1) RFA of RVOT VT 2) Amiodarone 200 mg daily 3) Spironolactone 25 mg daily 4) LIFEVEST-awaiting release of MRI compatible AICD

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