Tuberous sclerosis complex (TSC) is a neurocutaneous disorder characterized by benign tissue hamartomas in multiple organ systems, including cardiac rhabdomyomas. Though prevalent in TSC, cardiac tumors are rare in children, occurring in about 0.03%–0.17%. Rhabdomyomas are the most common, accounting for 45%. When present, they are multiple and in the ventricular myocardium. Frequently, they regress and surveillance is all that is required until spontaneous regression. Intervention is necessary when life-threatening obstruction or hemodynamically significant refractory arrhythmias occur. This case highlights the course of a 6-month-old infant with TSC and cardiac rhabdomyomas who presented in refractory ventricular tachycardia (VT) with decompensation and cardiac arrest necessitating venoarterial extracorporeal membrane oxygenation (VA-ECMO), complex antiarrhythmic therapy, and ultimately implantable cardioverter-defibrillator (ICD) implantation.

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Published in Heart Rhythm Case Reports, v. 5, issue 4, p. 196-200.

© 2019 Heart Rhythm Society. Published by Elsevier Inc.

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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