Venoarterial Extracorporeal Membrane Oxygenation and Implantable Cardioverter-Defibrillator Implantation in a Hemodynamically Unstable Infant with Ventricular Tachycardia from Multiple Cardiac Rhabdomyomas

Abstract

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