Use of Everolimus to treat cardiac rhabdomyomas and incessant arrhythmias in a newborn: Benefits and complications

Abstract

We report treating a term neonate with tuberous sclerosis and giant rhabdomyomas who presented with incessant supraventricular tachycardia with Everolimus. The treatment was efficient in reducing tumor size and assisted as an adjunct therapy in controlling arrhythmia and limiting preexcitation. Treatment was challenged by difficulty to achieve stable drug level and limited by neutropenia as a serious side effect.

Keywords: Everolimus; Wolff-Parkinson-White; rhabdomyomas.

Figure 1

12-lead electrocardiograms: (a) Day of life 1: Sinus rhythm at 108 bpm with no evidence of preexcitation, abnormal repolarization pattern suggestive of myocardial strain. (b) Day of life 7: Sinus rhythm at a rate of 124 bpm with preexcitation pattern suggestive of a left-sided accessory pathway. (c) Day of life 10: Wide-complex tachycardia at a rate of 227 bpm, likely AVT with aberrant interventricular conduction

Figure 2

Echocardiographic monitoring of tumor size: (a) Tumor size charted based on ellipse area calculation (A = π ab in cm²). The right ventricular and left ventricular apical tumors are measured from the apical 4-chamber view. The right ventricular outflow tract and left ventricular outflow tract tumors are measured from anterior apical or subcoastal views. The first time-point for right ventricular and left ventricular is from the fetal echocardiogram. Parasternal long axis view showing right and left apical rhabdomyomas (b; where the smaller left ventricular outflow tract tumor is seen as well) and the apical view (c) at end diastole on Day of life 7, before treatment with Everolimus. Echo images after 2 weeks of treatment with Everolimus show reduction in tumor size from the parasternal long (d) and apical (e) views