PMC9280109_01
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
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F1
A change in his ECG while in sinus rate was noted with a WPW pattern and evidence of a left-sided accessory pathway [Figure 1].
Echocardiographic monitoring of tumor size: (a) Tumor size charted based on ellipse area calculation (A = pi 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
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F2
A repeat echocardiogram the next day showed an interval growth in tumor size [Figure 2]. ECG evidence of flecainide toxicity (wide QRS without evidence of preexcitation) and short bursts of atrial ectopy, the flecainide dose was decreased (82 mg/m2/day) and propranolol was added (2 mg/kg/day). Echocardiogram at day of life 15 showed a significant decrease in the size of his tumors that continued through the time of his therapy [Figure 2].