Cardiac Rhabdomyomas as a Cause of Neonatal Arrhythmias

Excerpt

A term male newborn, 2.3 kg, born to a primigravida, by vaginal delivery was referred to authors’ unit because of respiratory distress. At the time of admission, heart rate was 220/min, with low volume pulses and ECG showed supraventricular tachycardia (SVT) (Fig. 1a). Sinus rhythm was restored after IV adenosine. General examination showed ash leaf macules (Fig. 1b) over the lumbosacral region, left hand and shoulder. Echocardiography showed multiple cardiac rhabdomyomas (Fig. 1c) in both ventricles with no significant obstruction to blood flow. The baby was started on propranolol, furosemide, and digoxin. On day 3 of admission, the baby had an increase in the number of hypopigmented patches and the size of rhabdomyomas, with another episode of SVT. Baby was started on sirolimus. The baby had malignant ventricular tachycardia (VT) on day 8 of life and could not be revived. Exomic sequencing was positive for TS 2 gene on chromosome 16. …