Giant Cardiac Rhabdomyoma with Mixed Atrial Tachycardia and Nonsustained Ventricular Tachycardia in a Newborn with Tuberous Sclerosis

Abstract

Cardiac rhabdomyomas are typically presented in the tuberous sclerosis. Although benign and often associated with spontaneous regression, in rare circumstances huge mass size and critical location can lead to heart failure, ventricular outflow tract obstruction and refractory tachyarrhythmias. An 1-day-old girl was diagnosed as cardiac tumor during perinatal period. At birth, transthoracic echocardiography revealed huge cardiac mass located in septal area of both ventricle measuring 34 ×30 mm. It protruded into the left ventricular (LV) outflow tract, potentially obstructing it. A surface ECG revealed atrial tachycardia with nonsustained ventricular tachycardia with heart rate of 217 beats per min. The tachyarrhythmias were controlled with intravenous amiodarone. Reduction of the giant cardiac mass was treated with mammalian target of rapamycin pathway inhibitor sirolimus. However, she unfortunately died at 10 days-old because of sudden cardiac arrest maybe due to LV outflow tract obstruction during therapy. Gene analysis revealed TSC2 mutation after death. (Ewha Med J 2022;45(3):e5)

Response to Letter to the Editor: Giant Cardiac Rhabdomyoma with Mixed Atrial Tachycardia and Nonsustained Ventricular Tachycardia in a Newborn with Tuberous Sclerosis

“…In the case, we demonstrated the multiple systemic involvement related to tuberous sclerosis [2]. Brain ultrasound showed multiple cortical and subcortical tubers on both fronto-parietooccipital area.…”

Letter to the Editor: Giant Cardiac Rhabdomyoma with Mixed Atrial Tachycardia and Nonsustained Ventricular Tachycardia in a Newborn with Tuberous Sclerosis

“…We found the article "Giant Cardiac Rhabdomyoma with Mixed Atrial Tachycardia and Nonsustained Ventricular Tachycardia in a Newborn with Tuberous Sclerosis" published by Kyung Hee Kim and Ji-Eun Ban published in your highly valued journal to be really intriguing [1].…”