Ewha Med J.  2022 Jul;45(3):e5. 10.12771/emj.2022.e5.

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

• Kim KH
• Ban JE

• KMID: 2532211
• DOI: http://doi.org/10.12771/emj.2022.e5

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)

Keyword

Rhabdomyoma; Tachycardia; Ectopicatrial; Tachycardia; ventricular; Tuberous sclerosis

Figure

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  Fig. 1. Transthoracic echocardiography of rhabdomyoma. (A) Huge cardiac mass (asterisks) located in both ventricle measuring 34×30 mm on apical four chamber view. Small multiple cardiac masses (arrows) were located in papillary muscle and chordae tendinae of LV. (B) Cardiac mass (asterisks) protruded into the left ventricular (LV) outflow tract, potentially LV outflow tract obstruction on parasternal long axis view. RV, right ventricle; RA, right atrium; LA, left atrium; LV, left ventricle; AAo, ascending aorta.

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  Fig. 2. Brain ultrasound image in neonate with tuberous sclerosis. Multiple cortical and subcortical tuber (arrows) in fronto-parieto-occipital area are showed in (A) coronal view and (B) parasagittal view of right and left brain.

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  Fig. 3. 12 leads surface ECG showing wide QRS tachycardia. (A) Initial ECG shows atrial tachycardia (arrows) with nonsustained ventriculat tachycardia (asterisks). (B) After amiodarone infusion, nonsustained atrial tachycardia (arrows) and (C) frequent atial premature complex with bigeminy (arrows) are shown.

Reference

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