Cardiac rhabdomyoma presenting as left ventricular outflow tract obstruction in a neonate

Abstract

A case of congenital cardiac rhabdomyoma presenting as left ventricular outflow tract obstruction is reported. Congenital cardiac tumours are rare. Rhabdomyomas are the most common. Fifty-one to 86% of them are associated with tuberous sclerosis. They have a tendency for spontaneous regression. The indications for surgery include haemodynamic compromise and intractable arrhythmias.

Cardiac rhabdomyoma with LVOT obstruction and anorectal malformation in a neonate: A rare association

“…Left ventricular outflow tract obstruction seems to be one of the rarer presentations of cardiac rhabdomyomas in infants. 5 But unlike our patient, most of these children are ill at diagnosis. As per natural history of cardiac rhabdomyomas in infants and children, these tumours have a propensity for spontaneous regression.…”

“…5 This has important implications for therapy. Surgical intervention is indicated only in the event of haemodynamic compromise or intractable arrhythmias.…”

Tuberous sclerosis with cardiac rhabdomyoma: a case report

“…1 Mutation of either of two genes, TSC1 and TSC2, which encode for the proteins hamartin and tuberin respectively are responsible for Tuberous sclerosis complex.…”

“…These proteins act as tumour growth suppressors that regulate cell proliferation and differentiation. 1 Prevalence is estimated to be 1 in 6000 newborns. It is characterized by growth of benign tumors in brain, heart, lungs, eyes, kidneys, skin and other organs leading to seizures, intellectual disability, autism or developmental delay.…”

Intracardiac rhabdomyoma in a newborn with tuberous sclerosis

“…The variety of clinical symptoms of cardiac rhabdomyomas depends on their number, size and position. 1,3,[12][13][14] Postnatally, they may be asymptomatic or may present as asymptomatic cardiac murmur, congestive cardiac failure with low cardiac output due to intracardiac flow obstruction, [10][11][12][13][14] arrhythmias of various types 1,3,0,14 or as infant sudden death. 1,3,4,12 The dysrhythmias associated with rhabdomyomas were usually supraventricular tachycardia, Wolff-Parkinson-White syndrome, atrial flutter and fibrillation, variable atrioventricular block, ectopic atrial tachycardias, premature extrasystoles, and ventricular tachycardia.…”