Key result
An asymptomatic newborn with multiple giant cardiac rhabdomyomas associated with tuberous sclerosis complex showed spontaneous normalization of elevated right ventricular pressure and remained asymptomatic during 6 months of follow-up.
Case Report (n=1)
This case report highlights the clinical presentation and favorable short-term natural history of giant cardiac rhabdomyomas in a newborn with tuberous sclerosis complex.
Observation may suffice for asymptomatic neonates with giant rhabdomyomas; leaves open generalizability and need for prospective data.
Primary heart tumours in the paediatric population are very rare and they range from 0.01% to 0.04%. Most are benign lesions of which about half are rhabdomyomas. Rhabdomyoma tumour diagnosis is associated with a 75-80% risk of tuberous sclerosis complex (TSC). TSC are characterised with numerous changes of hamartoma-type located in the brain, kidneys, skin and other organs including the heart. More than two-thirds of newborns with TSC present rhabdomyomas in the heart. These changes may be asymptomatic, but in some cases they may cause heart failure, arrhythmias and death. We present a case report of an infant with giant rhabdomyoma tumours in the course of TSC.
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Kwiatkowska et al. (2018) conducted a case report in Giant cardiac rhabdomyoma and tuberous sclerosis complex (n=1). Clinical observation was evaluated on Clinical status and right ventricular pressure. An asymptomatic newborn with multiple giant cardiac rhabdomyomas associated with tuberous sclerosis complex showed spontaneous normalization of elevated right ventricular pressure and remained asymptomatic during 6 months of follow-up.
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