Abstract

HERNANDEZ, Keerby; PUCHULU, Juanita; ZAPATA, Margarita  and  RUZ, Miguel. Cardiac rhabdomyoma: Clinical presentation of three cases and current therapeutic options. Rev. Colomb. Cardiol. [online]. 2020, vol.27, n.6, pp.611-615.  Epub Aug 04, 2021. ISSN 0120-5633.  https://doi.org/10.1016/j.rccar.2019.12.011.

Up to a few decades ago, cardiac tumours were only of academic interest given their low presentation rate. However, with the advances in cardiopulmonary, surgical management began to be possible, and more recently, they contribute to the development of new treatment strategies for these patients.

The estimated incidence of cardiac tumours is low and are generally benign with non-specific clinical signs and symptoms. In paediatrics, these tumours are associated with tuberous sclerosis, a disease with a dominant autosomal inheritance pattern, which is characterised by an alteration in cell differentiation and multiplication (hamartomas) in different systems. From a cardiac point of view, the morbidity level is determined by the size and location of the tumour within the cavity, the risk of obstructing ventricular outflow tracts, alterations in valvular function, and conduction disorders. Some patients only require clinical follow-up all their lives, as there is the risk of tumour regression. Others may benefit from surgical resection due to its haemodynamic impact or changes in cardiac rhythm. However, not everyone is a candidate for surgery, in which case, management with m-TOR inhibitors has emerged as a therapeutic alternative.

The case is presented on three neonates with tuberous sclerosis and a cardiac rhabdomyoma, diagnosed in the prenatal stage. An individualised therapeutic approach was made based on the current options available for this group of patients.

Keywords : Cardiac tumours; Rhabdomyoma; Everolimus; Newborn; Cardiac arrhythmias.

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