Resumen
Introducción: los tumores cardíacos neonatales son una patología poco frecuente, con una incidencia aproximada del 0.017% en autopsias y del 0.31% en ecografías obstétricas. El tumor cardiaco primario más frecuente son los rabdomiomas y sus manifestaciones clínicas son muy variables. La mayoría de los pacientes no requieren manejo terapéutico a excepción que exista compromiso hemodinámico, en los cuales una opción es el manejo quirúrgico que implica alta mortalidad. Los rabdomiomas suelen asociarse con la esclerosis tuberosa, enfermedad para la cual se ha utilizado fármacos como el everolimus o sirolimus.
Caso clínico: paciente hombre recién nacido sin antecedentes de importancia, en quien a la exploración física habitual se detectó una arritmia cardiaca. Posterior a la valoración por Cardiología pediátrica se determinó que el paciente tenía extrasístoles supraventriculares, además de múltiples masas intracardiacas que, por características imagenológicas, eran compatibles con rabdomiomas, por lo que se determinó que la causa de arritmia cardiaca era la presencia de múltiples tumores. Debido a la afección al sistema de conducción se utilizó everolimus y propranolol. A los 7 días se logró el control de las arritmias y a las 8 semanas se logró una resolución de los tumores.
Conclusiones: los rabdomiomas son tumores cardiacos asociados a esclerosis tuberosa, las complicaciones clínicas pueden ser manejados de manera eficaz con everolimus como en el caso presentado.
Abstract
Background: Neonatal cardiac tumors are a rare pathology, with an approximate incidence of 0.017% in autopsies and 0.31% in obstetric echographies. The most common primary cardiac tumor is rhabdomyomas and its clinical manifestations are highly variable. Most patients do not require therapeutic management unless there is hemodynamic compromise, in which one option is surgical management, which implies high mortality. Rhabdomyomas are usually associated with tuberous sclerosis, a disease for which drugs such as everolimus or sirolimus have been used.
Clinical case: Newborn male patient with no significant history, in whom a cardiac arrhythmia was detected during the routine physical examination. After the evaluation by Pediatric Cardiology, it was determined that the patient had supraventricular extrasystoles, in addition to multiple intracardiac masses that, due to imaging characteristics, were compatible with rhabdomyomas, so it was determined that the cause of cardiac arrhythmia was the presence of multiple tumors. Due to the condition of the conduction system, everolimus and propranolol were used. After 7 days, control of the arrhythmias was achieved and resolution of the tumors was achieved after 8 weeks.
Conclusions: Rhabdomyomas are cardiac tumors associated with tuberous sclerosis, clinical complications can be managed effectively with everolimus as in the case presented.
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