Evolution of a rare ECG pattern in an aggressive case of neonatal tuberous sclerosis complex

Abstract

Introduction: Rhabdomyomas are the most frequent cardiac tumors in children. Furthermore, they are often associated to tuberous sclerosis complex, an autosomal dominant neurocutaneous disorder characterized by tumor-like malformations that involve many organ systems.

Presentation of the case: We describe a rare ECG pattern in a severe case of neonatal tuberous sclerosis complex.

Discussion: In the presence of significant rhabdomyomatosis related to tuberous sclerosis, multiple clusters of rhabdomyoma-like cells can infiltrate the myocardium, with increased fibrosis areas.

Conclusion: Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up is sufficient in hemodynamically stable cases. Destruction of the conduction system, with arrhythmias as consequence, can be the presenting feature of diffuse rhabdomyomatosis.

Keywords: Diffuse rhadbomyomatosis; Rhabdomyoma-like cells; ST-elevation.