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

Federica Iezzi, Andrea Quarti, Alessandro Capestro, Francesca Chiara Surace, Marco Pozzi

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)197-201
Number of pages5
JournalInternational Journal of Surgery Case Reports
Volume44
DOIs
StatePublished - 2018

Keywords

  • Diffuse rhadbomyomatosis
  • Rhabdomyoma-like cells
  • ST-elevation

ASJC Scopus subject areas

  • Surgery

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