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

Federica Iezzi1, Andrea Quarti1, Alessandro Capestro1

  • 1Department of Paediatric and Congenital Cardiac Surgery and Cardiology, Azienda Ospedaliero-Universitaria Ospedali Riuniti Ancona "Umberto I, G. M. Lancisi, G. Salesi" Ancona, via Conca n. 71, 60128 Ancona, Italy.

International Journal of Surgery Case Reports
|March 13, 2018
PubMed

Insights

Rhabdomyomas, often linked to tuberous sclerosis complex, can infiltrate the heart. A rare ECG pattern was observed in a severe neonatal case, highlighting potential conduction system destruction and arrhythmias.

Area of Science:

  • Pediatric Cardiology
  • Genetics
  • Oncology

Background:

  • Rhabdomyomas are the most common cardiac tumors in children.
  • They are frequently associated with tuberous sclerosis complex (TSC), an autosomal dominant disorder.
  • TSC involves tumor-like malformations across multiple organ systems.

Purpose of the Study:

  • To describe a rare electrocardiogram (ECG) pattern.
  • To highlight this pattern in a severe neonatal case of tuberous sclerosis complex.

Main Methods:

  • Case report of a severe neonatal tuberous sclerosis complex.
  • Electrocardiogram (ECG) analysis.

Main Results:

  • A rare ECG pattern was identified in a severe neonatal TSC case.
  • Significant rhabdomyomatosis can lead to myocardial infiltration by rhabdomyoma-like cells.
  • Increased areas of fibrosis were observed in the myocardium.

Conclusions:

  • Rhabdomyomas may spontaneously regress, necessitating close follow-up in stable cases.
  • Diffuse rhabdomyomatosis can destroy the conduction system, leading to arrhythmias.
  • This case underscores the importance of recognizing specific ECG patterns in neonatal TSC.
Abstract

Keywords:
Diffuse rhadbomyomatosisRhabdomyoma-like cellsST-elevation

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