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Case Report: Complex cardiac arrhythmia management in the ICU for an adolescent with Friedreich ataxia

Oluwatomini A Fashina1, Stephen J Gleich2,3, Derek N Opp4

  • 1Department of Pediatrics Medicine, Mayo Clinic Children's, Rochester, MN, United States.

Frontiers in Pediatrics
|May 14, 2025
PubMed

Insights

Friedreich

Area of Science:

  • Cardiology
  • Genetics
  • Neurology

Background:

  • Friedreich ataxia (FRDA) is a progressive neurodegenerative disorder.
  • Scoliosis is a common manifestation of FRDA, complicating cardiac management.
  • FRDA patients may experience hypertrophic cardiomyopathy and arrhythmias.

Observation:

  • A 17-year-old male with FRDA and hypertrophic cardiomyopathy presented with refractory atrial flutter and heart failure.
  • Severe scoliosis impeded successful cavotricuspid isthmus ablation.
  • The patient developed cardiogenic shock, likely due to amiodarone-induced thyrotoxicosis, requiring ECMO.

Findings:

  • Treatment of refractory arrhythmias in FRDA is complex, with potential for multisystem complications.
  • Abnormal cardiac positioning due to scoliosis presents challenges for interventional procedures.
  • Amiodarone-induced thyrotoxicosis is a potential complication requiring careful monitoring.

Implications:

  • Intensivists must consider multisystem complications in FRDA patients with cardiac arrhythmias.
  • Management requires a multidisciplinary approach, addressing both cardiac and neurological aspects.
  • Genetic conditions like FRDA necessitate tailored treatment strategies for cardiovascular complications.
Abstract

Keywords:
ECMOFriedreichICUarrhythmiaataxiapediatric

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