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CASE REPORT article
Front. Pediatr.
Sec. Pediatric Critical Care
Volume 13 - 2025 | doi: 10.3389/fped.2025.1542513
Case Report: Complex Cardiac Arrhythmia Management in the ICU for an Adolescent with Friedreich Ataxia
Provisionally accepted- 1 Department of Pediatric and Adolescent Medicine, College of Medicine and Science, Mayo Clinic, Rochester, Massachusetts, United States
- 2 Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, Michigan, United States
- 3 Division of Pediatric Critical Care, Mayo Clinic, Rochester, United States
- 4 Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States
Friedreich ataxia is a progressive neurodegenerative disorder with specific clinical manifestations, such as scoliosis, which may impact the management of cardiac arrhythmias and heart failure.A 17-year-old male with Friedreich ataxia and hypertrophic cardiomyopathy presented with atrial flutter and resultant acute-on-chronic systolic and diastolic heart failure with reduced ejection fraction. The arrhythmias were refractory to medical management with adenosine and amiodarone. An attempted cavotricuspid isthmus ablation was unsuccessful due to abnormal cardiac positioning caused by severe scoliosis. Despite optimization with dofetilide and metoprolol, he was readmitted with recurrent atrial arrhythmias and cardiogenic shock, secondary to probable amiodarone-induced thyrotoxicosis, requiring extracorporeal membrane oxygenation. His clinical course involved multisystem complications, prolonged hospitalization, and disease progression, with no recovery in systolic function despite control of his arrhythmia burden.Intensivists should be cognizant of multi-system complications that can arise when treating refractory cardiac arrhythmias, especially in those with concomitant genetic conditions.
Keywords: arrhythmia, ICU, Friedreich, Ataxia, ECMO, pediatric
Received: 10 Dec 2024; Accepted: 04 Apr 2025.
Copyright: © 2025 Fashina, Gleich, Opp, Ouellette and Kawai. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Oluwatomini A Fashina, Department of Pediatric and Adolescent Medicine, College of Medicine and Science, Mayo Clinic, Rochester, Massachusetts, United States
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