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Updated: Mar 21, 2026
Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome
Published on: October 3, 2018
Hematopoietic Stem Cell Transplantation in a Fontan Patient With Myelodysplastic Syndrome
Josue Diaz-Frias1, Mark T Vander Lugt2, Timothy B Cotts3
1Division of Cardiovascular Medicine, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan, USA.
Background:
Adults with single-ventricle congenital heart disease with Fontan palliation can present with associated hematologic malignancies, with complex care implications.
Case Summary:
We present the case of a 23-year-old woman with univentricular heart disease after Fontan palliation with myelodysplastic syndrome who underwent allogenic hematopoietic stem cell transplantation (HSCT).
Discussion:
Fontan circulation leads to a delicate physiology that is susceptible to notable hemodynamic imbalances in a variety of clinical scenarios. HSCT is associated with significant hemodynamic sequelae and carries a significant risk of infection, fluid overload, and medication toxicity that may be potentiated by Fontan physiology.
Take-Home Message:
We recommend a multidisciplinary approach for decision-making and management with appropriate risk mitigation strategies for patients with Fontan physiology undergoing chemotherapy and HSCT.
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