Hematopoietic Stem Cell Transplantation in a Fontan Patient With Myelodysplastic Syndrome.

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.