JACC Case Rep. 2026 Mar 19:107385. doi: 10.1016/j.jaccas.2026.107385. Online ahead of print.
ABSTRACT
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.
PMID:41854572 | DOI:10.1016/j.jaccas.2026.107385