Abstract: SA-PO1213
Favorable Early Outcomes After Simultaneous Heart-Kidney Transplantation from a Donor with Fabry Disease
Session Information
- Transplantation: Clinical - Complications, Pediatrics, and Multi-Organ Considerations
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2002 Transplantation: Clinical
Authors
- Sardarli, Kamil, New York University Grossman School of Medicine, New York, New York, United States
- Coley, Shana M., New York University Grossman School of Medicine, New York, New York, United States
- Husain, Syed Ali, New York University Grossman School of Medicine, New York, New York, United States
- Mattoo, Aprajita, New York University Grossman School of Medicine, New York, New York, United States
Introduction
Fabry disease is an X-linked lysosomal storage disorder caused by deficient α-galactosidase A activity resulting in glycosphingolipid accumulation within renal and cardiac tissues. Utilization of organs from donors with Fabry disease remains uncommon because of concern for donor-derived progressive allograft dysfunction. Limited data exists regarding outcomes following kidney transplantation from affected donors.
Case Description
A 61-year-old male with ischemic cardiomyopathy and chronic kidney disease stage 3b due to chronic cardiorenal syndrome underwent simultaneous heart and kidney transplantation. The donor was a 22-year-old male with known Fabry disease previously treated with agalsidase beta but nonadherent to therapy who died from suicide.
Time zero biopsies showed no significant changes by light microscopy though electron microscopy showed extensive podocyte changes consistent with Fabry disease. The recipient achieved immediate graft function with creatinine nadir of 0.9 mg/dL within one month. Immunosuppression included basiliximab induction and maintenance with tacrolimus, mycophenolate, and prednisone. 2 weeks after post-transplant, allograft sinus node dysfunction was detected requiring pacemaker implantation. Recipient α-galactosidase activity was mildly reduced at 0.063 (NL 0.074–0.457), though not within the range typically associated with classical Fabry disease, and enzyme replacement therapy was deferred.
Creatinine elevation at 20 weeks post-transplant in the setting of hemodynamic instability prompted repeat kidney biopsy which showed acute tubular injury without evidence for rejection though again with electron microscopy findings consistent with donor-derived Fabry disease. Serum creatinine stabilized at approximately 1.3 mg/dL up to the last follow-up 22 weeks post-transplant.
Discussion
This case demonstrates favorable early renal allograft outcomes despite donor-derived features of Fabry disease in a simultaneous heart-kidney transplant recipient. Prior reports suggest histologic Fabry inclusions may persist following transplantation from affected donors despite preserved graft function. Carefully selected organs from young donors with Fabry disease and minimal chronic structural injury may represent an opportunity to safely expand the donor pool while maintaining acceptable renal allograft outcomes.