A Metabolic, Mechanical, Multi-Organ Masterpiece: Dural Device Support Bridge to En-Bloc Heart-Liver Transplantation in Propionic Acidemia

Authors

Rebecca Juhl, Children's Mercy Kansas CityFollow
Brian Birnbaum, Children's Mercy HospitalFollow
Aliessa P. Barnes, Children's Mercy HospitalFollow
William Gibson, Children's Mercy HospitalFollow
Bhargava Mullapudi, Children's Mercy Kansas CityFollow
Beth Lang, Children's Mercy Kansas CityFollow
Megan Faseler, Children's Mercy HospitalFollow
Daniel E. Heble, Children's Mercy HospitalFollow
Victoria Urban, Children's Mercy Kansas CityFollow
Ryan T. Fischer, Children's Mercy HospitalFollow
Jennifer L. Gannon, Children's Mercy HospitalFollow
David Sutcliffe, Children's Mercy Kansas CityFollow

Publication Date

4-2023

Files

Abstract

Introduction: Propionic Acidemia (PA) is a disorder related to abnormal protein and lipid metabolism resulting in progressive neurological injury and dilated cardiomyopathy (DCM). Interventions for PA and secondary disease manifestations can require multi-organ transplantation. Herein we report the case of a child with PA and end-stage DCM requiring left ventricular assist device (LVAD) support with eventual heart-liver transplant. Case Report: A 17 year old male diagnosed in childhood with PA developed chronically progressive DCM culminating in end stage heart failure with acute decompensations. In a recurrent admission, he progressed to require dual inotropic support and systemic anticoagulation for new LV thrombus. Heart-liver transplant eligibility was confirmed, and with further clinical deterioration requiring invasive mechanical ventilation and chemical paralysis he underwent durable, intrapericardial LVAD implant as bridge to candidacy. After accomplishing discharge, he achieved intense physical and metabolic-specific nutritional rehabilitation over the span of 3 months, and he was listed for combined heart-liver transplant. In preparation for the complexities related to dual organ transplant from LVAD support, collaborative operative simulations were completed as were adaptations of post operative immunosuppressive and medical management between transplant teams. After a 3 week waitlist duration, he underwent en-bloc heart-liver transplant with successful post operative recovery to discharge within 2 weeks. Summary: Even with effective and specialized nutritional management, DCM secondary to PA can progress to end stage heart failure requiring mechanical support and organ transplantation. Successful rehabilitation via VAD and bridge to multiorgan transplantation requires diligent collaboration across transplant teams. Despite both technical and clinical challenges, successful completion of en-bloc heart-liver transplant from VAD support can be accomplished.

Disciplines

Cardiology | Gastroenterology | Nursing | Pediatrics | Pharmacy and Pharmaceutical Sciences | Surgery

Notes

Presented at the International Society of Heart and Lung Transplantation Scientific Meeting; April 2023; Denver, Colorado.

Recommended Citation

Juhl, Rebecca; Birnbaum, Brian; Barnes, Aliessa P.; Gibson, William; Mullapudi, Bhargava; Lang, Beth; Faseler, Megan; Heble, Daniel E.; Urban, Victoria; Fischer, Ryan T.; Gannon, Jennifer L.; and Sutcliffe, David, "A Metabolic, Mechanical, Multi-Organ Masterpiece: Dural Device Support Bridge to En-Bloc Heart-Liver Transplantation in Propionic Acidemia" (2023). Posters. 368.
https://scholarlyexchange.childrensmercy.org/posters/368