Skip to Main Content
 

Global Search Box

 
 
 
 

Files

File List

Full text release has been delayed at the author's request until April 27, 2025

ETD Abstract Container

Abstract Header

Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality

Greenberg, Jason
http://rave.ohiolink.edu/etdc/view?acc_num=ucin1684772963411151

Abstract Details

2023, MS, University of Cincinnati, Medicine: Clinical and Translational Research.
Patients with heterotaxy syndrome and congenital heart disease (CHD) experience inferior cardiac surgical outcomes. Heart transplantation outcomes are understudied, however, particularly compared to non-CHD patients. Data from the United Network for Organ Sharing and Pediatric Health Information Service databases were used to identify 4,803 children (<18 years) undergoing first-time heart transplant between 2003-2022 with diagnoses of heterotaxy (n=278), other-CHD (n=2,236), and non-CHD cardiomyopathy (n=2,289). Heterotaxy patients were older (median = 5 yr) and heavier (median = 17 kg) at transplant than other-CHD (median = 2 yr and 12 kg), and younger and lighter than cardiomyopathy (median = 7 yr and 24 kg) (all p<0.001). UNOS status 1A/1 at listing was not different between groups (65-67%; p=0.68). At transplant, heterotaxy and other-CHD patients had similar rates of renal dysfunction (12% and 17%), inotropes (10% and 11%), and ventilator-dependence (19 and 18%). Compared to cardiomyopathy, heterotaxy patients had comparable renal dysfunction (9%, p=0.06) and inotropes (46%, p=0.10) but more hepatic dysfunction (17%, p<0.001) and ventilator-dependence (12%, p=0.003). Rates of ventricular assist device (VAD) were: heterotaxy-10%, other-CHD-11% (p=0.84 vs. heterotaxy), cardiomyopathy-37% (p<0.001 vs. heterotaxy). The one-year incidence of acute rejection post-transplant was comparable between heterotaxy and others (vs. other-CHD, p=0.69; vs. non-CHD, p=0.08). While overall post-transplant survival was significantly worse for heterotaxy than others (vs. other-CHD, p=0.03; vs. non-CHD, p<0.001), conditional one-year survival was comparable between heterotaxy and others (vs. other-CHD, p=0.63; vs. non-CHD, p=0.35). In conclusion, children with heterotaxy syndrome experience inferior post-heart transplant survival, although early mortality appears to influence this trend, with one-year survivors having equivalent outcomes. Given similar pre-transplant clinical status to others, heterotaxy patients are potentially under risk-stratified. Increased VAD utilization and pre-transplant end-organ function optimization may portend improved outcomes.
Scott Langevin, Ph.D. (Committee Chair)
Farhan Zafar, M.D. (Committee Member)
David Morales, M.D. (Committee Member)
Wei-Wen Hsu, Ph.D. (Committee Member)
21 p.
Medicine
Congenital heart disease; Clinical outcomes; Database; Heart transplantation; Pediatric heart transplantation; Pediatric heart transplantation

Recommended Citations

Citations

  • Greenberg, J. (2023). Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality [Master's thesis, University of Cincinnati]. OhioLINK Electronic Theses and Dissertations Center. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1684772963411151

    APA Style (7th edition)

  • Greenberg, Jason. Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality. 2023. University of Cincinnati, Master's thesis. OhioLINK Electronic Theses and Dissertations Center, http://rave.ohiolink.edu/etdc/view?acc_num=ucin1684772963411151.

    MLA Style (8th edition)

  • Greenberg, Jason. "Contemporary Outcomes of Heart Transplantation in Children with Heterotaxy Syndrome: Sub-Optimal Pre-Transplant Optimization Translates into Early Post-Transplant Mortality." Master's thesis, University of Cincinnati, 2023. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1684772963411151

    Chicago Manual of Style (17th edition)

ucin1684772963411151
© 2023, all rights reserved.
This open access ETD is published by University of Cincinnati and OhioLINK.