MS, University of Cincinnati, 2023, 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 (open full item for complete abstract)
Committee: 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)
Subjects: Medicine