title: Isolated heart transplant and combined heart-liver transplant in adult congenital heart disease patients: Insights from the united network of organ sharing.
abstract: METHODS
  The aging patient with severe congenital heart disease (CHD) faces many challenges: heart failure, arrhythmia, and in the Fontan patient, liver disease. Our goal was to define combined heart liver transplant (CHLT) and isolated orthotopic heart transplant (OHT) outcomes in U.S. adult CHD patients. The U.S. United Network for Organ Sharing (UNOS) thoracic and liver databases were queried for cardiac and CHD diagnoses, from inception-2014.
  
  
  RESULTS
  In CHLT, CHD made up 22% of waitlist patients (non-CHD n=262 vs. CHD n=58), and 20% of transplanted patients (non-CHD n=137 vs. CHD n=27). Liver function tests in the non-CHD and CHD groups were similar and there was no difference in CHD and non-CHD survival (HR 0.93, CI: 0.36-2.38, p 0.48). In isolated OHT, CHD patients comprised 2% of those listed (non-CHD n=74,080 vs. CHD n=1599) and transplanted (non-CHD n=48,985 vs. CHD n=967) and had higher early (<1year) mortality (HR 1.36, CI: 1.18-1.57, p<0.0001), but better long-term survival (HR 0.66, CI; 0.57-0.76, p<0.001) than non-CHD. Both groups benefitted from mechanical support when used (non-CHD HR 0.34, CI: 0.31-0.37 and CHD HR 0.14, CI: 0.03-0.58) and prior sternotomy had no effect on mortality in CHD (HR 0.63, CI: 0.15-2.58).
  
  
  CONCLUSIONS
  Survival of CHD patients undergoing CHLT is no different than in non-CHD, encouraging consideration of CHLT when clinically appropriate. Short-term mortality is higher in CHD (vs. non-CHD) patients undergoing OHT, regardless of prior cardiac surgery status. Modifications to CHD classification within UNOS would help better understand CHD CHLT and OHT outcomes.
authors.affiliation: The Ohio State University, 473 W 12h Avenue Suite 200, Columbus, OH 43210, USA; Nationwide Children's Hospital, 700 Children's Drive T3, Columbus, OH 43205, USA. Electronic address: elisa.bradley@osumc.edu.
authors.forename: Elisa A
authors.initials: EA
authors.lastname: Bradley
authors.affiliation: The Ohio State University, 473 W 12h Avenue Suite 200, Columbus, OH 43210, USA; Nationwide Children's Hospital, 700 Children's Drive T3, Columbus, OH 43205, USA.
authors.forename: Krong-On
authors.initials: KO
authors.lastname: Pinyoluksana
authors.affiliation: Nationwide Children's Hospital, 700 Children's Drive T3, Columbus, OH 43205, USA.
authors.forename: Melissa
authors.initials: M
authors.lastname: Moore-Clingenpeel
authors.affiliation: Nationwide Children's Hospital, 700 Children's Drive T3, Columbus, OH 43205, USA.
authors.forename: Yongjie
authors.initials: Y
authors.lastname: Miao
authors.affiliation: The Ohio State University, 473 W 12h Avenue Suite 200, Columbus, OH 43210, USA; Nationwide Children's Hospital, 700 Children's Drive T3, Columbus, OH 43205, USA.
authors.forename: Curt
authors.initials: C
authors.lastname: Daniels
mesh_terms: D000328:Adult; D016208:Databases, Factual; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D017093:Liver Failure / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009927:Tissue and Organ Procurement; D016896:Treatment Outcome; D014481:United States; D014850:Waiting Lists; D055815:Young Adult
keywords: Combined heart-liver transplant; Congenital heart disease; Fontan; Heart transplant