title: Perioperative mortality is the Achilles heel for cardiac transplantation in adults with congenital heart disease: Evidence from analysis of the UNOS registry.
abstract: BACKGROUND
  Adults with congenital heart disease may present with end-stage heart failure necessitating orthotopic heart transplant (OHT). We sought to review the United Network for Organ Sharing (UNOS) experience with this unique cohort focusing on surgical outcomes and survival.
  
  
  METHODS
  From the UNOS registry, 737 adult congenital heart disease recipients (ACHDR) out of 26,993 OHT patients (2.7%) who underwent OHT were studied to analyze early and late outcomes and compared to non-congenital recipients (NCR) over a 15-year period (2000-2014).
  
  
  RESULTS
  More ACHDR underwent OHT in the recent-era (3.1%; 2010-2014) as compared to the initial-era (2.5%; 2000-2004; p = 0.03). ACHDR were more likely female (40% vs. 24%; p < 0.01), younger (35 vs. 53 years; p < 0.01), less likely to have ventricular assist devices (4.2% vs. 19.3%; p < 0.01), more likely to have class-II panel-reactive antibody >10%; p < 0.01, and were listed for a longer time (249 vs. 181 days; p < 0.01). When compared to the NCR in the same period, the ACHDR cohort had longer postoperative length of stay (27 vs. 20 days; p < 0.01), higher operative mortality (11.5% vs. 4.6% p < 0.001), higher incidence of primary graft dysfunction (4.3% vs. 2.6%; p < 0.01), and higher need for dialysis (20% vs. 9%; p < 0.01). Primary graft dysfunction is the most common cause of death in (5.8%) ACHDR. Although short-term survival is poorer, long-term survival of ACHDR was found to be equivalent or better than NCR in long term.
  
  
  CONCLUSIONS
  Perioperative morbidity and mortality adversely affects short-term survival in ACHDR. ACHDR who survive the first post-transplant year have equivalent or better long-term survival than NCR.
authors.affiliation: Cardiothoracic Surgery, Heart and Vascular Institute, UPMC, Pittsburgh, Pennsylvania.
authors.forename: Dipesh K
authors.initials: DK
authors.lastname: Shah
authors.affiliation: Cardiothoracic Surgery, University Hospitals, Cleveland, Ohio.
authors.forename: Salil V
authors.initials: SV
authors.lastname: Deo
authors.affiliation: Biostatistician, Heart and Vascular Institute, UPMC, Pittsburgh, Pennsylvania.
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Althouse
authors.affiliation: Cardiovascular Diseases, Heart and Vascular Institute, UPMC, Pittsburgh, Pennsylvania.
authors.forename: Jeffery J
authors.initials: JJ
authors.lastname: Teuteberg
authors.affiliation: Cardiothoracic Surgery, University Hospitals, Cleveland, Ohio.
authors.forename: Soon J
authors.initials: SJ
authors.lastname: Park
authors.affiliation: Cardiothoracic Surgery, Heart and Vascular Institute, UPMC, Pittsburgh, Pennsylvania.
authors.forename: Robert L
authors.initials: RL
authors.lastname: Kormos
authors.affiliation: Pediatric Cardiothoracic Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma.
authors.forename: Harold M
authors.initials: HM
authors.lastname: Burkhart
authors.affiliation: Pediatric Cardiothoracic Surgery, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.
authors.forename: Victor O
authors.initials: VO
authors.lastname: Morell
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D015331:Cohort Studies; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006333:Heart Failure / Q000209:etiology / Q000601:surgery*; D016027:Heart Transplantation / Q000401:mortality*; D006353:Heart-Assist Devices / Q000706:statistics & numerical data; D006801:Humans; D007902:Length of Stay / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D059035:Perioperative Period; D055031:Primary Graft Dysfunction / Q000453:epidemiology; D012042:Registries / Q000706:statistics & numerical data*; D012737:Sex Factors; D015996:Survival Rate; D013997:Time Factors; D009927:Tissue and Organ Procurement / Q000706:statistics & numerical data*; D016896:Treatment Outcome