title: Outcomes of Adult Patients With Congenital Heart Disease After Heart Transplantation: Impact of Disease Type, Previous Thoracic Surgeries, and Bystander Organ Dysfunction.
abstract: BACKGROUND
  Adults with congenital heart disease (CHD) are at increased risk for adverse outcomes after heart transplantation (HT). However, small cohorts have constrained the identification of factors associated with poor prognosis. We hypothesized that number of sternotomies and bystander organ dysfunction would be associated with an increased risk for early death after HT.
  
  
  RESULTS
  We performed a retrospective observational study of all adult CHD patients who underwent HT at our institution from January 1997 to January 2014. Forty-eight adult CHD patients were followed for a mean of 5 years. Diagnoses included tetralogy of Fallot/pulmonary atresia/double-outlet right ventricle in 15 (31%), D-transposition of the great arteries (TGA) in 10 (21%), tricuspid atresia/double-inlet left ventricle in 9 (19%), ventricular or atrial septal defect in 4 (8%), heterotaxy in 3 (6%), congenitally corrected TGA in 2 (4%), and other diagnoses in 5 (10%). Survival at both 1 and 5 years was 77%. According to multivariate analysis, ≥3 sternotomies (hazard ratio [HR] 8.5; P = .02) and Model for End-Stage Liver Disease Excluding International Normalized Ratio (MELD-XI) score >18 (HR 6.2; P = .01) were significant predictors of mortality. Failed Fontan surgery was not a significant predictor of death (P = .19).
  
  
  CONCLUSIONS
  In our cohort of adult CHD patients undergoing HT, ≥3 sternotomies and MELD-XI score >18 were significantly associated with death. These findings may be important in patient selection and decision regarding tolerable number of CHD surgeries before considering HT.
authors.affiliation: Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York. Electronic address: ml3329@columbia.edu.
authors.forename: Matthew
authors.initials: M
authors.lastname: Lewis
authors.affiliation: Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York.
authors.forename: Jonathon
authors.initials: J
authors.lastname: Ginns
authors.affiliation: Division of Cardiology, Department of Medicine, Center for Advanced Cardiac Care, Columbia University Medical Center, New York, New York.
authors.forename: Christian
authors.initials: C
authors.lastname: Schulze
authors.affiliation: Division of Cardiology, Department of Medicine, Center for Advanced Cardiac Care, Columbia University Medical Center, New York, New York.
authors.forename: Matt
authors.initials: M
authors.lastname: Lippel
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, Columbia University Medical Center, New York, New York.
authors.forename: Paul
authors.initials: P
authors.lastname: Chai
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, Columbia University Medical Center, New York, New York.
authors.forename: Emile
authors.initials: E
authors.lastname: Bacha
authors.affiliation: Division of Cardiology, Department of Medicine, Center for Advanced Cardiac Care, Columbia University Medical Center, New York, New York.
authors.forename: Donna
authors.initials: D
authors.lastname: Mancini
authors.affiliation: Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York.
authors.forename: Marlon
authors.initials: M
authors.lastname: Rosenbaum
authors.affiliation: Division of Cardiology, Department of Medicine, Center for Advanced Cardiac Care, Columbia University Medical Center, New York, New York.
authors.forename: Maryjane
authors.initials: M
authors.lastname: Farr
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D063305:Organ Dysfunction Scores; D012086:Reoperation / Q000009:adverse effects / Q000401:mortality; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D056346:Sternotomy / Q000009:adverse effects / Q000401:mortality; D016896:Treatment Outcome; D055815:Young Adult
keywords: Congenital heart disease; heart transplantation; mortality