title: MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery.
abstract: OBJECTIVE
  The Fontan operation is a staged palliation for complex congenital heart disease and single ventricle physiology. Perioperative survivors of the Fontan operation experience long-term cardiac complications, including death. Liver and renal dysfunction are reported in these patients and have a direct effect on morbidity and mortality. This study aims to investigate whether the Model for End-stage Liver Disease eXcluding INR score (function of creatinine and total bilirubin, MELD-XI) predicts risk for cardiac mortality or transplantation in patients with Fontan circulation.
  
  
  METHODS
  Retrospective, single-centre study. Time of first evaluation was the time of the earliest available MELD-XI score measurement, and follow-up was terminated by a cardiac event or by the last clinical evaluation.
  
  
  METHODS
  Patients surviving after Fontan surgery and evaluated at Boston Children's Hospital between 1993 and 2008.
  
  
  METHODS
  Composite endpoint of sudden death, death from congestive heart failure or cardiac transplantation.
  
  
  RESULTS
  The MELD-XI score was calculated as MELD-XI=11.76(loge creatinine)+5.112(loge total bilirubin)+9.44. Ninety-six patients were included (52 male, median age 26 years). After a mean follow-up period of 5.7 years, 18 patients (19%) experienced the composite end point. Baseline MELD-XI score was independently and directly related to the incidence of the composite endpoint (HR for high MELD-XI score group of 7.76, 95% CI 2.05 to 29.33, p=0.008).
  
  
  CONCLUSIONS
  Fontan patients with a higher MELD-XI score have shorter freedom from sudden cardiac death, death from congestive heart failure and cardiac transplantation.
authors: Department of Cardiology, Boston's Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA. gabriele.egidyassenza@cardio.chboston.org
authors: Gabriele Egidy
authors: GE
authors: Assenza
authors: Dionne A
authors: DA
authors: Graham
authors: Michael J
authors: MJ
authors: Landzberg
authors: Anne Marie
authors: AM
authors: Valente
authors: Michael N
authors: MN
authors: Singh
authors: Aamir
authors: A
authors: Bashir
authors: Susan
authors: S
authors: Fernandes
authors: Koenraad J
authors: KJ
authors: Mortele
authors: Chinweike
authors: C
authors: Ukomadu
authors: Massimo
authors: M
authors: Volpe
authors: Fred
authors: F
authors: Wu
mesh_terms: D000293:Adolescent; D000328:Adult; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001900:Boston; D002648:Child; D003404:Creatinine / Q000097:blood; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D058625:End Stage Liver Disease / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality*; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality*; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000601:surgery*; D006333:Heart Failure / Q000097:blood / Q000209:etiology / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality*; D006439:Hemodynamics; D006776:Hospitals, Pediatric; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D015415:Biomarkers; D003404:Creatinine; D001663:Bilirubin