Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients

• Main Authors: Amdani, S. (Author), Cantor, R. (Author), Daly, K.P (Author), Hurley, K. (Author), Kirklin, J.K (Author), Knecht, K. (Author), Koehl, D. (Author), Mogul, D.B (Author), Naftel, D. (Author), Shih, R. (Author), Simmonds, J. (Author), Simpson, K.E (Author), Thrush, P. (Author)
• Format: Article
• Language: English
• Published: Elsevier Inc. 2022
• Subjects: adolescent; Adolescent; child; Child; Child, Preschool; congenital heart disease; congenital heart malformation; female; Female; Fontan procedure; Fontan Procedure; Fontan transplant outcomes; Fontan-associated liver disease; heart assist device; Heart Defects, Congenital; heart transplant; heart transplantation; Heart Transplantation; Heart-Assist Devices; human; Humans; male; Male; MELD-XI; Models, Statistical; mortality; post-transplant survival; preschool child; protein losing gastroenteropathy; Protein-Losing Enteropathies; risk factor; Risk Factors; statistical model; ventricular assist device
• Online Access: View Fulltext in Publisher

 Background: Fontan physiology results in multiorgan dysfunction, most notably affecting the liver and kidney. We evaluated the utility of Model for End-Stage Liver Disease Excluding INR (MELD-XI) score, a score evaluating the function of both liver and kidney to identify Fontan patients at increased risk for morbidity and mortality post–heart transplant. Methods: The Pediatric Heart Transplant Society database was queried to identify Fontan patients listed for heart transplant between January 2005 and December 2018. MELD-XI scores were calculated at listing and heart transplant. A multivariable analysis was conducted to identify risk factors for post–heart transplant mortality. Demographic, clinical characteristics, and survival differences were evaluated and compared between the high and low MELD-XI score cohorts. The impact of changing MELD-XI scores during the waitlist period on post–heart transplant outcomes was also evaluated. Results: Of 565 Fontan patients who underwent transplantation, 524 (93%) had calculable MELD-XI scores at the time of heart transplant: 421 calculable at listing and 392 calculable at listing and at heart transplant. On multivariable analysis, only MELD-XI score (squared) (hazard ratio, 1.007), history of protein-losing enteropathy (hazard ratio, 2.1), and ventricular assist device use at transplant (hazard ratio, 3.4) were risk factors for early phase post–heart transplant mortality. Patients with high MELD-XI scores at heart transplant had inferior survival post–heart transplant (P = .02); those in the high MELD-XI score cohort at wait listing and heart transplant tend to have the worst post–heart transplant survival; however, this was not significant (P = .42). Conclusions: The MELD-XI, an easily calculated score, serves as a valuable aid in identifying pediatric Fontan patients at increased risk for post–heart transplant mortality. © 2021 The American Association for Thoracic Surgery