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1.
J Pediatr ; 229: 78-85.e2, 2021 02.
Article in English | MEDLINE | ID: mdl-32976893

ABSTRACT

OBJECTIVE: To describe the assessment of Fontan-associated liver disease and determine the clinical and imaging measures that may identify hepatic morbidity risk in isolated heart transplantation candidates and trend those measures post-isolated heart transplantation. STUDY DESIGN: Retrospective analysis of pre-isolated heart transplantation and post-isolated heart transplantation Fontan-associated liver disease (FALD) status using blood tests, magnetic resonance imaging (MRI), and liver biopsy analysis within 6 months before isolated heart transplantation and 12 months after isolated heart transplantation in 9 consecutive patients with Fontan. Pre- and post-isolated heart transplantation standard laboratory values; varices, ascites, splenomegaly, thrombocytopenia (VAST) score; Fontan liver MRI score; liver biopsy scores; Model for End-stage Liver Disease (MELD); MELD excluding the International Normalized Ratio (MELD-XI); AST to platelet ratio index, and cardiac catheterization data were compared. RESULTS: Pretransplantation maximum MELD and MELD-XI was 15 and 16, respectively. Central venous pressures and VAST scores decreased significantly post-transplantation. In 5 paired studies, Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation. Arterially enhancing nodules on MRI persisted in 2 patients post-transplantation. Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens. CONCLUSIONS: Patients with FALD and MELD <15, MELD-XI <16, Fontan liver MRI score <10, and VAST score ≤2 can have successful short-term isolated heart transplantation outcomes. Liver MRI and VAST scores improved post-transplantation. Post-transplantation liver biopsy scores did not change significantly. Pretransplantation liver biopsy demonstrating fibrosis alone should not exclude consideration of isolated heart transplantation. The persistence of hepatic vascular remodeling and fibrosis post-isolated heart transplantation suggests that continued surveillance for hepatic complications post-transplantation for patients with Fontan is reasonable.


Subject(s)
Fontan Procedure/adverse effects , Heart Transplantation , Liver Diseases/diagnosis , Patient Selection , Adolescent , Ascites/diagnostic imaging , Biopsy , Central Venous Pressure , Child , Humans , Liver/diagnostic imaging , Liver Cirrhosis/pathology , Liver Diseases/etiology , Liver Function Tests , Magnetic Resonance Imaging , Postoperative Complications , Retrospective Studies , Splenomegaly/diagnostic imaging , Thrombocytopenia , Varicose Veins/diagnostic imaging , Vascular Remodeling , Young Adult
2.
J Pediatr ; 147(6): 739-43, 2005 Dec.
Article in English | MEDLINE | ID: mdl-16356422

ABSTRACT

OBJECTIVE: To examine the relationship of black race to graft survival after heart transplantation in children. STUDY DESIGN: United Network for Organ Sharing records of heart transplantation for subjects <18 years of age from 1987 to 2004 were reviewed. Analysis was performed using proportional hazards regression controlling for other potential risk factors. RESULTS: Of the 4227 pediatric heart transplant recipients, 717 (17%) were black. The 1-year graft survival rate did not differ among groups; however, the 5-year graft survival rate was significantly lower for black recipients, 51% versus 69%, P < .001. The median graft survival for black recipients was 5.3 years as compared with 11.0 years for other recipients. Black recipients had a greater number of human leukocyte antigen mismatches, lower median household income, and a greater percentage with Medicaid as primary insurance, P < .001, P < .001, and P < .001. After adjusting for economic disparities, black race remained significantly associated with graft failure, odds ratio = 1.67 (95% CI 1.47 to 1.87), P < .001. CONCLUSIONS: Median graft survival after pediatric heart transplantation for black recipients is less than half that of other racial groups. These differences do not appear to be related primarily to economic disparities.


Subject(s)
Black or African American , Graft Rejection/ethnology , Heart Transplantation/ethnology , Adolescent , Black or African American/statistics & numerical data , Child , Child, Preschool , Female , Heart Transplantation/economics , Humans , Income , Infant , Infant, Newborn , Male , Medicaid , Multivariate Analysis , Proportional Hazards Models , Risk Factors , Socioeconomic Factors , Treatment Outcome , United States/epidemiology
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