RESUMO
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.
Assuntos
Técnica de Fontan/efeitos adversos , Transplante de Coração , Hepatopatias/diagnóstico , Seleção de Pacientes , Adolescente , Ascite/diagnóstico por imagem , Biópsia , Pressão Venosa Central , Criança , Humanos , Fígado/diagnóstico por imagem , Cirrose Hepática/patologia , Hepatopatias/etiologia , Testes de Função Hepática , Imageamento por Ressonância Magnética , Complicações Pós-Operatórias , Estudos Retrospectivos , Esplenomegalia/diagnóstico por imagem , Trombocitopenia , Varizes/diagnóstico por imagem , Remodelação Vascular , Adulto JovemRESUMO
OBJECTIVE: To assess if a heterogeneous pattern on research liver ultrasound examination can identify children at risk for advanced cystic fibrosis (CF) liver disease. STUDY DESIGN: Planned 4-year interim analysis of a 9-year multicenter, case-controlled cohort study (Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF). Children with pancreatic insufficient CF aged 3-12 years without known cirrhosis, Burkholderia species infection, or short bowel syndrome underwent a screening research ultrasound examination. Participants with a heterogeneous liver ultrasound pattern were matched (by age, Pseudomonas infection status, and center) 1:2 with participants with a normal pattern. Clinical status and laboratory data were obtained annually and research ultrasound examinations biannually. The primary end point was the development of a nodular research ultrasound pattern, a surrogate for advanced CF liver disease. RESULTS: There were 722 participants who underwent screening research ultrasound examination, of which 65 were heterogeneous liver ultrasound pattern and 592 normal liver ultrasound pattern. The final cohort included 55 participants with a heterogeneous liver ultrasound pattern and 116 participants with a normal liver ultrasound pattern. All participants with at least 1 follow-up research ultrasound were included. There were no differences in age or sex between groups at entry. Alanine aminotransferase (42 ± 22 U/L vs 32 ± 19 U/L; P = .0033), gamma glutamyl transpeptidase (36 ± 34 U/L vs 15 ± 8 U/L; P < .001), and aspartate aminotransferase to platelet ratio index (0.7 ± 0.5 vs 0.4 ± 0.2; P < .0001) were higher in participants with a heterogeneous liver ultrasound pattern compared with participants with a normal liver ultrasound pattern. Participants with a heterogeneous liver ultrasound pattern had a 9.1-fold increased incidence (95% CI, 2.7-30.8; P = .0004) of nodular pattern vs a normal liver ultrasound pattern (23% in heterogeneous liver ultrasound pattern vs 2.6% in normal liver ultrasound pattern). CONCLUSIONS: Research liver ultrasound examinations can identify children with CF at increased risk for developing advanced CF liver disease.
Assuntos
Fibrose Cística/complicações , Hepatopatias/etiologia , Fígado/patologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Fígado/diagnóstico por imagem , Hepatopatias/diagnóstico , Masculino , Estudos Prospectivos , Medição de Risco , UltrassonografiaRESUMO
OBJECTIVE: To determine whether abdominal magnetic resonance imaging (MRI) detects hepatic abnormalities before clinical or biochemical perturbations in patients after the Fontan procedure. STUDY DESIGN: Thirty-nine children and adolescents who underwent the Fontan procedure and were referred to a pediatric hepatologist by cardiology services between 2011 and 2012 were reviewed retrospectively. Physical examination findings, routine laboratory tests of liver function, evaluation for chronic liver disease, and abdominal MRI findings were recorded. MRI findings were evaluated relative to time elapsed since surgery by 2 radiologists (blinded). RESULTS: Assessment for coexisting chronic liver disease was negative in all patients. All patients had a normal serum albumin level and International Normalized Ratio. Twenty-six of the 39 patients (67%) underwent abdominal MRI, 4 had MRI-incompatible hardware, and 9 did not undergo MRI because of insurance denial. All MRI scans demonstrated morphologic liver changes with varying degrees of reticular contrast enhancement compatible with fibrosis and congestion. Reticular contrast enhancement was often nonuniform, and 9 patients (35%) had multifocal arterially enhancing lesions. CONCLUSION: MRI can identify hepatic abnormalities in patients after Fontan surgery that go undetected by standard clinical and laboratory assessments. These abnormalities are not uniformly distributed throughout the liver, and thus assessment by liver biopsy analysis is subject to sampling error.