Surveillance of Fontan Associated Liver Disease in Childhood and Adolescence.

Autor: Hansen JH; Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel; DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Germany. Electronic address: JanHinnerk.Hansen@uksh.de., Khodami JK; Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel., Moritz JD; Department of Radiology and Neuroradiology, University Hospital Schleswig-Holstein, Campus Kiel., Rinne K; Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel., Voges I; Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel; DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Germany., Scheewe J; Department of Cardiovascular Surgery, University Hospital Schleswig-Holstein, Campus Kiel., Kramer HH; Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel; DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Germany., Uebing A; Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel; DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Germany.
Jazyk: angličtina
Zdroj: Seminars in thoracic and cardiovascular surgery [Semin Thorac Cardiovasc Surg] 2022 Summer; Vol. 34 (2), pp. 642-650. Date of Electronic Publication: 2021 May 09.
DOI: 10.1053/j.semtcvs.2021.04.005
Abstrakt: Fontan associated liver disease (FALD) has been recognized as a potentially serious sequela of the Fontan circulation. Prevalence of FALD among different age groups and risk factors for advanced changes were assessed. FALD screening included abdominal ultrasound and laboratory tests. A "liver disease score (LDS)" incorporating items from ultrasound and blood testing was calculated to grade FALD severity (5 items each, maximum score 10 points). 240 patients (male: n = 139, female: n = 101, systemic right ventricle: n = 160) underwent FALD screening 10 (IQR 7-15) years after Fontan surgery. Ultrasound was abnormal in 184 (76.6%) patients (surface nodularity / blunted liver edge: n = 133, 55.4%; heterogeneous parenchyma: n = 93, 38.8%; splenomegaly: n = 68, 28.3%; ascites: n = 23, 9.6%). At least one abnormal laboratory test was detected in 218 (90.8%) patients. Gamma-glutamyl-transpeptidase was elevated in the majority of patients (n = 206, 85.8%). Median LDS was 3 (2-4). Scores ≥5 were observed in 32 (13.3%) patients. Longer follow-up (15 (11-20) vs 9 (6-14) years, P <0.001), higher central venous (13 (11-15) vs 10 (9-12) mmHg, P <0.001) and end-diastolic pressure (8 (5-10) vs 6 (5-7) mmHg, P = 0.001), impaired ventricular function and absence of sinus rhythm were associated with LDS ≥5. Longer follow-up (OR 1.2 (1.1-1.3), P <0.001) and higher central venous pressure (OR 1.6 (1.3-2.1), p < 0.001) were the only independent predictors of advanced FALD. Abdominal ultrasound and laboratory abnormalities suggestive of FALD are common during routine follow-up already in childhood and adolescence irrespective of ventricular morphology. More advanced findings are associated with longer follow-up and higher central venous pressure.
(Copyright © 2021 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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