Effect of Fontan operation on liver stiffness in children with single ventricle physiology
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Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation.
Eighteen children undergoing the Fontan operation were prospectively enrolled. Eight US P-SWE measurements were obtained from the right hepatic lobe before surgery, and at multiple postoperative time points. Temporally related inferior vena cava pressure (IVC) data was collected from medical records, when available. Changes in mean liver shear wave speed (SWS) were assessed using a mixed-effect model with post hoc Tukey correction. Changes in IVC pressure were evaluated using the Wilcoxon signed-rank test. A p value less than 0.05 was considered significant.
Mean age at enrolment was 33.5 ± 10.5 months. Baseline mean liver SWS was normal at 1.18 ± 0.29 m/s, increased to 2.28 ± 0.31 m/s at 2.5 ± 1.2 days (p < 0.0001) and to 2.22 ± 0.38 m/s at 7.5 ± 1.4 days (p < 0.0001). Five subjects returned at a mean of 185 ± 28 days, and mean liver SWS remained elevated at 2.08 ± 0.24 m/s (p < 0.0001). Mean IVC pressure increased from 7.2 ± 2.6 mmHg at baseline to 16.44 ± 3.3 mmHg at 2.2 ± 0.8 days post-surgery (p = 0.004).
The Fontan operation immediately and chronically increases liver stiffness and IVC pressure. Our study provides further evidence that congestion is a key driver of Fontan-associated liver disease.
• The Fontan operation triggers immediate hepatic congestion and marked liver stiffening.
• Congestion, not fibrosis, drives early increased liver stiffness in Fontan patients.
• Hepatic congestion persists chronically for months after the Fontan operation.
• Congestion confounds shear wave elastography as a post-Fontan liver fibrosis biomarker.
KeywordsHepatopathy Congestion Congenital heart disease Ultrasound Shear wave elastography
Fontan-associated liver disease
Inferior vena cava
Point shear wave elastography
Shear wave elastography
Shear wave speed
The scientific guarantor of this publication is Frank W. DiPaola, MD. The authors of this manuscript declare relationships with the following companies: Dr. Dillman discloses that he receives unrelated, unrestricted, investigator-initiated research funding from Siemens Medical Solutions USA, Inc. The authors have no other conflicts of interest to disclose. This research was funded by a pilot grant provided by the Michigan Institute for Clinical and Health Research (MICHR), University of Michigan, Ann Arbor, MI (CTSA 2UL1TR000433).
Aishwarya Parameswaran, MS, of the Michigan Institute for Clinical and Health Research (MICHR) and one of the authors, has significant statistical expertise and kindly provided statistical advice for this manuscript. Institutional review board approval was obtained. Written informed consent was obtained from all subjects (patients) in this study. Methodology: prospective, cohort study, performed at one institution.
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