Effect of Fontan operation on liver stiffness in children with single ventricle physiology

• Published in: European radiology Vol. 27; no. 6; pp. 2434 - 2442
• Main Authors: DiPaola, Frank W., Schumacher, Kurt R., Goldberg, Caren S., Friedland-Little, Joshua, Parameswaran, Aishwarya, Dillman, Jonathan R.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.06.2017; Springer Nature B.V
• Subjects: Biopsy; Cardiology; Cardiovascular disease; Central Venous Pressure - physiology; Child; Child, Preschool; Congenital diseases; Cross-Sectional Studies; Diagnostic Radiology; Elasticity Imaging Techniques; Female; Fontan Procedure - adverse effects; Heart Ventricles - abnormalities; Heart Ventricles - surgery; Hospitals; Humans; Imaging; Infant; Internal Medicine; Interventional Radiology; Liver Cirrhosis - diagnostic imaging; Liver Cirrhosis - physiopathology; Liver diseases; Liver Diseases - etiology; Liver Diseases - physiopathology; Male; Medicine; Medicine & Public Health; Neuroradiology; Pediatrics; Physiology; Postoperative Complications - etiology; Postoperative Complications - physiopathology; Prospective Studies; Radiology; Ultrasonic imaging; Ultrasound; Vena Cava, Inferior - diagnostic imaging; Vena Cava, Inferior - physiology; Ann Arbor Michigan; United States > US; Hepatopathy; Shear wave elastography; Congenital heart disease; Congestion; Ultrasound
• ISSN: 0938-7994; 1432-1084; 1432-1084
• DOI: 10.1007/s00330-016-4614-x

Objectives Assess liver stiffness using ultrasound point shear wave elastography (US P-SWE) in children before and after the Fontan operation. Methods 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. Results 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). Conclusion 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. Key points • 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.