Quantification of Hepatic and Splenic Stiffness After Fontan Procedure in Children and Clinical Implications
Bahruz Aliyev1, Zuhal Bayramoglu2, Kemal Nişli1
1Pediatric Radiology Department.
Insights
Shear wave elastography (SWE) reveals elevated liver and spleen stiffness in children with Fontan physiology, indicating Fontan-associated liver disease. This noninvasive method predicts early hepatic changes missed by standard tests.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Medical Imaging
Background:
- Fontan-associated liver disease (FALD) is a progressive complication in patients with Fontan physiology.
- Early detection of FALD is crucial for timely intervention and management.
- Noninvasive methods for assessing liver stiffness are needed.
Purpose of the Study:
- To investigate the utility of shear wave elastography (SWE) in detecting and quantifying liver and spleen stiffness in children with Fontan physiology.
- To correlate SWE findings with clinical and biochemical markers of liver disease.
Main Methods:
- A prospective case-control study involving 41 patients with Fontan physiology and 30 healthy controls.
- Shear wave elastography (SWE) was used to measure hepatic and splenic elasticity in kPa and m/s.
- Statistical analysis was performed to compare groups and assess correlations.
Main Results:
- Fontan patients exhibited significantly higher mean hepatic (15.8 kPa) and splenic (25.6 kPa) SWE values compared to controls (5.59 kPa and 15.9 kPa, respectively) (P < 0.001).
- Positive correlations were found between follow-up duration and NT-proBNP/prothrombin time, and between hepatic SWE and liver enzymes/PT.
- Splenic stiffness correlated with PT and INR. A liver stiffness cutoff of 11.1 kPa showed 95% sensitivity and 100% specificity for FALD.
Conclusions:
- Hepatic and splenic stiffness are significantly increased in children with Fontan physiology.
- SWE is a reliable, noninvasive predictor of early hepatic alterations in FALD, outperforming biochemical tests and routine ultrasounds.
- SWE can aid in the early detection and monitoring of Fontan-associated liver disease.
Abstract:
We aimed to investigate Fontan associated liver disease in children by shear wave elastography (SWE). This is a single-center, prospective case-control study included 41 patients with Fontan physiology and 30 healthy controls. Hepatic and splenic shear wave elasticity values were exhibited both as kPa and m/s. The mean hepatic SWE values of Fontan patients (n = 41; 15.8 ± 3.2 kPa or 2.5 ± 1.8 m/s) were significantly higher than the control group (n = 30; 5.59 ± 0.6 kPa or 1.37 ± 0.07 m/s) (P < 0.001). The mean splenic SWE values of Fontan patients were (25.6 ± 4.61 kPa or 2.85 ± 0.22 m/s) significantly higher than the control group (15.9 ± 1.44 kPa or 2.29 ± 0.1 m/s) (P < 0.001). There were statistically significant positive correlations among the follow-up duration after the Fontan procedure with NT-proBNP (P = 0.008, r = 1) and prothrombin time (P = 0.009, r = 0.4) as well as the hepatic SWE values with alanine aminotransferase (P = 0.039, r = 0.32), gamma-glutamyl transferase (P = 0.045, r = 0.31), and PT (P = 0.011, r = 0.39). There has been statistically significant moderate positive correlations of splenic stiffness values with PT (P = 0.047, r = 0.34), and INR (P = 0.038, r = 0.35). The sensitivity and specificity of liver stiffness cutoff value as 11.1 kPa for detection of Fontan associated liver disease were 95% and 100%, respectively. The hepatic and splenic stiffness increase independently in Fontan patients due to parenchymal disease. Hepatic SWE is a reliable and noninvasive predictor of early hepatic alterations that could not be detected only by biochemical results or routine ultrasound examinations.
