MR elastography-based staging of liver fibrosis in Fontan procedure associated liver disease is confounded by effects of venous congestion

Serai, Suraj D.; Tsitsiou, Y.; Wilkins, B.J.; Ghosh, Adarsh; Cahill, Anne Marie; Biko, D.M.; Rychik, Jack; Rand, Elizabeth B.; Goldberg, David J.

doi:10.1016/j.crad.2022.06.016

Cited by 8 publications

(17 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…30 Unfortunately, the elastography based estimation of fibrosis is confounded in the Fontan patient by the presence of passive congestion, which can increase liver stiffness independently of fibrosis. 8 Magnetic resonance elastography-measured liver stiffness has been shown to be independently associated with central venous pressure and inversely related to the cardiac index. [31][32][33] Magnetic resonance elastography-derived stiffness maps typically demonstrate the greatest stiffness in the periphery of the liver, which corresponds well with the distribution of congestive abnormalities seen on anatomic imaging with CT and MRI 25 (Fig.…”

Section: Elastographymentioning

confidence: 99%

“…Attempts to correlate MRE-derived liver stiffness with hepatic fibrosis have shown variable results with some studies demonstrating promising results while other studies do not. 8,14,[34][35][36] The current state of the art 2-dimensional MRE technique is not able to distinguish between various causes of liver stiffness. Three-dimensional MRE is a technique currently under investigation to separate out the effects of fibrosis, congestion, and inflammation.…”

Section: Elastographymentioning

confidence: 99%

“…The elevated stiffness on MRE may reflect a combination of congestion and fibrosis. Attempts to correlate MRE-derived liver stiffness with hepatic fibrosis have shown variable results with some studies demonstrating promising results while other studies do not 8,14,34–36 . The current state of the art 2-dimensional MRE technique is not able to distinguish between various causes of liver stiffness.…”

Section: Imaging Assessment Of Fibrosis In Faldmentioning

confidence: 99%

“…7 An organ particularly vulnerable to damage by the Fontan circulation is the liver, with patients universally developing chronic liver injury and fibrosis. 7,8…”

mentioning

confidence: 99%

“…In addition, dysfunction of other organs is common with patients developing plastic bronchitis, thromboembolic disease, venous insufficiency, and protein-losing enteropathy 7 . An organ particularly vulnerable to damage by the Fontan circulation is the liver, with patients universally developing chronic liver injury and fibrosis 7,8 …”

mentioning

confidence: 99%

See 4 more Smart Citations

Imaging of Fontan-Associated Liver Disease

Brown

Kolbe

Hull

et al. 2023

J Comput Assist Tomogr

View full text Add to dashboard Cite

The Fontan procedure is the definitive treatment for patients with single-ventricle physiology. Surgical advances have led to a growing number of patients surviving into adulthood. Fontan-associated liver disease (FALD) encompasses a spectrum of pathologic liver changes that occur secondary to altered physiology including congestion, fibrosis, and the development of liver masses. Assessment of FALD is difficult and relies on using imaging alongside of clinical, laboratory, and pathology information. Ultrasound, computed tomography, and magnetic resonance imaging are capable of demonstrating physiologic and hepatic parenchymal abnormalities commonly seen in FALD. Several novel imaging techniques including magnetic resonance elastography are under study for use as biomarkers for FALD progression. Imaging has a central role in detection and characterization of liver masses as benign or malignant. Benign FNH-like masses are commonly encountered; however, these can display atypical features and be mistaken for hepatocellular carcinoma (HCC). Fontan patients are at elevated risk for HCC, which is a feared complication and has a poor prognosis in this population. While imaging screening for HCC is widely advocated, no consensus has been reached regarding an optimal surveillance regimen.

show abstract

Section: Elastographymentioning

confidence: 99%

Section: Elastographymentioning

confidence: 99%

Section: Imaging Assessment Of Fibrosis In Faldmentioning

confidence: 99%

“…7 An organ particularly vulnerable to damage by the Fontan circulation is the liver, with patients universally developing chronic liver injury and fibrosis. 7,8…”

mentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations

Imaging of Fontan-Associated Liver Disease

Brown

Kolbe

Hull

et al. 2023

J Comput Assist Tomogr

View full text Add to dashboard Cite

show abstract

Shear‐wave elastography for monitoring Fontan‐associated liver disease: A prospective cohort study

Bolia,

Alremawi,

Noble

et al. 2024

J. pediatr. gastroenterol. nutr.

View full text Add to dashboard Cite

The spectrum of Fontan‐associated liver disease (FALD) varies from abnormal liver function tests to fibrosis and even cirrhosis. In this prospective study, we evaluated the role of shear‐wave elastography (SWE) in predicting the presence of advanced FALD. Forty‐eight patients (30 males, 13.9 [6–21] years) with a Fontan circulation were evaluated at 8.3 (2.1–18.7) years since the Fontan surgery. The median liver stiffness measurement (LSM) value was higher than values in normal children at 15.4 (9.5–38.7) kPa. The LSMs had a weak but significant correlation with age at the time of LSM (r = 0.25, p = 0.01) and duration post‐Fontan surgery (r = 0.31, p = 0.02). It had a poor correlation with the concomitant aspartate transaminase‐to‐platelet ratio index (r = 0.1, p = 0.39). No difference in the elastography values between children with and without ultrasound evidence of advanced liver disease (17.7 [interquartile range, IQR: 4] vs. 16.1 [IQR: 6], p = 0.62] was observed. Further studies are required to determine the precise role of SWE as a noninvasive marker of liver fibrosis in FALD.

show abstract

Turning to “Omics” in Understanding Fontan-Associated Liver Disease

Kim,

Hoteit

2024

Journal of the American College of Cardiology

View full text Add to dashboard Cite

MR elastography-based staging of liver fibrosis in Fontan procedure associated liver disease is confounded by effects of venous congestion

Cited by 8 publications

References 37 publications

Imaging of Fontan-Associated Liver Disease

Imaging of Fontan-Associated Liver Disease

Shear‐wave elastography for monitoring Fontan‐associated liver disease: A prospective cohort study

Turning to “Omics” in Understanding Fontan-Associated Liver Disease

Contact Info

Product

Resources

About