Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Mar;40(3):638-645.
doi: 10.1111/liv.14375. Epub 2020 Jan 22.

Fontan protein-losing enteropathy is associated with advanced liver disease and a proinflammatory intestinal and systemic state

Enrique Rodríguez de Santiago  1   2 , Luis Téllez  1   2   3 , Elvira Garrido-Lestache Rodríguez-Monte  2   4 , Elena Garrido-Gómez  1   2 , Lara Aguilera-Castro  1 , María Álvarez-Fuente  2   4 , María Jesús Del Cerro  2   4 , Agustín Albillos  1   2   3
Affiliations

Fontan protein-losing enteropathy is associated with advanced liver disease and a proinflammatory intestinal and systemic state

Enrique Rodríguez de Santiago et al. Liver Int. 2020 Mar.

Abstract

Background and aims: Protein-losing enteropathy (PLE) after Fontan surgery carries significant morbimortality. Its pathophysiology and association with other Fontan complications are poorly understood. Our aims were to examine whether Fontan-PLE is associated with greater liver damage and to assess the presence of systemic and intestinal inflammation.

Methods: Fontan patients with PLE and Fontan controls without PLE matched for age and Fontan surgery procedure were included. Data were prospectively compiled on blood and stool tests, liver imaging, elastography, cardiac-MRI and cardiac catheterization.

Results: Twenty-nine Fontan patients were enrolled (14 with PLE and 15 controls without PLE). Patients with PLE had more advanced liver disease estimated by non-invasive methods: blunt liver margins on ultrasonography (71.4% vs 26.7%, P = .027), greater median liver stiffness (25.4 vs 14.5 kPa, P = .003) and higher FIB-4 (P = .016). Portal hypertension-related signs were more common in patients with PLE including ascites (P = .035), larger spleen size (P = .005), oesophageal varices/splanchnic collateral shunts (P = .03), higher liver stiffness-spleen size-to-platelet ratio risk score (P < .001) and lower platelet count (P = .01). Systemic proinflammatory cytokines (TNF-α, interleukin-6), biomarkers of intestinal permeability (intestinal fatty-acid binding protein) and faecal calprotectin concentrations were also significantly increased in Fontan-PLE (P < .05). Faecal calprotectin directly correlated with alpha-1 antitrypsin clearance and inversely with cardiac index, total serum proteins and body mass index.

Conclusion: Fontan-PLE is associated with advanced liver disease and increased markers of systemic inflammation and intestinal permeability. Faecal calprotectin is elevated and correlates with Fontan-PLE severity. Liver assessment is mandatory in all Fontan patients, and especially in those with PLE.

Keywords: Fontan; calprotectin; inflammation; liver disease; protein-losing enteropathy.

PubMed Disclaimer

References

REFERENCES

    1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240-248.
    1. Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102:1081-1086.
    1. Ostrow AM, Freeze H, Rychik J. Protein-losing enteropathy after fontan operation: investigations into possible pathophysiologic mechanisms. Ann Thorac Surg. 2006;82:695-700.
    1. Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998;115:1063-1073.
    1. Gursu HA, Erdogan I, Varan B, et al. Oral budesonide as a therapy for protein-losing enteropathy in children after the Fontan operation. J Card Surg. 2014;29:712-716.

Publication types

MeSH terms

LinkOut - more resources