Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies
Abstract
Protein-losing enteropathy (PLE) is a condition characterized by the abnormal loss of serum proteins into the intestinal lumen, and it is one of the most challenging complications in individuals with Fontan circulation. It affects approximately 5%-12% of Fontan patients and is associated with increased morbidity and mortality. As these patients age and milder or transient forms of PLE are more frequently recognized, its incidence is expected to rise. Although early detection and recent advances in treatment have improved quality of life, the risk of death remains significant. This review explores the pathogenesis of PLE in Fontan patients and discusses current management strategies. Treatment options are diverse and include medical therapies such as pulmonary vasodilators to reduce pulmonary vascular resistance, alpha-adrenergic agents to enhance lymphatic tone and promote lymphatic peristalsis and interventional procedures such as widening the fenestration to decongest the inferior vena cava. Additionally, lymphatic embolization guided by liver lymphangiography has emerged as a promising therapeutic approach in refractory cases. This strategy reflects a shift from palliation toward targeted, pathophysiology-driven interventions that aim to improve outcomes and potentially delay the need for transplantation.
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BibTeX
@article{atrash2026fontanrelated,
title = {{Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies}},
author = {Eman Al Atrash and Doaa Zourob and Huda Alghfeli and Antoine AbdelMassih},
journal = {World Journal of Clinical Pediatrics},
year = {2026},
month = nov,
doi = {10.5409/wjcp.119970},
url = {https://doi.org/10.5409/wjcp.119970},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies
AU - Eman Al Atrash
AU - Doaa Zourob
AU - Huda Alghfeli
AU - Antoine AbdelMassih
PY - 2026
DA - 2026/11/03
JO - World Journal of Clinical Pediatrics
DO - 10.5409/wjcp.119970
UR - https://doi.org/10.5409/wjcp.119970
AB - Protein-losing enteropathy (PLE) is a condition characterized by the abnormal loss of serum proteins into the intestinal lumen, and it is one of the most challenging complications in individuals with Fontan circulation. It affects approximately 5%-12% of Fontan patients and is associated with increased morbidity and mortality. As these patients age and milder or transient forms of PLE are more frequently recognized, its incidence is expected to rise. Although early detection and recent advances in treatment have improved quality of life, the risk of death remains significant. This review explores the pathogenesis of PLE in Fontan patients and discusses current management strategies. Treatment options are diverse and include medical therapies such as pulmonary vasodilators to reduce pulmonary vascular resistance, alpha-adrenergic agents to enhance lymphatic tone and promote lymphatic peristalsis and interventional procedures such as widening the fenestration to decongest the inferior vena cava. Additionally, lymphatic embolization guided by liver lymphangiography has emerged as a promising therapeutic approach in refractory cases. This strategy reflects a shift from palliation toward targeted, pathophysiology-driven interventions that aim to improve outcomes and potentially delay the need for transplantation.
SN - 2219-2808
ER -