Al Atrash E, Zourob D, Alghfeli H, AbdelMassih A. Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies. World J Clin Pediatr 2026; 15(4): 119970 [DOI: 10.5409/wjcp.119970]
Corresponding Author of This Article
Eman Al Atrash, MD, Division of Pediatric Gastroenterology, Department of Pediatrics, Mediclinic Airport Hospital, Airport Road, Abu Dhabi 971, United Arab Emirates. emanalatrash8@gmail.com
Research Domain of This Article
Cardiac & Cardiovascular Systems
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review-article
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Al Atrash E, Zourob D, Alghfeli H, AbdelMassih A. Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies. World J Clin Pediatr 2026; 15(4): 119970 [DOI: 10.5409/wjcp.119970]
World J Clin Pediatr. Dec 9, 2026; 15(4): 119970 Published online Dec 9, 2026. doi: 10.5409/wjcp.119970
Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies
Eman Al Atrash, Doaa Zourob, Huda Alghfeli, Antoine AbdelMassih
Eman Al Atrash, Division of Pediatric Gastroenterology, Department of Pediatrics, Mediclinic Airport Hospital, Abu Dhabi 971, United Arab Emirates
Doaa Zourob, Al Mushrif Children’s Specialty Center, AHS, Pure Health Group, Abu Dhabi 971, United Arab Emirates
Huda Alghfeli, Division of Pediatric Gastroenterology, Department of Pediatrics, Burjeel Medical City, Abu Dhabi 971, United Arab Emirates
Antoine AbdelMassih, Division of Pediatric Cardiology, Department of Pediatrics, SKMC, Pure Health Group, Abu Dhabi 971, United Arab Emirates
Author contributions: Al Atrash E and AbdelMassih A wrote the management and conclusion; Zourob D wrote the introduction and pathophysiology; Alghfeli H wrote the clinical presentation and diagnosis; all of the authors read and approved the final version of the manuscript to be published.
AI contribution statement: AI tools were used only for language editing and improving clarity, and all scientific content is entirely the authors’ own.
Conflict-of-interest statement: All authors declare no conflict of interest in publishing the manuscript.
Corresponding author: Eman Al Atrash, MD, Division of Pediatric Gastroenterology, Department of Pediatrics, Mediclinic Airport Hospital, Airport Road, Abu Dhabi 971, United Arab Emirates. emanalatrash8@gmail.com
Received: February 12, 2026 Revised: February 26, 2026 Accepted: May 7, 2026 Published online: December 9, 2026 Processing time: 222 Days and 0.5 Hours
Core Tip
Core Tip: Fontan-associated protein-losing enteropathy is a rare but life-threatening complication of Fontan circulation, driven by abnormal hemodynamics, lymphatic dysfunction, and intestinal barrier injury. This review synthesizes current understanding of protein-losing enteropathy pathophysiology and highlights contemporary management strategies, including nutritional optimization, targeted pharmacologic therapies, and emerging lymphatic interventions. Particular emphasis is placed on individualized, mechanism-based treatment and the evolving role of advanced imaging and interventional techniques. Improved recognition and multidisciplinary care have enhanced outcomes; however, refractory disease remains challenging, underscoring the need for novel therapeutic approaches.
