Protein-losing enteropathy in Fontan circulation: Pathophysiology, outcome and treatment options of a complex...

Area of Science:

• Cardiology
• Gastroenterology
• Pediatric Surgery

Background:

• Protein-losing enteropathy (PLE) is a rare but serious complication following the Fontan operation in patients with single ventricle congenital heart disease.
• PLE leads to significant enteric protein loss, resulting in multiorgan dysfunction, increased morbidity, and mortality.
• Despite advances in congenital heart disease care, managing PLE remains challenging due to poorly understood mechanisms and limited evidence for treatment efficacy.

Purpose of the Study:

• To discuss the pathogenesis and diagnosis of PLE in Fontan patients.
• To provide a comprehensive overview of current therapeutic strategies for PLE.
• To propose a stepwise management approach for PLE in this patient population.

Main Methods:

• Literature review and synthesis of existing data on PLE pathogenesis, diagnosis, and treatment.
• Analysis of proposed therapeutic strategies, including their potential advantages and disadvantages.
• Development of a stepwise clinical management guideline.

Main Results:

• The review details the complex pathophysiology of PLE post-Fontan operation.
• Diagnostic criteria and methods for identifying PLE are outlined.
• Various treatment options are evaluated, highlighting their limitations and potential benefits.

Conclusions:

• Effective management of PLE requires a thorough understanding of its pathogenesis and accurate diagnosis.
• A stepwise, evidence-informed approach is crucial for optimizing therapeutic strategies.
• Further research is needed to improve treatment efficacy and patient outcomes for PLE after Fontan surgery.