Protein losing enteropathy after the Fontan operation

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• The Fontan procedure is a palliative surgery for single ventricle congenital heart disease, enabling survival into adulthood.
• Aging patients with Fontan circulation face diverse complications, with protein-losing enteropathy (PLE) being a significant cause of morbidity and mortality.
• PLE after Fontan surgery presents weeks to years post-operation, often linked to adverse hemodynamics.

Purpose of the Study:

• To review the current understanding of protein-losing enteropathy (PLE) in patients with Fontan circulation.
• To highlight the importance of a standardized approach to the diagnosis and management of PLE.
• To discuss potential therapeutic strategies and the need for further research.

Main Methods:

• Comprehensive literature review of protein-losing enteropathy in Fontan patients.
• Analysis of contributing factors including lymphatic insufficiency, elevated central venous pressure, and mesenteric circulation abnormalities.
• Discussion of diagnostic modalities such as multimodality imaging and cardiac catheterization.

Main Results:

• Protein-losing enteropathy is multifactorial, involving lymphatic issues, high venous pressure, and intestinal inflammation.
• Diagnosis requires thorough evaluation to identify and treat reversible causes.
• Advanced cases may necessitate heart transplantation or lymphatic decompression.

Conclusions:

• Despite advancements, mortality from PLE in Fontan patients remains high.
• Standardizing PLE definition and management is essential for research and clinical practice.
• A standardized diagnostic and treatment strategy is proposed to improve patient outcomes.