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Management of protein-losing enteropathy after the Fontan procedure
1Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA.
Insights
Protein-losing enteropathy (PLE) after the Fontan procedure causes edema and immune issues. The exact cause is unknown but linked to abnormal hemodynamics, requiring further research for better treatments.
Area of Science:
- Pediatric Cardiology
- Gastroenterology
- Critical Care Medicine
Background:
- Protein-losing enteropathy (PLE) is increasingly diagnosed post-Fontan procedure.
- Key complications include edema, immunodeficiency, and hypercoagulability.
- The underlying pathophysiology remains unclear.
Purpose of the Study:
- To explore the potential pathophysiological mechanisms of PLE after Fontan procedure.
- To review current and potential treatment strategies for this condition.
Main Methods:
- Review of existing literature and clinical observations.
- Discussion of hemodynamic factors potentially contributing to PLE.
- Categorization of current treatment approaches.
Main Results:
- PLE is associated with hemodynamic derangements not readily identified by standard measures.
- Treatment strategies are categorized into symptomatic relief, hemodynamic improvement, intestinal stabilization, and primary derangement alteration.
- No single treatment has proven universally effective.
Conclusions:
- Understanding the precise cause of PLE post-Fontan procedure is crucial for developing effective therapies.
- Further research into the hemodynamic mechanisms is warranted.
- Current treatments offer symptomatic relief but do not address the root cause.
Abstract:
Enteric loss of protein with the sequelae of edema, immunodeficiency, and hypercoagulability is being diagnosed with increasing frequency after Fontan procedure. The precise pathophysiological mechanism is unknown; however, protein-losing enteropathy (PLE) after Fontan procedure is likely related to a hemodynamic derangement that is not easily detectable via standard hemodynamic measures presently obtainable in the cardiac catheterization laboratory. Treatment options include (1) symptomatic relief via diuretics, supplemental albumin infusion, and dietary change to high protein/high medium-chain triglyceride intake, (2) hemodynamic improvement via afterload reduction (angiotensin-converting enzyme inhibitors), repair of branch pulmonary artery stenoses, or coil embolization of aortopulmonary collaterals, (3) intestinal cell membrane stabilization via high-dose steroids or heparin infusion, and (4) attempt at alteration of the primary hemodynamic derangement via fenestration of the systemic venous baffle or via heart transplantation. Further understanding of the cause of PLE after Fontan procedure is needed before more effective treatment options can be used. Copyright 1998 by W.B. Saunders Company