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Role of caval venous manipulation in treatment of protein-losing enteropathy
Shaji Menon1, Donald Hagler, Frank Cetta
1Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA.
Insights
Protein-losing enteropathy linked to heart conditions can be treated by restoring unobstructed blood flow. Addressing superior caval venous pressure is key, though outcomes vary with the Fontan operation.
Area of Science:
- Cardiology
- Gastroenterology
- Pediatric Surgery
Background:
- Protein-losing enteropathy (PLE) is a known complication in patients with complex congenital heart disease.
- Conditions associated with PLE include Fontan operation, restrictive cardiomyopathy, constrictive pericarditis, and venous obstruction.
Observation:
- This study reports on three patients experiencing PLE.
- Two patients presented with superior caval venous occlusion.
- One patient had an extracardiac Fontan operation with a stenosed extracardiac conduit.
Findings:
- Restoring unobstructed blood flow in the superior or inferior caval vein resolved PLE in all three patients.
- Reducing pressure in superior caval vein tributaries can be curative.
- PLE associated with the Fontan operation presents greater treatment challenges.
Implications:
- Early diagnosis and intervention for venous obstruction are crucial for managing PLE in cardiac patients.
- Further research into the multifactorial mechanisms of PLE is warranted.
- Optimizing surgical techniques for the Fontan procedure may reduce PLE incidence.
Abstract:
Protein-losing enteropathy has been associated with the Fontan operation, restrictive cardiomyopathy, constrictive pericarditis, tricuspid valvar stenosis and insufficiency, and superior caval venous obstruction. The mechanism of development of this complication of cardiac disease likely is multifactorial. We report here our experience with 3 patients with protein-losing enteropathy. Two had superior caval venous occlusion. The third patient had undergone an extracardiac Fontan operation, with stenosis of the extracardiac conduit placed to the right pulmonary artery. In all three cases, the complication was resolved by restoring unobstructed flow of blood in the superior or inferior caval vein. Since the aetiology of protein-losing enteropathy associated with cardiac disease is multifactorial, reduction of pressure in the tributaries of the superior caval vein may, in some cases, be curative. It is more difficult to cure the problem when it is associated with the Fontan operation.
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