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.

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