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Isolation of Endocardial and Coronary Endothelial Cells from the Ventricular Free Wall of the Rat Heart
08:22

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An Uncommon Finding in an Adult: A Case Report of Hypoplastic Left Heart Syndrome and Protein-Losing Enteropathy

Jayasree Ravilla1, Siva Naga Yarrrarapu1, Sai Rakshith Gaddameedi1

  • 1Internal Medicine, Monmouth Medical Center, Long Branch, USA.

Cureus
|June 21, 2024
PubMed

Insights

Protein-losing enteropathy (PLE) is a rare disorder often seen in infants with hypoplastic left heart syndrome (HLHS). This case highlights managing PLE after the Fontan procedure, offering insights for challenging hypoproteinemia cases.

Area of Science:

  • Pediatric Cardiology
  • Gastroenterology
  • Rare Diseases

Background:

  • Protein-losing enteropathy (PLE) is a rare condition with varied etiologies and limited treatment options.
  • Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect with high mortality without intervention.
  • The Fontan procedure is a multi-stage surgery to treat HLHS, but can be associated with complications like PLE.

Observation:

  • This report details a case of a patient with HLHS who developed PLE post-Fontan procedure.
  • The clinical course and management of this complex case are described.
  • The patient presented with refractory hypoproteinemia.

Findings:

  • The case illustrates a specific instance of PLE occurring in the context of HLHS treated with the Fontan procedure.
  • Successful management strategies for hypoproteinemia in this setting were explored.
  • The outcome of the described clinical course was documented.

Implications:

  • Increased physician awareness regarding PLE in HLHS patients undergoing the Fontan procedure is crucial.
  • This case provides a basis for discussing appropriate management strategies for refractory hypoproteinemia in similar complex pediatric cases.
  • Further research into the pathophysiology and treatment of PLE in post-Fontan patients is warranted.

Keywords:
congenitalenteropathyfontanhypoplasticprotein

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