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A new surgical strategy for medically uncontrolled protein-losing enteropathy after Fontan surgery

Seul Gi Cha1, Gi Beom Kim1, Chung Il Noh1

  • 1Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea.

Interactive Cardiovascular and Thoracic Surgery
|April 20, 2018
PubMed

Insights

Managing protein-losing enteropathy after Fontan surgery is difficult. Surgical intervention involving Fontan takedown and pulsatile bidirectional cavopulmonary shunting successfully treated a pediatric patient unresponsive to medical management.

Area of Science:

  • Cardiology
  • Pediatric Gastroenterology
  • Surgical Innovation

Background:

  • Protein-losing enteropathy (PLE) is a challenging complication following Fontan surgery.
  • Managing PLE in patients with Fontan circulation requires innovative therapeutic strategies.

Observation:

  • A 14-year-old boy with Fontan circulation developed protein-losing enteropathy.
  • The patient underwent two years of medical treatment without significant clinical improvement.

Findings:

  • Surgical intervention, including Fontan takedown and conversion to pulsatile bidirectional cavopulmonary shunting, resolved the protein-losing enteropathy.
  • This case highlights the potential efficacy of surgical revision in refractory PLE post-Fontan.

Implications:

  • Fontan takedown and conversion to pulsatile flow may be a viable treatment option for intractable protein-losing enteropathy.
  • This approach could improve outcomes for Fontan patients experiencing severe gastrointestinal complications.

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