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A pediatric patient with complex heart disease developed protein-losing enteropathy (PLE) after a Fontan procedure. Thoracic duct ligation proved life-saving when medical therapy failed, offering a new treatment option for this rare complication.

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Area of Science:

  • Pediatric Cardiology
  • Gastroenterology
  • Thoracic Surgery

Background:

  • Single ventricle pathway and complex cardiac conditions require advanced surgical interventions.
  • The Fontan procedure is a palliative surgery for single-ventricle physiology.
  • Protein-losing enteropathy (PLE) is a rare but serious complication following Fontan surgery.

Observation:

  • A 9-year-old boy with a history of complex cardiac issues and a non-fenestrated Fontan shunt presented with recurrent respiratory symptoms.
  • Symptoms included persistent cough with thick, whitish sputum and progressive shortness of breath.
  • The patient was diagnosed with protein-losing enteropathy (PLE) after initial medical management failed.

Findings:

  • Medical therapy for PLE was ineffective in this pediatric patient.
  • Surgical intervention via ligation of the thoracic duct was performed.
  • Thoracic duct ligation resulted in a life-saving outcome for the patient.

Implications:

  • Thoracic duct ligation may be a viable, life-saving treatment for refractory protein-losing enteropathy post-Fontan.
  • This case highlights the importance of considering surgical options for complex pediatric cardiac complications.
  • Further research into the mechanisms and optimal management of PLE after Fontan surgery is warranted.