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Published on: November 4, 2010

Plastic bronchitis in children with Fontan palliation: analogue to protein losing enteropathy?

B Stiller1, F Riedel, K Paul

  • 1Department of Pediatric Cardiology, Deutsches Herzzentrum Berlin, Germany. stiller@dhzb.de

Pediatric Cardiology
|April 2, 2002
PubMed

Insights

Bronchitis fibroplastica after Fontan operation caused life-threatening pulmonary failure in two children. High-molecular-weight heparin showed therapeutic potential, improving symptoms and normalizing albumin levels.

Area of Science:

  • Pediatric Cardiology
  • Pulmonary Medicine
  • Vascular Biology

Background:

  • Bronchitis fibroplastica is a rare complication following Fontan operation, characterized by obstructive endobronchial casts.
  • This condition can lead to severe pulmonary failure, posing a significant clinical challenge in pediatric patients.

Observation:

  • Two pediatric patients presented with life-threatening pulmonary failure due to large, rubber-like endobronchial casts post-Fontan surgery.
  • Initial management focused on optimizing cardiac function with diuretics in one patient.

Findings:

  • One patient experienced dramatic improvement, including symptom resolution and normalized serum albumin, after subcutaneous high-molecular-weight heparin treatment.
  • A severe relapse occurred upon heparin discontinuation, with subsequent successful re-treatment, suggesting a crucial role for heparin.
  • Optimizing heart function with diuretics provided some symptomatic relief.

Implications:

  • High-molecular-weight heparin may represent a novel therapeutic option for managing bronchitis fibroplastica, particularly in conjunction with optimized cardiac function.
  • Further research is warranted to elucidate the underlying mechanisms and confirm the efficacy of heparin in this poorly understood condition.
  • This study highlights the potential benefits of anticoagulation strategies in managing complex post-Fontan complications.

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