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Plastic bronchitis in children with Fontan palliation: analogue to protein losing enteropathy?
1Department of Pediatric Cardiology, Deutsches Herzzentrum Berlin, Germany. stiller@dhzb.de
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.
Abstract:
We studied a 6-year-old boy and a 2-year-old girl with bronchitis fibroplastica following Fontan operation. Large endobronchial casts of rubber-like consistency resulted in life-threatening pulmonary failure. In one patient symptoms improved after optimizing heart function with diuretics, and in the other a dramatic improvement with the resolution of the clinical symptoms and normalized serum albumin followed subcutaneous high-molecular-weight heparin treatment. The severe relapse after discontinuation of the heparin medication and the once more successful treatment with heparin suggest that in addition to optimizing heart function, high-molecular-weight heparin might be a therapeutic option for this poorly understood condition.