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.
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Stiller, B., Riedel, F., Paul , K. et al. Plastic bronchitis in children with fontan palliation: Analogue to protein losing enteropathy?. Pediatr Cardiol 23, 90–94 (2002). https://doi.org/10.1007/s00246-001-0024-0
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DOI: https://doi.org/10.1007/s00246-001-0024-0