Plastic bronchitis and protein-losing enteropathy in the Fontan patient: evolving understanding and emerging therapies
AS Mackie, GR Veldtman, L Thorup, VE Hjortdal… - Canadian Journal of …, 2022 - Elsevier
Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially
devastating complications of the Fontan circulation. PB occurs in∼ 4% of Fontan patients,
typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing,
fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are
expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually
within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia …
devastating complications of the Fontan circulation. PB occurs in∼ 4% of Fontan patients,
typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing,
fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are
expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually
within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia …
