Plastic Bronchitis Developing 5 Years After Fontan Procedure in a Girl with Kartagener’s Syndrome

Abstract

Although rare, plastic bronchitis (PB) is an important early complication after Fontan procedure. Kartagener's syndrome is characterized by mucociliary dysfunction of the respiratory tract and has a triad of features, including situs inversus totalis, chronic sinusitis, and bronchiectasia. We experienced PB in a patient with Kartagener's syndrome 5 years after Fontan procedure.

Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall

“…This finding alerts to regularly evaluate cardiac function and watch over the symptoms of PB in patients who received Fontan intervention. Second, only one case of PB in Kartagener’s syndrome has been reported so far, and the therapeutic approach after emergency removing of bronchial casts was not described [6]. It is likely that underlying immotile cilia dysfunction and decreased mucociliary clearance may influence the development of recurrent bronchial casts in patients with associated PCD and CHD who received Fontan circulation.…”