Fenestration of the Fontan Circuit as Treatment for Plastic Bronchitis

摘要

Plastic bronchitis is a rare, potentially life-threatening condition in which protein casts form within and occlude the bronchus, resulting in pulmonary failure, and has been identified as a complication after the Fontan procedure. We present a case of a 5-year-old girl who had undergone an extracardiac fenestrated Fontan repair as a component of staged palliation for tricuspid atresia. Six weeks following surgery, the patient presented with airway obstruction, coughing a bronchial cast. Medical therapies to optimize heart function and attempt to control cast formation were implemented, with little clinical impact. Following cardiac catheterization to stent open the fenestration, the symptoms of plastic bronchitis resolved. Cast expectoration recurred following spontaneous closure of the stented fenestration and again resolved with recreation of the baffle defect. Fenestration of the Fontan circuit alters hemodynamics, thereby providing an additional therapeutic option for this devastating disorder.