Plastic Bronchitis After Extracardiac Fontan Operation

Background We discuss 4 cases of plastic bronchitis (PB) after Fontan procedure observed at our Institution, with the aim to identify an effective treatment and possible factors favoring the onset of PB. Plastic bronchitis is an uncommon disease characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and, in few reports, aerosolized fibrinolytic and pulmonary vasodilators. Methods Four cases of PB after Fontan procedure occurred in our center from January 2008 to January 2012. Results All patients showed preserved ventricle function and underwent embolization of systemic-pulmonary collaterals prior to a Fontan procedure and all had chylothorax in the perioperative period. Two patients died; 1 of massive thrombosis of the superior vena cava and right pulmonary artery and the other of acute asphyxiation and refractory cardiac failure. Conclusions The exact causes of PB in Fontan patients remain unknown. An early diagnosis and a multiple therapy with steroids, mucolytics, pulmonary vasodilators, and aerosolized tissue plasminogen activator may be effective.