Plastic bronchitis in patients with fontan physiology: review of the literature and preliminary experience with fontan conversion and cardiac transplantation.

ABSTRACT

Plastic bronchitis is a rare, life-threatening condition characterized by the formation of mucofibrinous casts within the pulmonary bronchi. In patients with congenital heart disease, it is most frequently observed in single ventricular anatomies after Fontan palliation. The pathophysiology of plastic bronchitis remains unknown, and a consistently effective treatment strategy has yet to be identified. We report two cases of plastic bronchitis in patients with Fontan physiology. The first was treated with Fontan conversion and, despite encouraging short-term results, experienced recurrence of cast formation seven months postoperatively. The second underwent cardiac transplantation and has been free of bronchial casts for over one year. In addition, we explore the similarities between plastic bronchitis and protein-losing enteropathy, considering theories of their pathophysiologic mechanisms and reports of mutually effective treatment strategies. We propose that bronchial cast formation may result from the confluence of genetic makeup, inflammation, and the Fontan physiology and conclude that further investigation into therapies directed at these factors is merited.