TY - JOUR
T1 - Plastic Bronchitis in Patients With Fontan Physiology
T2 - Review of the Literature and Preliminary Experience With Fontan Conversion and Cardiac Transplantation
AU - Larue, Madeleine
AU - Gossett, Jeffrey G.
AU - Stewart, Robert D.
AU - Backer, Carl L.
AU - Mavroudis, Constantine
AU - Jacobs, Marshall L.
PY - 2012/7
Y1 - 2012/7
N2 - 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.
AB - 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.
KW - Fontan conversion
KW - bronchial casts
KW - cardiac transplantation
KW - congenital heart disease
KW - plastic bronchitis
KW - protein-losing enteropathy
KW - single ventricle anatomy
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U2 - 10.1177/2150135112438107
DO - 10.1177/2150135112438107
M3 - Review article
C2 - 23804871
AN - SCOPUS:84875461828
VL - 3
SP - 364
EP - 372
JO - World journal for pediatric & congenital heart surgery
JF - World journal for pediatric & congenital heart surgery
SN - 2150-1351
IS - 3
ER -