Plastic Bronchitis in Patients With Fontan Physiology: Review of the Literature and Preliminary Experience With Fontan Conversion and Cardiac Transplantation

Madeleine Larue, Jeffrey G Gossett*, Robert D. Stewart, Carl L. Backer, Constantine Mavroudis, Marshall L. Jacobs

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

20 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)364-372
Number of pages9
JournalWorld Journal for Pediatric and Congenital Heart Surgery
Volume3
Issue number3
DOIs
StatePublished - Jul 2012

Keywords

  • Fontan conversion
  • bronchial casts
  • cardiac transplantation
  • congenital heart disease
  • plastic bronchitis
  • protein-losing enteropathy
  • single ventricle anatomy

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Fingerprint Dive into the research topics of 'Plastic Bronchitis in Patients With Fontan Physiology: Review of the Literature and Preliminary Experience With Fontan Conversion and Cardiac Transplantation'. Together they form a unique fingerprint.

Cite this