Document Detail

Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis.
MedLine Citation:
PMID:  22797520     Owner:  NLM     Status:  MEDLINE    
Plastic bronchitis (PB) is a poorly understood disease that can complicate any underlying pulmonary disease. However, it appears to most often occur in patients with surgically palliated congenital heart disease, particularly after the Fontan procedure. Few data exist about the prevalence and etiology of PB in this population. In an effort to establish data about prevalence, we conducted a retrospective study of an existing Fontan surgery database (n = 654) comprised of data, including sex, age at date of surgery, alive/dead status, New York Heart Association classification at last follow-up, right-ventricular end-diastolic pressure and pulmonary artery pressure before Fontan surgery, and the presence of a Fontan fenestration. An initial medical record review of 173 patients in the database who were followed at the University of Michigan identified seven patients with PB resulting in an estimated prevalence of 4 %. Subsequently, 14 % of 211 surveyed patients reported that they presently expectorate mucus or fibrin plugs (casts). Demographic and clinical variables did not differ between patients with or without possible PB. Collectively, these findings suggest that Fontan patients presently with PB may range from 4 to 14 %, indicating potential under-diagnosis of the disease. There were no remarkable physical or hemodynamic indicators that differentiated patients with or without possible PB. These data also highlight the need for more elaborate, prospective studies to improve our understanding of PB pathogenesis so that more definitive diagnostic criteria for this devastating disease can be established and its prevalence more accurately determined.
Regine L Caruthers; Mollie Kempa; Angela Loo; Erin Gulbransen; Elizabeth Kelly; Steven R Erickson; Jennifer C Hirsch; Kurt R Schumacher; Kathleen A Stringer
Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural     Date:  2012-07-15
Journal Detail:
Title:  Pediatric cardiology     Volume:  34     ISSN:  1432-1971     ISO Abbreviation:  Pediatr Cardiol     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-02-18     Completed Date:  2013-08-13     Revised Date:  2014-02-04    
Medline Journal Info:
Nlm Unique ID:  8003849     Medline TA:  Pediatr Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  256-61     Citation Subset:  IM    
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MeSH Terms
Age Distribution
Age Factors
Bronchitis / diagnosis,  epidemiology*,  etiology
Child, Preschool
Follow-Up Studies
Fontan Procedure / adverse effects*
Heart Defects, Congenital / surgery*
Michigan / epidemiology
Population Surveillance / methods*
Postoperative Complications / diagnosis,  epidemiology*,  etiology
Retrospective Studies
Risk Factors
Sex Distribution
Sex Factors
Grant Support

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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