Skip to main content
Springer Nature Link
Log in

Demographic Characteristics and Estimated Prevalence of Fontan-Associated Plastic Bronchitis

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Angelos P, MacArthur C (2010) Pediatric plastic bronchitis: a case report and literature review. Int J Pediatr Otorhinolaryngol Extra 5:66–69

    Article  Google Scholar 

  2. Brogan TV, Finn LS, Pyskaty DJ Jr, Redding GJ, Ricker D, Inglis A et al (2002) Plastic bronchitis in children: a case series and review of the medical literature. Pediatr Pulmonol 34:482–487

    Article  PubMed  Google Scholar 

  3. Cajaiba MM, Borralho P, Reyes-Mugica M (2008) The potentially lethal nature of bronchial casts: plastic bronchitis. Int J Surg Pathol 16:230–232

    Article  PubMed  Google Scholar 

  4. Chaudhari M, Stumper O (2004) Plastic bronchitis after Fontan operation: treatment with stent fenestration of the Fontan circuit. Heart 90:801

    Article  PubMed  CAS  Google Scholar 

  5. Costello JM, Steinhorn D, McColley S, Gerber ME, Kumar SP (2002) Treatment of plastic bronchitis in a Fontan patient with tissue plasminogen activator: a case report and review of the literature. Pediatrics 109:e67

    Article  PubMed  Google Scholar 

  6. Davies RR, Chen JM, Mosca RS (2011) The Fontan procedure: evolution in technique; attendant imperfections and transplantation for “failure”. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 14:55–66

    Article  PubMed  Google Scholar 

  7. Deng J, Zheng Y, Li C, Ma Z, Wang H, Rubin BK (2010) Plastic bronchitis in three children associated with 2009 influenza A(H1N1) virus infection. Chest 138:1486–1488

    Article  PubMed  Google Scholar 

  8. Do P, Randhawa I, Chin T, Parsapour K, Nussbaum E (2012) Successful management of plastic bronchitis in a child post Fontan: case report and literature review. Lung March 20. doi:10.1007/s00408-012-9384-x

  9. Fredenburg TB, Johnson TR, Cohen MD (2011) The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics 31:453–463

    Article  PubMed  Google Scholar 

  10. Goldberg DJ, Dodds K, Rychik J (2010) Rare problems associated with the Fontan circulation. Cardiol Young 20(Suppl 3):113–119

    Article  PubMed  Google Scholar 

  11. Healy F, Hanna BD, Zinman R (2012) Pulmonary complications of congenital heart disease. Paediatr Respir Rev 13:10–15

    Article  PubMed  CAS  Google Scholar 

  12. Heath L, Ling S, Racz J, Mane G, Schmidt L, Myers JL et al (2011) Prospective, longitudinal study of plastic bronchitis cast pathology and responsiveness to tissue plasminogen activator. Pediatr Cardiol 32:1182–1189

    Article  PubMed  Google Scholar 

  13. Hirsch JC, Ohye RG, Devaney EJ, Goldberg CS, Bove EL (2007) The lateral tunnel Fontan procedure for hypoplastic left heart syndrome: results of 100 consecutive patients. Pediatr Cardiol 28:426–432

    Article  PubMed  CAS  Google Scholar 

  14. Hirsch JC, Goldberg C, Bove EL, Salehian S, Lee T, Ohye RG et al (2008) Fontan operation in the current era: a 15-year single institution experience. Ann Surg 248:402–410

    PubMed  Google Scholar 

  15. Languepin J, Scheinmann P, Mahut B, Le Bourgeois M, Jaubert F, Brunelle F et al (1999) Bronchial casts in children with cardiopathies: the role of pulmonary lymphatic abnormalities. Pediatr Pulmonol 28:329–336

    Article  PubMed  CAS  Google Scholar 

  16. Seear M, Hui H, Magee F, Bohn D, Cutz E (1997) Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care Med 155:364–370

    PubMed  CAS  Google Scholar 

  17. Shah SS, Drinkwater DC, Christian KG (2006) Plastic bronchitis: Is thoracic duct ligation a real surgical option? Ann Thorac Surg 81:2281–2283

    Article  PubMed  Google Scholar 

  18. Tzifa A, Robards M, Simpson JM (2005) Plastic bronchitis: a serious complication of the Fontan operation. Int J Cardiol 101:513–514

    Article  PubMed  CAS  Google Scholar 

  19. Wakeham MK, Van Bergen AH, Torero LE, Akhter J (2005) Long-term treatment of plastic bronchitis with aerosolized tissue plasminogen activator in a Fontan patient. Pediatr Crit Care Med 6:76–78

    Article  PubMed  Google Scholar 

  20. Werkhaven J, Holinger LD (1987) Bronchial casts in children. Ann Otol Rhinol Laryngol 96:86–92

    PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This work was supported by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) Grant No. R15 HD065594 (KAS). One of the objectives of the R15 funding mechanism is to expose students to research. We therefore acknowledge the work of the four student coauthors (M. K., A. L., E. K., and E. G.). The content is solely the responsibility of the authors and does not necessarily represent the official views of the NICHD or the National Institutes of Health.

Author information

Authors and Affiliations

  1. Department of Pharmacy, University of Michigan Hospitals and Health Centers, Ann Arbor, MI, USA

    Regine L. Caruthers & Mollie Kempa

  2. Department of Clinical, Social and Administrative Sciences, College of Pharmacy, University of Michigan, Ann Arbor, MI, USA

    Angela Loo, Erin Gulbransen, Elizabeth Kelly, Steven R. Erickson & Kathleen A. Stringer

  3. Department of Cardiac Surgery, Pediatric Surgery, School of Medicine, University of Michigan, Ann Arbor, MI, USA

    Jennifer C. Hirsch

  4. Department of Pediatrics, Pediatric Cardiology, School of Medicine, University of Michigan, Ann Arbor, MI, USA

    Kurt R. Schumacher

Authors
  1. Regine L. Caruthers
    View author publications

    You can also search for this author inPubMed Google Scholar

  2. Mollie Kempa
    View author publications

    You can also search for this author inPubMed Google Scholar

  3. Angela Loo
    View author publications

    You can also search for this author inPubMed Google Scholar

  4. Erin Gulbransen
    View author publications

    You can also search for this author inPubMed Google Scholar

  5. Elizabeth Kelly
    View author publications

    You can also search for this author inPubMed Google Scholar

  6. Steven R. Erickson
    View author publications

    You can also search for this author inPubMed Google Scholar

  7. Jennifer C. Hirsch
    View author publications

    You can also search for this author inPubMed Google Scholar

  8. Kurt R. Schumacher
    View author publications

    You can also search for this author inPubMed Google Scholar

  9. Kathleen A. Stringer
    View author publications

    You can also search for this author inPubMed Google Scholar

Corresponding author

Correspondence to Kathleen A. Stringer.

Additional information

Mollie Kempa and Angela Loo equally contributed to the work.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Caruthers, R.L., Kempa, M., Loo, A. et al. Demographic Characteristics and Estimated Prevalence of Fontan-Associated Plastic Bronchitis. Pediatr Cardiol 34, 256–261 (2013). https://doi.org/10.1007/s00246-012-0430-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-012-0430-5

Keywords