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Fenestration of the Fontan circuit as treatment for plastic bronchitis

J Wilson1, J Russell, W Williams

  • 1Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto School of Medicine, 555 University Avenue, Toronto, Ontario M5G1X8, Canada.

Pediatric Cardiology
|September 1, 2005
PubMed

Insights

Plastic bronchitis, a rare complication after Fontan procedures, involves bronchial cast formation. Restoring fenestration in the Fontan circuit effectively resolved symptoms in a pediatric patient.

Area of Science:

  • Cardiology
  • Pulmonary Medicine
  • Pediatric Surgery

Background:

  • Plastic bronchitis is a rare, life-threatening condition characterized by bronchial cast formation, leading to pulmonary failure.
  • It has been identified as a significant complication following the Fontan procedure, a complex surgery for single-ventricle congenital heart defects.

Observation:

  • A 5-year-old girl, who underwent an extracardiac fenestrated Fontan repair for tricuspid atresia, developed plastic bronchitis six weeks post-surgery.
  • The patient presented with airway obstruction and coughed up bronchial casts, with medical therapies showing minimal impact.

Findings:

  • Cardiac catheterization to stent open the Fontan fenestration led to the resolution of plastic bronchitis symptoms.
  • Recurrence of cast expectoration coincided with spontaneous closure of the stented fenestration, resolving again upon recreation of the baffle defect.

Implications:

  • Fenestration within the Fontan circuit influences hemodynamics, offering a novel therapeutic approach for managing plastic bronchitis.
  • This case highlights the potential of manipulating Fontan circuit hemodynamics to treat this devastating post-surgical complication.

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