Objective: To report the successful use of Tissue Plasminogen Activator (TPA) in a patient with respiratory failure from plastic bronchitis.

Methods: Case report.

Results: Approximately 18 months after modified Fontan surgery for complex heart disease that included obstructed anomalous right pulmonary venous drainage, an eight year old boy developed plastic bronchitis. He was treated with bronchodilators, physiotherapy and nightly nebulised DNAse. He remained well on this treatment over the next year but continued to produce bronchial casts on most days. During this period he required two brief admissions for worsening respiratory distress. These were managed successfully with intensification of bronchodilator treatment and physiotherapy. Sixteen months after the initial diagnosis he was admitted with rhinoviral infection, increased cast production and severe respiratory failure. He developed profound hypoxemia and continued to worsen despite all attempts at more conventional treatment including ventilation, nebulised DNAse, antibiotics, physiotherapy and repeated bronchoscopic removal of casts. After commencing nebulised TPA (5mg in 5ml four hourly) cast production ceased, air entry improved dramatically and systemic saturations rose from levels that were persistently below 50% back to previous baseline levels between 85 and 90%. TPA treatment was successfully weaned as his infection settled. No adverse effects were noted. He was discharged 18 days later.

Conclusion: Nebulised TPA may be lifesaving in respiratory failure from plastic bronchitis.