BACKGROUND: Patients with complex congenital heart disease associated with tracheobronchomalacia (TBM) remain difficult to manage after cardiac surgery. We studied the influence of TBM on the outcomes of pediatric patients after cardiac surgery for congenital heart disease to determine how to manage these patients better. METHODS: Twenty-two consecutive pediatric patients who had TBM diagnosed by bronchoscopy or dynamic contrast bronchography before or after cardiac surgery for congenital heart disease during a 5.5-year period were compared with an age- and procedure-matched control group operated on during the same period. Patients diagnosed postoperatively were investigated after a second failed extubation. Patients were managed by oxygen administration, endotracheal suctioning, and positive end-expiratory or continuous positive airway pressure through a nasotracheal tube or tracheostomy. RESULTS: There were 4 deaths within 1 year of surgery, all in the study group, with 2 early (neither of which appeared related to TBM) and 2 late. The estimated survival at 5 years was 82% (95% confidence interval, 59% to 93%) for the study group compared with 100% for control patients (p = 0.012). All deaths occurred in patients undergoing palliative procedures (p = 0.0004), and both children who underwent redo operations died (p = 0.02). Postoperatively, 50% of children with TBM required prolonged ventilation and tracheostomy. Compared with control patients the average postoperative ventilation time, pediatric intensive care unit stay, and hospital stay were 6.5, 11.5, and 20 days versus 1, 2, and 6.5 days, respectively (p < 0.001). CONCLUSIONS: Although associated with longer postoperative ventilation time, pediatric intensive care unit stay, hospital stay, and mortality, outcomes after cardiac procedures in children with TBM are acceptable. Palliative and redo procedures in this group of patients are associated with significantly higher risk of death.