Outcome of patients with cystic fibrosis admitted to the intensive care unit: is invasive mechanical ventilation a risk factor for death in patients waiting lung transplantation?

Heart Lung. 2010 Mar-Apr;39(2):153-9. doi: 10.1016/j.hrtlng.2009.06.014. Epub 2009 Aug 25.


Objective: The admission of patients with cystic fibrosis (CF) to the intensive care unit (ICU) is controversial. Our aim was to study the long-term outcome of patients with CF who were admitted to the ICU and the effect of ventilation modality.

Methods: The medical records of 104 admissions (1996-2006) of 48 patients with CF (age 18+/-9 years) were reviewed. Seventeen patients were admitted with reversible conditions (group 1). Thirty-one patients were admitted for acute on chronic respiratory failure (group 2).

Results: In group 1, 16 of 17 patients survived up to 10 years from ICU admission. Conversely, in group 2, 23 of 31 patients (74%) died of respiratory failure. In group 2, 17 of 18 patients who were mechanically ventilated died within 90 days from admission, and 7 of 10 patients treated for prolonged periods with bi-level positive airway pressure are still alive up to 10 years after admission and transplantation.

Conclusion: Patients requiring mechanical ventilation may have a poor prognosis. The outcome of treatment with bi-level positive airway pressure is good, even in patients who had many episodes of acute respiratory failure.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cystic Fibrosis / mortality*
  • Cystic Fibrosis / physiopathology
  • Female
  • Humans
  • Infant
  • Intensive Care Units
  • Lung Transplantation
  • Male
  • Prognosis
  • Respiration, Artificial / adverse effects*
  • Respiratory Function Tests
  • Respiratory Insufficiency / mortality*
  • Respiratory Insufficiency / physiopathology
  • Risk Factors
  • Survival Rate
  • Treatment Outcome