, Volume 185, Issue 2, pp 73–79

Improved Survival Following Lung Transplantation with Long-Term Use Of Bilevel Positive Pressure Ventilation in Cystic Fibrosis

  • Ori Efrati
  • Mordechai R. Kremer
  • Asher Barak
  • Arie Augarten
  • Nira Reichart
  • Amir Vardi
  • Dalit Modan-Moses


Bilevel positive airway pressure ventilation (BIPAP) has been used in cystic fibrosis (CF) patients as a bridge to transplantation. Our aim was to evaluate the effect of BIPAP use before transplantation on post-transplantation morbidity and mortality. We performed a retrospective study at a tertiary care center. Twelve CF patients (9 males; mean age = 26 years) were assessed. Group 1 consisted of eight patients that did not use BIPAP before lung transplantation. Group 2 comprised four patients who used BIPAP for 3–15 months while awaiting transplantation. Patients were evaluated before and two to ten years after transplantation. All eight patients who did not use BIPAP died two months to ten years after transplantation. All four BIPAP users are alive with no evidence of bronchiolitis obliterans two to eight years after lung transplantation. We demonstrated a significant improvement in acid-base balance (p < 0.01) and body mass index (p < 0.05) and a tendency toward improvement in the work of breathing and number of hospitalizations. We conclude that improvement in nutritional status and respiratory muscle strength before lung transplantation in BIPAP users may prevent post lung transplantation infection and acute rejection rate, which in turn may reduce chronic rejection (bronchiolitis obliterans) and improve long-term survival after lung transplantation.


  1. 1.
    Kerem E, Reisman J, Corey M, Canny GJ, Levison H (1992) Prediction of mortality in patients with cystic fibrosis. N Engl J Med 326:1187–1191PubMedCrossRefGoogle Scholar
  2. 2.
    Mayer-Hamblett N, Rosenfeld M, Emerson J, Goss CH, Aitken ML (2002) Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. Am J Respir Crit Care Med 166:1550–1555PubMedCrossRefGoogle Scholar
  3. 3.
    Davis PB, di Sant’Agnese PA (1978) Assisted ventilation for patients with cystic fibrosis. JAMA 239:1851–1854PubMedCrossRefGoogle Scholar
  4. 4.
    Waldhorn RE (1992) Nocturnal nasal intermittent positive pressure ventilation with bi-level positive airway pressure (BiPAP) in respiratory failure. Chest 101:516–521PubMedGoogle Scholar
  5. 5.
    Sood N, Paradowski LJ, Yankaskas JR (2001) Outcomes of intensive care unit care in adults with cystic fibrosis. Am J Respir Crit Care Med 163:335–338PubMedGoogle Scholar
  6. 6.
    Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH (1991) Non-invasive mechanical ventilation for cystic fibrosis patients—a potential bridge to transplantation. Eur Respir J 4:524–527PubMedGoogle Scholar
  7. 7.
    Sweet SC (2003) Pediatric lung transplantation: update 2003. Pediatr Clin North Am 50:1393–1417PubMedCrossRefGoogle Scholar
  8. 8.
    Kroshus TJ, Kshettry VR, Savik K, et al. (1997) Risk factors for the development of bronchiolitis obliterans syndrome after lung transplantation. J Thorac Cardiovasc Surg 114:195–202PubMedCrossRefGoogle Scholar
  9. 9.
    Hadjiliadis D, Duane Davis R, Steele MP, et al. (2003) Gastroesophageal reflux disease in lung transplant recipients. Clin Transplant 17:363–368PubMedCrossRefGoogle Scholar
  10. 10.
    Gilljam M, Chaparro C, Tullis E, et al. (2003) GI complications after lung transplantation in patients with cystic fibrosis. Chest 123:37–41PubMedCrossRefGoogle Scholar
  11. 11.
    Cooper JD, Billingham M, Egan T, et al. (1993) A working formulation for the standardization of nomenclature and for clinical staging of chronic dysfunction in lung allografts. International Society for Heart and Lung Transplantation. J Heart Lung Transplant 12:713–716Google Scholar
  12. 12.
    Padman R, Lawless ST, Kettrick RG (1998) Noninvasive ventilation via bilevel positive airway pressure support in pediatric practice. Crit Care Med 26:169–173PubMedCrossRefGoogle Scholar
  13. 13.
    Efrati O, Modan-Moses D, Barak A, et al. (2004) Long-term non-invasive positive pressure ventilation among cystic fibrosis patients awaiting lung transplantation. Isr Med Assoc J 6:527–530PubMedGoogle Scholar
  14. 14.
    Keenan RJ, Lega ME, Dummer JS, et al. (1991) Cytomegalovirus serologic status and postoperative infection correlated with risk of developing chronic rejection after pulmonary transplantation. Transplantation 51:433–438PubMedCrossRefGoogle Scholar
  15. 15.
    El-Gamel A, Sim E, Hasleton P, et al. (1999) Transforming growth factor beta (TGF-beta) and obliterative bronchiolitis following pulmonary transplantation. J Heart Lung Transplant 18:828–837PubMedCrossRefGoogle Scholar
  16. 16.
    Sharples LD, Tamm M, McNeil K, et al. (1996) Development of bronchiolitis obliterans syndrome in recipients of heart-lung transplantation—early risk factors. Transplantation 61:560–566PubMedCrossRefGoogle Scholar
  17. 17.
    Flume PA, Egan TM, Paradowski LJ, et al. (1994) Infectious complications of lung transplantation. Impact of cystic fibrosis. Am J Respir Crit Care Med 149:1601–1607Google Scholar
  18. 18.
    Duncan SR, Paradis IL, Yousem SA, et al. (1992) Sequelae of cytomegalovirus pulmonary infections in lung allograft recipients. Am Rev Respir Dis 146:1419–1425PubMedGoogle Scholar
  19. 19.
    Billings JL, Hertz MI, Savik K, Wendt CH (2002) Respiratory viruses and chronic rejection in lung transplant recipients. J Heart Lung Transplant 21:559–566PubMedCrossRefGoogle Scholar
  20. 20.
    Zuckerman JB, Kotloff RM (1998) Lung transplantation for cystic fibrosis. Clin Chest Med 9:535–554CrossRefGoogle Scholar
  21. 21.
    Piper AJ, Parker S, Torzillo PJ, Sullivan CE, Bye PT (1992) Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure. Chest 102:846–850PubMedGoogle Scholar
  22. 22.
    Snell GI, Bennetts K, Bartolo J, et al. (1998) Body mass index as a predictor of survival in adults with cystic fibrosis referred for lung transplantation. J Heart Lung Transplant 17:1097–1103PubMedGoogle Scholar
  23. 23.
    Schwebel C, Pin I, Barnoud D, et al. (2000) Prevalence and consequences of nutritional depletion in lung transplant candidates. Eur Respir J 16:1050–1055PubMedCrossRefGoogle Scholar
  24. 24.
    Plochl W, Pezawas L, Artemiou O, et al. (1996) Nutritional status, ICU duration and ICU mortality in lung transplant recipients. Intensive Care Med 22:1179–1185PubMedGoogle Scholar
  25. 25.
    Madill J, Gutierrez C, Grossman J, et al. (2001) Toronto Lung Transplant Program. Nutritional assessment of the lung transplant patient: body mass index as a predictor of 90-day mortality following transplantation. J Heart Lung Transplant 20:288–296PubMedCrossRefGoogle Scholar
  26. 26.
    Sharma R, Florea VG, Bolger AP, et al. (2001) Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax 56:746–750PubMedCrossRefGoogle Scholar
  27. 27.
    Hardy KA, Anderson BD (1996) Noninvasive clearance of airway secretions. Respir Care Clin North Am 2:323–345Google Scholar
  28. 28.
    Holland AE, Denehy L, Ntoumenopoulos G, Naughton MT, Wilson JW (2003) Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax 58:880–884PubMedCrossRefGoogle Scholar
  29. 29.
    Daviskas E, Anderson SD (2006) Hyperosmolar agents and clearance of mucus in the diseased airway. J Aerosol Med 19:100–109PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • Ori Efrati
    • 1
  • Mordechai R. Kremer
    • 2
  • Asher Barak
    • 1
  • Arie Augarten
    • 1
  • Nira Reichart
    • 1
  • Amir Vardi
    • 3
  • Dalit Modan-Moses
    • 1
  1. 1.Pediatric Pulmonology UnitSafra Children’s Hospital, The Chaim Sheba Medical CenterTel-HashomerIsrael
  2. 2.Institute of PulmonologyRabin Medical Center (Beilinson Campus)Petah-TiqvaIsrael
  3. 3.Department of Pediatric Critical CareSafra Children’s Hospital, The Chaim Sheba Medical CenterTel-HashomerIsrael

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