Lung Transplantation for Cystic Fibrosis

  • T. M. Egan


Cystic fibrosis (CF) is the most common lethal genetic disease of Caucasians. Although it is a multisystem disease it is estimated that 95% of patients with CF will succumb from end-stage pulmonary disease[1]. Up to 400 patients with CF die annually in the United States. The past decade has seen remarkable advances in understanding the pathophysiology of CF, including identification of a chloride channel abnormality 3. identification of the gene responsible for the chloride channel[4], and creation of a transgenic mouse with CF[5]. Even before the genetic defect was identified, it was apparent that the chloride channel epithelial abnormality present in CF, evidenced by an altered potential difference[3], was not manifested in the epithelium of heart-lung grafts in transplanted CF patients[6]. This provided assurance that the pulmonary abnormalities observed in CF patients were unlikely to recur following lung transplantation.


Cystic Fibrosis Lung Transplant Bronchiolitis Obliterans Syndrome Lung Transplant Recipient Airway Complication 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Scanlin TF. Cyslic fibrosis. In: Fishman AP, editor. Pulmonary diseases and disorders, 2nd edn. New York: McGraw-Hill; 1988:1273–94.Google Scholar
  2. 2.
    Fitz Simmons SC. The changing epidemiology of cyslic fibrosis. J Pediatr. 1993;122:1–9.CrossRefGoogle Scholar
  3. 3.
    Knowles M, Gatzy J, Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic librosis. N Engl J Med. 1981;305:1489–95.PubMedCrossRefGoogle Scholar
  4. 4.
    Riordan JR, Rommens JM, Kerem B-S et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245:1066–73.PubMedCrossRefGoogle Scholar
  5. 5.
    Snouwaert JN, Brigman KK, Latour AM et al. An animal model for cystic librosis made by gene targeting. Science. 1992;257:1083–88.PubMedCrossRefGoogle Scholar
  6. 6.
    Wood A, Higenbottam T, Jackson M, Scott J, Stewart S, Wallwork J. Airway mucosal bioelectric potential difference in cystic fibrosis after lung transplantation. Am Rev Respir Dis. 1989;140:1645–9.PubMedGoogle Scholar
  7. 7.
    Egan TM, Detterbeck FC, Mill MR et al. Improved results of lung transplantation for cystic fibrosis. J Thorac Cardiovasc Surg. 1995;109:224–35.PubMedCrossRefGoogle Scholar
  8. 8.
    Reitz BA, Wallwork JL, Hunt SA et al. Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med. 1982;306:557–64.PubMedCrossRefGoogle Scholar
  9. 9.
    Yacoub MH, Banner NR, Khaghani A et al. Heart-lung transplantation for cystic fibrosis and subsequent domino heart transplantation. J Heart Transplant. 1990;9:459–67.PubMedGoogle Scholar
  10. 10.
    Toronto Lung Transplant Group. Unilateral lung transplantation for pulmonary fibrosis. N Engl J Med. 1986;314:1140–5.CrossRefGoogle Scholar
  11. 11.
    Hardy JD, Webb WR, Dalton ML, Walker GR. Lung homotransplantation in man. J Am Med Assoc. 1963;186:1065–74.Google Scholar
  12. 12.
    Dark JH, Patterson GA, Al-Jilaihawi AN, Hsu H, Egan T, Cooper JD. Experimental en bloc double-lung transplantation. Ann Thorac Surg. 1986;42:394–8.PubMedCrossRefGoogle Scholar
  13. 13.
    Patterson GA, Cooper JD, Dark JH, Jones MT, and the Toronto Lung Transplant Group. Experimental and clinical double lung transplantation. J Thorac Cardiovasc Surg. 1988;95:70–4.PubMedGoogle Scholar
  14. 14.
    Patterson GA, Todd TR, Cooper JD, Pearson FG, Winton TL, Maurer J, and the Toronto Lung Transplant Group. Airway complications after double lung transplantation. J Thorac Cardiovasc Surg. 1990;99:14–21.PubMedGoogle Scholar
  15. 15.
    Noirclerc MJ, Metras D, Vaillant A et al. A Bilateral bronchial anastomosis in double lung and heart-lung transplantations. Eur J Cardio-thorac Surg. 1990;4:314–17.CrossRefGoogle Scholar
  16. 16.
    Pasque MK, Cooper JD, Kaiser LR, Haydock DA, Triantafillou A, Trulock EP. Improved technique for bilateral lung transplantation: rationale and initial clinical experience. Ann Thorac Surg. 1990;49:785–91.PubMedCrossRefGoogle Scholar
  17. 17.
    Billingham ME. Cardiac transplant atherosclerosis. Transplant Proc. 1987;19(Suppl. 5):19–25.PubMedGoogle Scholar
  18. 18.
    Traill TA. Physiology and function of the transplant allograft. In: Baumgartner WA, Reitz BA, Achuff SC, editors. Heart and heart-lung transplantation. Philadelphia, PA: Saunders; 1990:266–78.Google Scholar
  19. 19.
    Frist WH, Fox MD, Campbell PW, Fiel SB, Loyd JE, Merrill WH. Cystic fibrosis treated with heart-lung transplantation: North American results. Transplant Proc. 1991;23:1205–6.PubMedGoogle Scholar
  20. 20.
    de Leval MR, Smyth R, Whitehead B et al. Heart and lung transplantation for terminal cystic librosis: a 4 1/2-year experience. J Thorac Cardiovasc Surg. 1991;101:633–42.PubMedGoogle Scholar
  21. 21.
    Ramirez JC, Patterson GA, Winton TL, De Hoyos AL, Miller JD, Maurer JR, and the Toronto Lung Transplant Group. Bilateral lung transplantation for cystic fibrosis. J Thorac Cardiovasc Surg. 1992;103:287–94.PubMedGoogle Scholar
  22. 22.
    Shennib H, Noirclere M, Ernst P et al., the Cystic Fibrosis Transplant Study Group. Double-lung transplantation for cystic fibrosis. Ann Thorac Surg. 1992;54:27–32.PubMedCrossRefGoogle Scholar
  23. 23.
    Egan TM, Trulock E, Boychuk J, Ochoa L, Cooper J. Analysis of patient referrals for lung transplantation. Chest. 1991;99:867–70.PubMedGoogle Scholar
  24. 24.
    Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326:1187–91.PubMedCrossRefGoogle Scholar
  25. 25.
    Ciriaco P, Egan TM, Cairns EL, Thompson JT, Delterbeck FC, Paradowski LJ. Analysis of cyslic fibrosis referrals for lung transplantation. Chest. 1995;107:1323–7.PubMedGoogle Scholar
  26. 26.
    Reisman J, Corey M, Canny G, Levison H. Diabetes mellitus in patients with cyslic fibrosis: effect on survival. Pediatrics. 1990;86:374–7.PubMedGoogle Scholar
  27. 27.
    Madill J, Maurer JR, De Hoyos A. A comparison of preopcrative and postoperative nutritional states of lung transplant recipients. Transplantation. 1993;56:347–50.PubMedCrossRefGoogle Scholar
  28. 28.
    Massard G, Shennib H, Metras D et al. Double-lung transplantation in mechanically ventilated patients with cystic fibrosis. Ann Thorac Surg. 1993;55:1087–92.PubMedCrossRefGoogle Scholar
  29. 29.
    Flume PA, Egan TM, Westerman JH et al. Lung transplantation for mechanically ventilated patients. J Heart Lung Transplant. 1994;13:15–21.PubMedGoogle Scholar
  30. 30.
    Snell GI, De Hoyos A, Krajden M, Winton T, Maurer JR. Pseudomonas cepacia in lung transplant recipients with cystic fibrosis. Chest. 1993;103.466–71.PubMedGoogle Scholar
  31. 31.
    Steinbach S, Sun L, Jiang R-Z et al. Persistent, clonal Pseudomonus cepacia infection in cystic fibrosis lung transplant recipients and clinic patients. N Engl J Med. 1994;331:981–7.PubMedCrossRefGoogle Scholar
  32. 32.
    Egan TM, Thompson JT, Detterbeck FC et al. Effect of size (mis)matching in clinical double lung transplantation. Transplantation. 1995;59:707–13.PubMedCrossRefGoogle Scholar
  33. 33.
    Egan TM, Paradowski LJ, Detterbeek FC, Mill MR. Is bronchial omentopexy helpful in human lung transplantation? Chest. 1992; 102(Suppl):74S (abstract).Google Scholar
  34. 34.
    Miller JD, De Hoyos A. An evaluation of the role of omentopexy and of early peri-operative corticosteroid administration in clinical lung transplantation. J Thorac Cardiovasc Surg. 1993;105:247–52.PubMedGoogle Scholar
  35. 35.
    Cuilli F, Malouf M, Ferrari L et al. Incidence, evolution of anastomotic technique and treatment of airway complications in lung transplantation. J Heart Lung Transplant. 1995;14(Suppl.):S60 (abstract).Google Scholar
  36. 36.
    Anderson MB, Kriett JM, Harrcll J et al., Techniques for bronchial anastomosis. J Heart Lung Transplant. 1995;14(Suppl.):S60 (abstract).Google Scholar
  37. 37.
    Smith JM, Hows JM, Gordon-Smith EC, Baughan A, Goldman JM. Interaction of CyA and ketoconazole. Clin Sci. 1983;64:67–8.Google Scholar
  38. 38.
    Shennib H, Auger J-L. Diltiazem improves cyclosporin dosage in cystic fibrosis lung transplant recipients. J Heart Lung Transplant. 1994;13:292–6.PubMedGoogle Scholar
  39. 39.
    Kriett JM, Kayo MP. The Registry of the International Society for Heart and Lung Transplantation: eighth official report-1991. J Heart Lung Transplant. 1991;10:491–8.PubMedGoogle Scholar
  40. 40.
    Hosenpud JD, Novick RJ, Breen TJ, Daily OP. The Registry of the International Society for Heart and Lung Transplantation: eleventh official report-1994. J Heart Lung Transplant. 1994;13:561–70.PubMedGoogle Scholar
  41. 41.
    Hosenpud JD. The Registry of the International Society for Heart and Lung Transplantation: twelfth official report-1995. J Heart Lung Transplant. 1995;808–15.Google Scholar
  42. 42.
    Egan T, Detterbeck F, Paradowski L et al. Intermediate term results of lung transplant for CF. Pediatr Pulmonol. (In press).Google Scholar
  43. 43.
    Cooper JD, Billingham M. Egan T et al. A working formulalion for the standardization of nomenclature and for clinical staging of chronic dysfunction in lung allografts (Consensus Document). J Heart Lung Transplant. 1993;12: 713–16.PubMedGoogle Scholar
  44. 44.
    Dumon J-E. A dedicated tracheobronchial stent. Chest. 1990;97:328–32.PubMedGoogle Scholar
  45. 45.
    Flume PA, Egan TM, Paradowski LJ, Detterbeck FC, Thompson JT, Yankaskas JR. Infectious complications of lung transplantation: impact of cystic fibrosis. Am Rev Respir Crit Care Med. 1994;149:1601–7.Google Scholar
  46. 46.
    Lewiston N, King V, Umetsu D et al. Cystic fibrosis patients who have undergone heart-lung transplantation benelit from maxillary sinus antrostomy and repeated sinus lavage. Transplant Proc. 1991;23:1207–8.PubMedGoogle Scholar
  47. 47.
    Dennis C, Caine N, Sharpies L et al. Heart-lung transplantation for end stage respiratory disease in cystic fibrosis patients at Papworth Hospital. J Heart Lung Transplant. 1993;12:893–902.PubMedGoogle Scholar
  48. 48.
    Gibson DL. Lung transplantation for cyslic librosis: a patient’s perspective. Pediatr Pulmonol. 1992;(Suppl. 8):213–14.Google Scholar
  49. 49.
    Al-Dossari GA, Kshettry VR, Jessurun J, Bolman RM III. Experimental large-animal model of obliterative bronchioluis after lung transplantation. Ann Thorac Surg. 1994;58:34–40.PubMedCrossRefGoogle Scholar
  50. 50.
    Starnes VA, Barr ML, Cohen RG. Lobar transplantation: indications, technique, and outcome. J Thorac Cardiovasc Surg. 1994;108:403–11.PubMedGoogle Scholar
  51. 51.
    Starnes VA, Barr ML, Cohen FA, Schenkel FA, Barbers RG and the USC Transplant Group. Bilateral living-related lobar transplantation for cystic fibrosis: initial experience. J Heart Lung Transplant. 1994;13(Suppl.):S57 (abstract).Google Scholar
  52. 52.
    Cohen RG, Barr ML, Schenkel FA, DcMeester TR, Wells WJ, Starnes VA. Living-related donor lobectomy for bilateral lobar transplantation in patients with cystic fibrosis. Ann Thorac Surg. 1994;57:1423–8.PubMedCrossRefGoogle Scholar
  53. 53.
    Platt JL, Bach FH. The barrier to xenotransplantation. Transplantation. 1991;52:937–47.PubMedCrossRefGoogle Scholar
  54. 54.
    Egan TM, Lambert CJ Jr. Reddick RL, Ulicny KS Jr. Keagy BA, Wilcox BR. A strategy to increase the donor pool: the use of cadaver lungs for transplantation. Ann Thorac Surg. 1991;52:1113–21.PubMedCrossRefGoogle Scholar
  55. 55.
    D’Armini AM, Roberts CS, Griffith PK, Lemasters JJ, Hgan TM. When does the lung die? I. Histochemical evidence of pulmonary viability aftcr ‘death’. J Heart Lung Transplant. 1994;13:741–7.PubMedGoogle Scholar
  56. 56.
    Alessandrini F, D’Armini AM, Roberts CS, Reddick RL, Egan TM. When does the lung die? II. Ultrastructural evidence of pulmonary viability after ‘death’. J Heart Lung Transplant. 1994;13:748–57.PubMedGoogle Scholar
  57. 57.
    D’Armini AM, Tom EJ, Roberts CS, Henke DC, Lemasters JJ, Egan TM. When docs the lung die? Time course of high energy phosphate depletion and relationship to lung viability after ‘death’. J Surg Res. 1995;468–74.Google Scholar
  58. 58.
    Egan TM, Detterbeck FC. Technique and results of double lung transplantation. Chest Surg Clin N Am. 1993;3:89–111.Google Scholar

Copyright information

© Kluwer Academic Publishers 1996

Authors and Affiliations

  • T. M. Egan

There are no affiliations available

Personalised recommendations