Lung and heart-lung transplantation in patients with end-stage cystic fibrosis: the Stanford experience.
BACKGROUND
Bilateral lung (BLTx) and heart-lung transplantation have gained wide acceptance as treatment of end-stage lung disease from cystic fibrosis. We reviewed our 13-year experience with thoracic transplantation for cystic fibrosis with an operative approach that favors use of cardiopulmonary bypass for BLTx.


METHODS
Sixty-four patients with cystic fibrosis underwent heart-lung transplantation (n = 22, 34.4%) or BLTx (n = 42, 65.6%) between 1988 and 2000. Mean age and weight at transplantation were 29 +/- 8 years and 51 +/- 11 kg, respectively. Mean follow-up for survivors was 4.4 +/- 3.6 years. Immunosuppression regimen included cyclosporine, tapered corticosteroids, azathioprine, and induction therapy with OKT3 (murine monoclonal antibodies) or rabbit antithymocyte globulin. Cardiopulmonary bypass was used in all but 5 patients (7.8%). However, in 8 (19%) of the 42 patients having BLTx, only the grafting of the second lung was performed with cardiopulmonary bypass.


RESULTS
The operative mortality rate was 1.6%. The actuarial survival rates at 1 year, 3 years, 5 years and 10 years were 93.2%, 77.7%, 61.8%, and 48.1%, respectively, with no significant difference between BLTx and heart-lung transplantation. The major hospital complications were pneumonia (n = 11, 17.2%) and bleeding (n = 8, 12.5%). Clinically significant reperfusion injury was observed in 6 patients, 3 of whom required reintubation. Freedom from acute lung rejection beyond 1 year was 47.7%. One patient underwent late retransplantation, and 4 required bronchial stenting. Obliterative bronchiolitis accounted for eight (50.0%) of 16 late deaths.


CONCLUSIONS
Though postoperative bleeding and pneumonia are still of concern, satisfactory early and intermediate-term results can be expected in patients undergoing BLTx or heart-lung transplantation for cystic fibrosis. Cardiopulmonary bypass can be used for BLTx with no adverse impact on intermediate and long-term outcomes.
Department of Cardiothoracic Surgery, Stanford University School of Medicine, California 94305-5407, USA. vricella@stanford.edu
Luca A
LA
Vricella
John M
JM
Karamichalis
Shahzad
S
Ahmad
Robert C
RC
Robbins
Richard I
RI
Whyte
Bruce A
BA
Reitz
D000328:Adult; D002315:Cardiopulmonary Bypass; D003550:Cystic Fibrosis / Q000601:surgery*; D005260:Female; D016041:Heart-Lung Transplantation / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D016040:Lung Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D012189:Retrospective Studies; D016019:Survival Analysis