HEART-LUNG TRANSPLANTATION FOR CYSTIC-FIB ROSIS

Background.- Heart lung transplantation for cystic fibrosis is now per formed in patients with severe lung disease but the experience is stil l scarce with the exception of some specialized centers. Patients and methods.- Twenty-one patients underwent heart-lung transplantation bet ween September 1989 and November 1994 in our institution, with a high standard of reliability in tracheal anastomosis and with a low inciden ce of hospital mortality (5%). Results.- The actuarial patient surviva l is 90.2% (95% confidence interval, 70 to 97%) at 1 year and 75.7% (9 5% confidence interval, 51 to 90%) at 3 and 4 years. The mean forced e xpiratory volume in 1 second (FEV1) increases from 20.1% predicted pre operatively to 76.1%. Conclusion.- Despite the presence of airway path ogens, these results confirm that heart-lung transplantation for cysti c fibrosis leads to a pronounced improvement in lung function and goon rehabilitation after surgery. The two main obstacles are the shortage of donor organs and the possibility of late deterioration in lung fun ction with a progressive airflow obstruction.