Background: Heart lung transplantation for++ cystic fibrosis is now performed in patients with severe lung disease but the experience is still scarce with the exception of some specialized centers.
Patients and methods: Twenty-one patients underwent heart-lung transplantation between September 1989 and November 1994 in our institution, with a high standard of reliability in tracheal anastomosis and with a low incidence of hospital mortality (5%).
Results: The actuarial patient survival is 90.2% (95% confidence interval, 70 to 97%) at 1 year and 75.7% (95% confidence interval, 51 to 90%) at 3 and 4 years. The mean forced expiratory volume in 1 second (FEV1) increases from 20.1% predicted preoperatively to 76.1%.
Conclusion: Despite the presence of airway pathogens, these results confirm that heart-lung transplantation for cystic fibrosis leads to a pronounced improvement in lung function and good rehabilitation after surgery. The two main obstacles are the shortage of donor organs and the possibility of late deterioration in lung function with a progressive airflow obstruction.