Exercise testing and prognosis in adult cystic fibrosis

Background - The assessment of prognosis is an important issue in cystic fibrosis. The prognostic value of exercise testing in comparison with other predictors of mortality was examined. Methods - Ninety two adult patients with cystic fibrosis performed progressive maximal exercise tests and outcome was assessed at five years. The results of exercise testing were examined along with spirometric values, age, sex, body mass index (BMI), and sputum culture. Results - Twenty two subjects died during the five year follow up period and 67 survived. Five subjects received a lung transplant and were excluded from the analysis. There were significant differences between those who survived and those who died: mean(SE) forced expiratory volume in one second (FEV(1)) 68.9 (2.7) versus 39.7 (3.5)% predicted, BMI 19.0 (0.3) versus 17.1 (0.4) kg/m(2), peak oxygen uptake (Vo(2) peak) 66.6 (2.2) versus 53.7 (3.7)% predicted, peak work rate (Wpeak) 89.4 (3.8) versus 71.2 (5.5)% predicted, peak minute ventilation (VEpeak) 51.3 (2.0) versus 43.3 (3.1) 1/min, and ventilatory equivalent for oxygen (VE/Vo(2)) 32.4 (0.6) versus 38.7 (1.7). Age, sex, oxygen saturation and Burkholderia cepacia colonisation were not found to be significant predictors of mortality. When significant independent factors were entered into a multivariate logistic regression model only FEV(1) was found to be a significant correlate of mortality. A cutoff for FEV(1) of 55% predicted gave the best combination of specificity and sensitivity with 54% of those below this value dying within five years and 96% of those above it surviving. Conclusions - The results of maximal exercise testing are correlated with survival but they are not better than the FEV, as prognostic indicators.