Multiple factors limit exercise capacity in cystic fibrosis.

Exercise testing was performed in 50 patients with cystic fibrosis to determine whether hemodynamic factors limit exercise capacity in the disease. Prior to exercise testing, lung function and blood gas values were measured. Nutritional status was determined by calculating a weight for height (Wt for Ht) ratio for each subject. A progressive exercise test was used to determine maximum work capacity (Wmax). Cardiac output (Q) (indirect Fick method), and stroke volume (SV) were computed during steady-state exercise at 50% Wmax in 21 of 50 patients. Wmax, SV, Q, and lung function results are expressed as per cent predicted. The mean (+/- SD) Wmax was 75 +/- 23%. Multiple regression analysis showed that maximum voluntary ventilation, resting PaO2, and Wt for Ht accounted for 84% of the variance in Wmax. Although some patients had a reduced SV (mean = 96%) during steady-state exercise, all patients achieved a normal cardiac output (mean = 115%). SV correlated with resting PaO2 but not with lung function. We conclude that exercise capacity in cystic fibrosis is influenced by lung function, nutritional status, and resting hypoxemia, but not by cardiac function; the SV limitation noted in some patients may be due to increased pulmonary vascular resistance related to hypoxemia.