Gas exchange in stable patients with moderate-to-severe lung disease from cystic fibrosis

Background: Studies using the multiple inert gas elimination technique (MIGET) to characterise the mechanisms of impaired gas exchange in CF, provide conflicting results on the importance of ventilation-perfusion (V-A/Q) inequality over shunt. We hypothesise that the mechanisms of gas exchange abnormality have changed with changing CF management over the last two decades. Methods: Detailed gas exchange was evaluated by MIGET with venous sampling in stable patients, age>20 years, FEV1% predicted <= 50. Results: Fifteen (14 male) subjects were studied with a mean +/- SD age 28.1 +/- 8.4 years, FEV1% 32.6 +/- 10.3 TLC% 111.5 +/- 12.9, NO, 9.3 +/- 1.3 kPa, (69.5 +/- 9.6 mm Hg), and PaCO2 6.2 +/- 0.7 kPa, (45.9 +/- 5.3 mm Hg). The predominant gas exchange abnormality was V-A/Q inequality with a log SD of the distributions Of Perfusion 0.91 +/- 0.30 and of ventilation 0.60 +/- 0.14. Unimodal distributions were seen in nine subjects, a low V-A/Q mode in five and one subject had a bimodal distribution, mean intrapulmonary shunt was negligible. Conclusions: Subjects had a lower FEV1% by comparison with previously published studies and demonstrated severe V-A/Q inequality and negligible shunt. This Suggests a low degree of complete obstruction of airways in adults with CF and severe stable Pulmonary disease. The primary mechanism of hypoxaemia in CF Subjects reaching adulthood today appears to have changed with modern management over the last two decades. (C) 2007 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.