Systemic haemodynamic changes in patients with cystic fibrosis with and without chronic liver disease

Background/Aims: There are well-documented systemic haemodynamic changes associated with chronic liver disease. Patients with cystic fibrosis may develop chronic liver disease, but it is not known whether these systemic haemodynamic changes develop and, if they do, whether they are influenced by the associated chronic lung disease. We therefore undertook a study to document the circulatory status of cystic fibrosis patients with and without chronic liver disease. Methods: Fifty-six subjects with cystic fibrosis were studied. Systemic haemodynamic and pulmonary parameters, in sub-groups both with (LD) and without (NLD) liver disease, were measured at rest and during measured exertion. Cystic fibrosis-related chronic liver disease was diagnosed using previously validated ultrasound criteria. Patients underwent assessment at rest and in the fourth minute of seated bicycle exercise at 25W. Heart rate (ECG), blood pressure (semiautomated sphygmomanometer), aortic blood velocity (pulsed Doppler suprasternal probe), arterial oxygen saturation (pulse oximeter) and respiratory variables (pneumotachometer with expired gas analysis by an automatic system) were measured. Results: A complete data set was available for 45 patients (22 LD) at rest and 40 patients (19 LD) on exercise. The patients were well matched for age, sex, height, weight, and pulmonary function. Patients with chronic liver disease had a hyperkinetic circulation while ventilatory variables before and during exercise were similar for the two groups. There was evidence that the circulatory changes were exacerbated by both deteriorating hepatic and pulmonary function. Conclusions: Cystic fibrosis patients with chronic liver disease have a hyperdynamic circulation similar to that documented in other forms of chronic liver disease. These circulatory changes are exacerbated by deteriorating hepatic and pulmonary function.