SYSTEMIC HEMODYNAMIC-CHANGES IN PATIENTS WITH CYSTIC-FIBROSIS WITH AND WITHOUT CHRONIC LIVER-DISEASE

Background/Aims: There are well-documented systemic haemodynamic chang es associated with chronic liver disease. Patients with cystic fibrosi s may develop chronic liver disease, but it is not known whether these systemic haemodynamic changes develop and, if they do, whether they a re influenced by the associated chronic lung disease. We therefore und ertook a study to document the circulatory status of cystic fibrosis p atients with and without chronic liver disease. Methods: Fifty-six sub jects with cystic fibrosis were studied. Systemic haemodynamic and pul monary parameters, in sub-groups both with (LD) and without (NLD) live r 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 a nd in the fourth minute of seated bicycle exercise at 25W. Heart rate (EGG), blood pressure (semiautomated sphygmomanometer), aortic blood v elocity (pulsed Doppler suprasternal probe), arterial oxygen saturatio n (pulse oximeter) and respiratory variables (pneumotachometer with ex pired gas analysis by an automatic system) were measured. Results: A c omplete 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 liv er 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 deterio rating hepatic and pulmonary function. Conclusions: Cystic fibrosis pa tients with chronic liver disease have a hyperdynamic circulation simi lar to that documented in other forms of chronic liver disease. These circulatory changes are exacerbated by deteriorating hepatic and pulmo nary function.