[Mucoviscidosis and precapillary pulmonary hypertension]

Various pathomechanisms are responsible for the development of pulmonary arterial hypertension (PAH) in cystic fibrosis (CF). Main factors are chronic hypoxia and the loss of peripheral vessels caused by recurrent infectious lung disease and lung fibrosis. Measurement of pulmonary artery pressure and pulmonary resistance provide reliable information on PAH. Many non invasive procedures, including echocardiography, myocardial and pulmonary imaging by radionuclide, proved to be not sensitive enough to diagnose PAH. Arterialized pO2 (taken from the hyperemized ear lobe), however, measured during standardized submaximum work load, seems to be a valuable parameter to estimate PAH (r = 0.92). Treatment of PAH in CF-patients is, at the moment, predominantly based on the improvement of the general therapeutical regimen of CF and on long-term oxygen insufflation, which should be initiated as soon as there are first signs of PAH.