Interv Akut Kardiol. 2009;8(2):81-86

Pulmonary arterial hypertension

MUDr. Pavel Jansa¹, David Ambrož¹, Pavel Poláček¹, Jana Marešová¹, Ludmila Jelínková¹, Michael Aschermann², Aleš Linhart¹

¹ Centrum pro plicní hypertenzi, II. interní klinika kardiologie a angiologie, VFN a 1. LF UK, Praha

² II. interní klinika kardiologie a angiologie 1. LF UK a VFN, Praha

Pulmonary arterial hypertension (PAH) is primary diasease of pulmonary arterioles, which arises from unknown causes (idiopathic and

familiar PAH) or it is associated with known cause (connective tissue diseases, liver diseases, congenital heart diseases, HIV infection,

anorexigen intake). Changes in the pulmonary circulation lead to precapillary pulmonary hypertension, which progresses quickly without

therapy, leads to right heart failure and death. Up to the mid-nineties the therapeutic procedures used in PAH (treatment of heart

failure, nonspecific vasodilator and anticoagulant therapy) have not been successful. The introduction of intravenous prostacyclin and

later its analogs and the orally administered endothelin receptor antagonist and phosphodiesterase-5 inhibitors changed the situation

significantly. In recent years the specific vasodilator therapy is used not only as monotherapy but also as combination therapy. Specific

pharmacotherapy significantly improves not only symptoms and functional capacity, but also prognosis of the patients. The late diagnosis

of PAH remains as important problem. The cause of this problem is late and nonspecific manifestation of the symptoms. The basic

assessment used to detect PAH is doppler echocardiography. Echocardiography should be regularly performed in patients with increased

risk of PAH. The right heart catheterization is necessary to establish definitive diagnosis. Centralization is trend in the management of

patients with PAH. In the Czech Republic the treatment of PAH is centralized on 2nd Internal Clinic of the General University Hospital and

Charles University in Prague and on the Cardiology Clinic of Institute of Clinical and Experimental Medicine in Prague.

Keywords: pulmonary arterial hypertension, prostacyclin, endothelin receptor antagonists, phosphodiesterase-5 inhibitors.

Published: May 1, 2009  Show citation