Cor Vasa 2009, 51(7-8):455-461 | DOI: 10.33678/cor.2009.118

Pulmonary arterial hypertension

Pavel Jansa^*, David Ambrož, Pavel Poláček, Jana Marešová, Michael Aschermann, Aleš Linhart

Centrum pro plicní hypertenzi, 2. interní klinika kardiologie a angiologie, Všeobecná fakultní nemocnice a 1. lékařská fakulta -Univerzity Karlovy, Praha, Česká republika

Pulmonary hypertension is a heterogeneous group of diseases characterized by increased mean pulmonary artery pressure. Clinical classification distinguishes pulmonary arterial hypertension, pulmonary hypertension associated with left-heart disease, pulmonary hypertension associated with lung disease and/or hypoxemia, chronic thromboembolic pulmonary hypertension and/or embolic disease, and miscellaneous. Pulmonary arterial hypertension (PAH) is the primary disease involving the lung arterioles and occurring either from unknown causes (idiopathic or, possibly, familial PAH), or its development is associated with a known causative factor connective tissue diseases, liver disease, congenital shunts between the systemic and pulmonary circulations, HIV infection, abuse of some anorectics.
The principal diagnostic method in PAH is echocardiography with Doppler. Patients at increased risk for developing PAH should have regular echocardiographic examination. However, the definitive diagnosis can only be established by right-heart catheterization. Late diagnosis of PAH continues to be a clinical challenge, with the reason being mainly non-specific symptoms presenting late.
The goal of therapeutic intervention in PAH is to modulate the underlying pathophysiological mechanisms (vasoconstriction, remodeling, and thrombosis). The choice of drug therapy is made on the basis of acute pulmonary vasodilator testing. Only patients testing positive are scheduled for therapy with high-dose calcium-channel blockers. This therapy, combined with anticoagulation therapy and treatment of heart failure, is referred to as conventional therapy. Those testing negative are indicated for so-called specific drug therapy (prostanoids, endothelin receptor antagonists, phosphodiesterase 5 inhibitors) in addition to chronic anticoagulation therapy. Once the drug therapy options have failed, atrial septostomy and lung transplantation are to be considered.

Keywords: Pulmonary arterial hypertension; Prostanoids; Endothelin receptor antagonists; Phosphodiesterase 5 inhibitors

Published: July 1, 2009  Show citation