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Cardiovascular Research 2006 72(1):30-40; doi:10.1016/j.cardiores.2006.07.025
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Copyright © 2006, European Society of Cardiology

Hypoxia- and non-hypoxia-related pulmonary hypertension – Established and new therapies

Hossein A. Ghofrani*, Robert Voswinckel, Frank Reichenberger, Norbert Weissmann, Ralph T. Schermuly, Werner Seeger and Friedrich Grimminger

Medical Clinic II/V, Department of Internal Medicine, University Hospital Giessen and Marburg GmbH, Klinikstrasse 36, 35392 Giessen, Germany

* Corresponding author. University of Giessen Lung Center, University Hospital Giessen and Marburg GmbH, Klinikstrasse 36, 35392 Giessen, Germany. Tel.: +49 641 99 42421; fax: +49 641 99 42419. Email address: ardeschir.ghofrani{at}uglc.de

Pulmonary hypertension can occur as an isolated disease affecting the lung vessels only, in association with underlying hypoxic lung disorders, or due to chronic thromboembolic disease. Pulmonary hypertension caused by pulmonary venous congestion will not be focused on in this review. Regardless of the underlying disease, chronic cor pulmonale is associated with progressive clinical deterioration and a poor prognosis in most cases. The aim of specific therapies for pulmonary hypertension is to reduce pulmonary vascular resistance and thereby improve right ventricular function. Currently, three classes of drugs (prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors) are approved for the treatment of pulmonary arterial hypertension (PAH) in a defined patient population (group I according to the recent WHO classification). However, these medications may also lower pulmonary vascular resistance in patients with associated lung diseases (e.g. chronic obstructive pulmonary disease or lung fibrosis) and significant pulmonary hypertension, for whom these drugs are not yet approved. As non-selective vasodilators may induce gas-exchange disturbances, which preclude their long term use in these patients, such substances should be avoided in the hypoxemic patient. In this article we provide an update of the current understanding of hypoxia- and non-hypoxia-related pulmonary hypertension, addressing both the pathophysiological understanding of different disease aetiologies as well as the therapeutic options currently available.

KEYWORDS Pulmonary hypertension; Hypoxia; Chronic obstructive pulmonary disease; Interstitial lung disease; Phosphodiesterase-5 inhibitor; Endothelin; Endothelin antagonist; Platelet derived growth factor; Prostacyclin


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