Pathobiology of pulmonary arterial hypertension and right ventricular failure

被引：226

作者：

Voelkel, Norbert F. ^{[1
]}

Gomez-Arroyo, Jose

Abbate, Antonio

Bogaard, Harm J. ^{[3
]}

Nicolls, Mark R. ^{[2
]}

机构：

[1] Virginia Commonwealth Univ, Victoria Johnson Ctr Obstruct Lung Dis Res, Dept Med, Richmond, VA 23298 USA

[2] Stanford Univ, Dept Pulm & Crit Care Med, Palo Alto, CA 94304 USA

[3] Vrije Univ Amsterdam Med Ctr, Dept Pulm Med, Amsterdam, Netherlands

来源：

EUROPEAN RESPIRATORY JOURNAL | 2012年 / 40卷 / 06期

关键词：

Bone morphogenic type II receptor; genetics; growth factors; inflammation; ENDOTHELIAL-CELL GROWTH; RIGHT-HEART-FAILURE; REGULATORY T-CELLS; MORPHOGENETIC PROTEIN-RECEPTOR; SYSTEMIC-LUPUS-ERYTHEMATOSUS; MOLECULAR-MECHANISMS; PROGENITOR CELLS; BMPR2; MUTATIONS; IMMUNOSUPPRESSIVE THERAPY; CLINICAL-OUTCOMES;

D O I：

10.1183/09031936.00046612

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Pulmonary arterial hypertension (PAH) is no longer an orphan disease. There are three different classes of drugs for the treatment of PAH that are currently being used and an increasing number of patients are being treated with a single drug or combination therapy. During the last 25 yrs, new insights into the pathobiology of PAH have been gained. The classical mechanical concepts of pressure, flow, shear stress, right ventricle wall stress and impedance have been complemented with the new concepts of cell injury and repair and interactions of complex multicellular systems. Integrating these concepts will become critical as we design new medical therapies in order to change the prognosis of patients with these fatal diseases. This review intends to summarise recent pathobiological concepts of PAH and right ventricle failure mainly derived from human studies, which reflect the progress made in the understanding of this complex group of pulmonary vascular diseases.

引用

页码：1555 / 1565

页数：11

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