Pulmonary Hypertension and Right Ventricular Dysfunction in Chronic Lung Diseases: New Pathobiologic Concepts

Jose Gomez-Arroyo, Herman J. Bogaard, Laszlo Farkas, Antonio Abbate, Norbert F. Voelkel

Abstract

Here we provide background information for the pathobiological concept of a “sick lung circulation”, and how it may affect cardiac function. We propose that in most chronic and progressive lung diseases, but in particular COPD/emphysema and interstitial lung diseases (ILD), there are significant pathological changes of the small pulmonary vessels. Complementing the WHO classification of pulmonary vascular diseases we provide a classification which is based on pathogenetic mechanisms such as inflammation, hypoxia and angioproliferation. We also propose that chronic lung disease – related ‘cor pulmonale’ is insufficiently explained by an elevation of the pulmonary arterial pressure.

Keywords: COPD, emphysema, lung vascular remodeling, HIF-1 &agr;, VEGF, endothelial-mesenchyma transition [EnMT], “sick lung circulation”

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