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PVRI Review  2010 

The diseased endothelium: Would it explain it all?

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Abstract:

The field of pulmonary hypertension pathogenesis has struggled with the pseudo-dichotomy of the causal role of pulmonary vascular endothelium vis- -vis smooth muscle cells as the central element of the disease. Medial hypertrophy and intima thickening due to migration or proliferation of vascular smooth muscle cells is seen in all forms of pulmonary hypertension, while endothelial cell lesions, particularly plexiform and concentric lesions, predominate in cases of severe pulmonary hypertension. Growing insight that the pathogenesis of pulmonary hypertension involves metabolic alterations of pulmonary vascular cells, including vascular smooth muscle and endothelial cells, opens new venues for therapies and diagnoses.

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