Molecular genetics of pulmonary arterial hypertension

Published on September 3, 2014   21 min

Other Talks in the Series: Molecular Genetics of Human Disease

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Professor Richard Trembath and I'm going to talk to you today about the molecular genetics of pulmonary arterial hypertension.
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Pulmonary hypertension is defined as an elevation of the pulmonary arterial pressure and has a multitude of causes. The recent classification from an expert group identified some of the associated conditions that can lead to an elevation of the pressure within the pulmonary arterial circulation. A noted important group of patients who have no known or recognized associated cause and fit into the category of heritable and idiopathic pulmonary arterial hypertension are the focus of this talk.
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The characteristic histopathology of pulmonary arterial hypertension in its idiopathic and heritable form is one of increase in the smooth musculature of the pulmonary arterial circulation, a predisposition to formation of intravascular thrombosis and occlusion together with the pathognomonic plexiform lesions that are shown in this illustration in panel D.
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Molecular genetics of pulmonary arterial hypertension

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