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The definition of Pulmonary arterial hypertension (PAH)

A disorder primarily of small pulmonary arteries which results in a progressive rise in pulmonary vascular resistance and right ventricular failure. There are several forms of PAH including Idiopathic Pulmonary Arterial Hypertension (IPAH) and Associated PAH related to a range of disease processes, including connective tissue disease, congenital heart disease, HIV infection and sickle-cell disease. For the haemodynamic definition, please refer to the website.

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If there are any questions about these definitions or how to apply them, please contact your PAH centre or specialist.