How is idiopathic pulmonary arterial hypertension (IPAH) defined?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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By definition, pulmonary hypertension refers to the condition in which resting mean pulmonary arterial pressure (mPAP) is greater than 25 mmHg. Further, in order to hemodynamically distinguish PAH (IPAH and APAH) from other forms of PH, the pulmonary capillary wedge (PCW) pressure must be less than 15 mmHg, and the pulmonary vascular resistance (PVR) must be more than 3 Wood Units. Thus, cardiac catheterization is the criterion standard test to definitively confirm any form of PAH, including IPAH. However, a thorough workup includes a range of additional testing to exclude all reasonable causes of secondary pulmonary hypertension (see Workup).

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