How is idiopathic pulmonary arterial hypertension (IPAH) classified?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Within this classification, IPAH represents a subset of pulmonary vascular disease called pulmonary arterial hypertension (Group I PH, or PAH), which includes conditions known to be associated with pulmonary hypertension that share similar pathophysiology to IPAH. Conditions in which PAH and these associated conditions co-exist are called associated PAH (APAH).

Dresdale and colleagues first reported a hemodynamic account of IPAH in 1951. [3] However, the pathophysiology of IPAH remains poorly understood. At least 15-20% of patients previously thought to have IPAH actually have a familial (heritable) form of PAH involving at least one genetic defect, which has only recently been characterized (see Pathophysiology).


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