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Pathobiology of PAH 

Pathobiology of PAH
Chapter:
Pathobiology of PAH
Author(s):

Clive Handler

and Gerry Coghlan

DOI:
10.1093/med/9780199572564.003.011
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date: 21 October 2019

Pathobiology of PAH 40

The cause of PAH remains unclear. Several mechanisms have been proposed, involving multiple biochemical pathways and cell types.

Endothelial dysfunction leads to an increase in PVR via several mechanisms:

Vasoconstriction due to chronically impaired production of vasodilator and antiproliferative agents such as nitric oxide (NO), vasoactive intestinal polypeptide (VIP), and prostacyclin, along with overexpression of vasoconstrictor and proliferative substances such as thromboxane A...

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