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Review

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

, &
Pages 919-931 | Published online: 10 Jan 2014

Figures & data

Figure 1. The prostacyclin, nitric oxide and endothelin pathways.

Prostacyclin (PGI2) is synthesized from arachidonic acid by COX-1 and PGI2 synthase. PGI2 activates the receptor of smooth muscle cells and in turn activates adenylate cyclase. This leads to increased production of cAMP from ATP with the effect of vasodilation and antiproliferation. Endothelial nitric oxide (NO) synthase produces NO from l-arginine. NO stimulates soluble guanylate cyclase resulting in increased production of cyclic guanylate monophosphate and thus antiproliferation and vasodilation. Preproendothelin is converted to big-endothelin (ET)-1 and subsequently ET-1 by furin-like enzyme and ET-converting enzymes, respectively. ET-1 activates ET-A and ET-B receptors, leading to calcium release and activation of protein kinase C, which leads to vasoconstriction and proliferation of smooth muscle cells.

Figure 1. The prostacyclin, nitric oxide and endothelin pathways.Prostacyclin (PGI2) is synthesized from arachidonic acid by COX-1 and PGI2 synthase. PGI2 activates the receptor of smooth muscle cells and in turn activates adenylate cyclase. This leads to increased production of cAMP from ATP with the effect of vasodilation and antiproliferation. Endothelial nitric oxide (NO) synthase produces NO from l-arginine. NO stimulates soluble guanylate cyclase resulting in increased production of cyclic guanylate monophosphate and thus antiproliferation and vasodilation. Preproendothelin is converted to big-endothelin (ET)-1 and subsequently ET-1 by furin-like enzyme and ET-converting enzymes, respectively. ET-1 activates ET-A and ET-B receptors, leading to calcium release and activation of protein kinase C, which leads to vasoconstriction and proliferation of smooth muscle cells.

Table 1. Currently available studies assessing disease targeting therapies in pulmonary arterial hypertension associated with congenital heart disease and selected studies of mixed pulmonary arterial hypertension populations.

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