2015
DOI: 10.1177/2040622315590757
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Medical treatment update on pulmonary arterial hypertension

Abstract: Pulmonary arterial hypertension is a chronic, progressive disease of the pulmonary vasculature resulting in poor outcomes if left untreated. The management of group 1 pulmonary arterial hypertension has included the use of prostanoids, phosphodiesterase-5 inhibitors, and endothelin receptor antagonists targeting the prostacyclin, endothelin-1, and nitric oxide pathways. Three new medications have been approved by the US Food and Drug Administration over the past couple of years. Macitentan is the newest endoth… Show more

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Cited by 23 publications
(23 citation statements)
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“…The medications target 3 major pathways: nitric oxide enhancement, endothelin-1 blockade, or prostacyclin analog or receptor agonist; therefore, the drugs may be used as monotherapy or in combination. 11 , 13 , 14 Indeed, increasing evidence seems to indicate improved outcomes with combination therapy, either sequentially or as first-line treatment. 3 , 6 , 7 , 15 18 …”
Section: Resultsmentioning
confidence: 99%
“…The medications target 3 major pathways: nitric oxide enhancement, endothelin-1 blockade, or prostacyclin analog or receptor agonist; therefore, the drugs may be used as monotherapy or in combination. 11 , 13 , 14 Indeed, increasing evidence seems to indicate improved outcomes with combination therapy, either sequentially or as first-line treatment. 3 , 6 , 7 , 15 18 …”
Section: Resultsmentioning
confidence: 99%
“…Management of group 1 PAH has included the use of prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors that target the prostacyclin receptor, endothelin-1 receptor, and nitric oxide signaling pathway. [1][2][3][4] During recent years, riociguat, a soluble guanylate cyclase stimulator, and selexipeg, a first-in-class, orally available, selective, non-prostanoid prostacyclin receptor (IP) agonist, have been used in the treatment of PAH. 5,6 The aforementioned pharmacotherapeutic agents have resulted in improved prognosis and quality of life but have also increased the complexity of decision making in treatment.…”
Section: Introductionmentioning
confidence: 99%
“…The standard treatment options include oral anticoagulants, diuretics, oxygen supplementation, and calcium channel blockers. Targeted treatments such as prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors, 4 , 5 which mainly address the increased vascular tone, lack the ability to reverse pulmonary vascular remodeling. As the impact of targeted therapies on the secondary forms of PH is uncertain, 6 , 7 , 8 the search for new therapeutic drugs is an urgent need.…”
Section: Introductionmentioning
confidence: 99%