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Pulmonary Arterial Hypertension: Pathophysiology and Treatment

1
School of Medicine and Pharmacology, University of Western Australia, Perth 6009, Australia
2
Division of Molecular and Clinical Medicine, Mailbox 2, Ninewells Hospital and Medical School, University of Dundee, Dundee DD1 9SY, UK
*
Author to whom correspondence should be addressed.
Diseases 2018, 6(2), 38; https://doi.org/10.3390/diseases6020038
Received: 27 April 2018 / Revised: 12 May 2018 / Accepted: 12 May 2018 / Published: 16 May 2018
(This article belongs to the Section Cardiology)
Pulmonary arterial hypertension (PAH), the first category of pulmonary hypertension, is a chronic and progressive disorder characterised by angioproliferative vasculopathy in the pulmonary arterioles, leading to endothelial and smooth muscle proliferation and dysfunction, inflammation and thrombosis. These changes increase pulmonary vascular resistance and subsequent pulmonary arterial pressure, causing right ventricular failure which leads to eventual death if untreated. The management of PAH has advanced rapidly in recent years due to improved understanding of the condition’s pathophysiology, specifically the nitric oxide, prostacyclin-thromboxane and endothelin-1 pathways. Five classes of drugs targeting these pathways are now available: phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, prostacyclin receptor agonists and endothelin receptor antagonists. These developments have led to substantial improvements in mortality rate in recent decades. Recently, long-term studies have demonstrated sustained progression-free survival and have created a new paradigm of initial combination therapy. Despite these targeted therapies, PAH is still associated with significant morbidity and mortality. As such, further research into broadening our understanding of PAH pathophysiology is underway with potential of increasing the repertoire of drugs available. View Full-Text
Keywords: pulmonary arterial hypertension; nitric oxide; prostacyclin-thromboxane; endothelin-1; phosphodiesterase-5 inhibitor; soluble guanylate cyclase stimulators; prostacyclin analogues; prostacyclin receptor agonists; endothelin receptor antagonists; mortality pulmonary arterial hypertension; nitric oxide; prostacyclin-thromboxane; endothelin-1; phosphodiesterase-5 inhibitor; soluble guanylate cyclase stimulators; prostacyclin analogues; prostacyclin receptor agonists; endothelin receptor antagonists; mortality
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Lan, N.S.H.; Massam, B.D.; Kulkarni, S.S.; Lang, C.C. Pulmonary Arterial Hypertension: Pathophysiology and Treatment. Diseases 2018, 6, 38.

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