Next Article in Journal
Heat Shock Gene Inactivation and Protein Aggregation with Links to Chronic Diseases
Previous Article in Journal
Zika Virus and the Risk for Renter Households
Article Menu
Issue 2 (June) cover image

Export Article

Open AccessFeature PaperReview
Diseases 2018, 6(2), 38; https://doi.org/10.3390/diseases6020038

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.
Received: 27 April 2018 / Revised: 12 May 2018 / Accepted: 12 May 2018 / Published: 16 May 2018
(This article belongs to the Section Cardiology)
Full-Text   |   PDF [1280 KB, uploaded 16 May 2018]   |  

Abstract

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
Figures

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).
SciFeed

Share & Cite This Article

MDPI and ACS Style

Lan, N.S.H.; Massam, B.D.; Kulkarni, S.S.; Lang, C.C. Pulmonary Arterial Hypertension: Pathophysiology and Treatment. Diseases 2018, 6, 38.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
Diseases EISSN 2079-9721 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top