Pulmonary arterial hypertension

Common Name(s)

Pulmonary arterial hypertension, Idiopathic pulmonary hypertension, Primary pulmonary hypertension

Pulmonary arterial hypertension (PAH) is a progressive condition that affects the heart and lungs. It is characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. The most common signs and symptoms are shortness of breath (dyspnea) during exertion and fainting spells. As the condition worsens, people can experience dizziness, swelling (edema) of the ankles or legs, chest pain, and a racing pulse. Most cases of PAH occur in individuals with no family history of the disorder. Although some cases are due to mutations in the BMPR2 gene and inherited in an autosomal dominant pattern, a gene mutation has not yet been identified in most individuals. When PAH is inherited from an affected relative it is called "familial" PAH. Cases with no identifiable cause may be referred to as "idiopathic" PAH. PAH can also occur secondary to an underlying disorder such as connective tissue diseases, HIV infection, chronic hemolytic anemia, and congenital heart disease, to name a few. PAH can also be induced by certain drugs and toxins, for example fenfluramine and dexfenfluramine (appetite suppressants now banned by the FDA), toxic rapeseed oil, and amphetamines.  
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Pulmonary arterial hypertension" for support, advocacy or research.

International Scleroderma Network

Our mission is to provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses.

Last Updated: 18 Apr 2010

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PHCentral

Pulmonary Hypertension Information, Support and Advocacy Since 1999

Last Updated: 19 Dec 2012

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Pulmonary arterial hypertension" for support, advocacy or research.

International Scleroderma Network

Our mission is to provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses.

http://www.sclero.org/

Last Updated: 18 Apr 2010

View Details
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PHCentral

Pulmonary Hypertension Information, Support and Advocacy Since 1999

http://www.phcentral.org

Last Updated: 19 Dec 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Pulmonary arterial hypertension" returned 974 free, full-text research articles on human participants. First 3 results:

Riociguat for pulmonary arterial hypertension associated with congenital heart disease.
 

Author(s): Stephan Rosenkranz, Hossein-Ardeschir Ghofrani, Maurice Beghetti, Dunbar Ivy, Reiner Frey, Arno Fritsch, Gerrit Weimann, Soundos Saleh, Christian Apitz

Journal: Heart. 2015 Nov;101(22):1792-9.

 

The Pulmonary Arterial hyperTENsion sGC-stimulator Trial-1 (PATENT-1) was a randomised, double-blind, placebo-controlled phase III trial evaluating riociguat in patients with pulmonary arterial hypertension (PAH). PATENT-2 was an open-label long-term extension to PATENT-1. Here, we ...

Last Updated: 27 Oct 2015

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Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension.
 

Author(s): Lu Long, Mark L Ormiston, Xudong Yang, Mark Southwood, Stefan Gräf, Rajiv D Machado, Matthias Mueller, Bernd Kinzel, Lai Ming Yung, Janine M Wilkinson, Stephen D Moore, Kylie M Drake, Micheala A Aldred, Paul B Yu, Paul D Upton, Nicholas W Morrell

Journal: Nat. Med.. 2015 Jul;21(7):777-85.

 

Genetic evidence implicates the loss of bone morphogenetic protein type II receptor (BMPR-II) signaling in the endothelium as an initiating factor in pulmonary arterial hypertension (PAH). However, selective targeting of this signaling pathway using BMP ligands has not yet been explored ...

Last Updated: 8 Jul 2015

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Borderline pulmonary pressures in scleroderma - a 'pre-pulmonary arterial hypertension' condition?
 

Author(s): Gabor Kovacs, Horst Olschewski

Journal:

 

Patients with systemic sclerosis may develop borderline pulmonary arterial pressure. The clinical relevance of this condition is not always clear. Reported data support the evidence that this subgroup may represent an intermediate stage between normal pulmonary arterial pressure and ...

Last Updated: 18 May 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Pulmonary arterial hypertension" returned 240 free, full-text review articles on human participants. First 3 results:

Epigenetic modulation as a therapeutic approach for pulmonary arterial hypertension.
 

Author(s): Jun-Dae Kim, Aram Lee, Jihea Choi, Youngsook Park, Hyesoo Kang, Woochul Chang, Myeong-Sok Lee, Jongmin Kim

Journal:

 

Pulmonary arterial hypertension (PAH) is a rare but progressive and currently incurable disease, which is characterized by vascular remodeling in association with muscularization of the arterioles, medial thickening and plexiform lesion formation. Despite our advanced understanding ...

Last Updated: 3 Aug 2015

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Determinants of an elevated pulmonary arterial pressure in patients with pulmonary arterial hypertension.
 

Author(s): Seiichiro Sakao, Norbert F Voelkel, Nobuhiro Tanabe, Koichiro Tatsumi

Journal:

 

Given the difficulty of diagnosing early-stage pulmonary arterial hypertension (PAH) due to the lack of signs and symptoms, and the risk of an open lung biopsy, the precise pathological features of presymptomatic stage lung tissue remain unknown. It has been suggested that the maximum ...

Last Updated: 7 Jul 2015

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Sildenafil in pediatric pulmonary arterial hypertension.
 

Author(s): A K Dhariwal, S B Bavdekar

Journal: J Postgrad Med. ;61(3):181-92.

 

Pulmonary arterial hypertension (PAH) is a life-threatening disease of varied etiologies. Although PAH has no curative treatment, a greater understanding of pathophysiology, technological advances resulting in early diagnosis, and the availability of several newer drugs have improved ...

Last Updated: 29 Jun 2015

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Efficacy Study of Cardizem in Pulmonary Arterial Hypertension
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Arterial Hypertension; Primary Pulmonary Hypertension; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension

 

Last Updated: 6 Sep 2013

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Pharmacogenomics in Pulmonary Arterial Hypertension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension; Pulmonary Hypertension; PAH WHO Group I

 

Last Updated: 20 Jan 2012

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Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension; Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease; Pulmonary Hypertension

 

Last Updated: 19 Feb 2008

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