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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General Support Organizations

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How do you compare to others with this condition?

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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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General Support Organizations

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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 1102 free, full-text research articles on human participants. First 3 results:

Undiagnosed connective tissue diseases: High prevalence in pulmonary arterial hypertension patients.
 

Author(s): Lorenzo Cavagna, Veronica Codullo, Stefano Ghio, Carlo Alberto Scirè, Eleonora Guzzafame, Laura Scelsi, Silvia Rossi, Carlomaurizio Montecucco, Roberto Caporali

Journal: Medicine (Baltimore). 2016 Sep;95(39):e4827.

 

Among different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) have distinct hemodynamic and prognostic features; a correct etiologic diagnosis is thus mandatory.To estimate frequency and prognosis of previously undiagnosed ...

Last Updated: 30 Sep 2016

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New echocardiographic indexes for evaluating cardiac function in idiopathic pulmonary arterial hypertension using three-dimensional echocardiography.
 

Author(s): H Yao, D Zhou, D Kong, C Pan, L Dong, Y Wang, X Shu

Journal: Acta Cardiol. 2016 Jun;71(3):359-66.

 

Last Updated: 5 Sep 2016

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[The assessment of right ventricular function in patients with pulmonary arterial hypertension with cardiac magnetic resonance imaging: a Meta-analysis].
 

Author(s): Cui Qian, Ge Xihong, Yu Jing, Gao Guangfeng, Shen Wen

Journal: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue. 2015 Dec;27(12):998-1001.

 

To explore the clinical value of cardiac magnetic resonance imaging (CMRI) in assessment of right ventricular function in patients with pulmonary arterial hypertension (PAH).

Last Updated: 27 Jun 2016

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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 272 free, full-text review articles on human participants. First 3 results:

Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature.
 

Author(s): Sébastien Sanges, Cécile M Yelnik, Olivier Sitbon, Olivier Benveniste, Kuberaka Mariampillai, Mathilde Phillips-Houlbracq, Christophe Pison, Christophe Deligny, Jocelyn Inamo, Vincent Cottin, Luc Mouthon, David Launay, Marc Lambert, Pierre-Yves Hatron, Laurence Rottat, Marc Humbert, Eric Hachulla

Journal: Medicine (Baltimore). 2016 Sep;95(39):e4911.

 

Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French ...

Last Updated: 30 Sep 2016

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Diffuse Pulmonary Arteriovenous Fistulas With Pulmonary Arterial Hypertension: Case Report and Review.
 

Author(s): Rong Jiang, Su-Gang Gong, Bigyan Pudasaini, Qin-Hua Zhao, Lan Wang, Jing He, Jin-Ming Liu

Journal: Medicine (Baltimore). 2016 Apr;95(14):e3177.

 

Pulmonary arteriovenous fistulas (PAVFs) are rare. Diffuse type PAVFs with pulmonary arterial hypertension (PAH) are even rarer and can elude anatomy imaging like a plain chest film or a computed tomography. The rapid blood flow that ensues due to lack of a capillary bed leads to ...

Last Updated: 9 Apr 2016

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New Therapeutic Paradigms and Guidelines in the Management of Pulmonary Arterial Hypertension.
 

Author(s): Nicholas S Hill, Michael J Cawley, Cherilyn L Heggen-Peay

Journal: J Manag Care Spec Pharm. 2016 Mar;22(3 Suppl A):S3-21.

 

Recent and ongoing developments in the diagnosis, treatment, and management of pulmonary arterial hypertension (PAH) provide deeper insights into pathogenic mechanisms. Approvals of new pharmacotherapies that improve function and reduce morbidity and mortality risks; advances in clinical ...

Last Updated: 23 Mar 2016

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Clinical Trial Information This information is provided by ClinicalTrials.gov

REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 16 Jan 2017

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Physiopathology of Pulmonary Arterial Hypertension: Mechanistic Studies
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Arterial Hypertension

 

Last Updated: 8 Nov 2016

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Aerobic Exercise Training in Pulmonary Arterial Hypertension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 13 May 2015

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