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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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

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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 1087 free, full-text research articles on human participants. First 3 results:

Therapeutic Benefits of Induced Pluripotent Stem Cells in Monocrotaline-Induced Pulmonary Arterial Hypertension.
 

Author(s): Wei-Chun Huang, Meng-Wei Ke, Chin-Chang Cheng, Shih-Hwa Chiou, Shue-Ren Wann, Chih-Wen Shu, Kuan-Rau Chiou, Ching-Jiunn Tseng, Hung-Wei Pan, Guang-Yuan Mar, Chun-Peng Liu

Journal:

 

Pulmonary arterial hypertension (PAH) is characterized by progressive increases in vascular resistance and the remodeling of pulmonary arteries. The accumulation of inflammatory cells in the lung and elevated levels of inflammatory cytokines in the bloodstream suggest that inflammation ...

Last Updated: 4 Feb 2016

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Endothelin-1 Predicts Hemodynamically Assessed Pulmonary Arterial Hypertension in HIV Infection.
 

Author(s): Rushi V Parikh, Yifei Ma, Rebecca Scherzer, Amanda S Heringer, John S Macgregor, Jeffrey N Martin, Steven G Deeks, Peter Ganz, Priscilla Y Hsue

Journal:

 

HIV infection is an independent risk factor for PAH, but the underlying pathogenesis remains unclear. ET-1 is a robust vasoconstrictor and key mediator of pulmonary vascular homeostasis. Higher levels of ET-1 predict disease severity and mortality in other forms of PAH, and endothelin ...

Last Updated: 12 Jan 2016

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Quantitative Right Ventricular Function in Pulmonary Arterial Hypertension: A Quest for a More Reliable Metric.
 

Author(s): Aiden Abidov, Franz Rischard

Journal: Echocardiography. 2016 Feb;33(2):174-6.

 

Last Updated: 28 Jan 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:

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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Comparative Efficacy and Safety of Prostacyclin Analogs for Pulmonary Arterial Hypertension: A Network Meta-Analysis.
 

Author(s): Huijun Zhang, Xiaobing Li, Jiancheng Huang, Hongying Li, Zhenyu Su, Jun Wang

Journal: Medicine (Baltimore). 2016 Jan;95(4):e2575.

 

Prostacyclin analogs, such as epoprostenol, treprostinil, iloprost, and beraprost, have long been used for pulmonary arterial hypertension (PAH) treatment, yet their relative efficiency remains disputed. Eligible randomized controlled trials (RCTs) involving the 4 therapies mentioned ...

Last Updated: 30 Jan 2016

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Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension.
 

Author(s): Irene M Lang, Sean P Gaine

Journal: Eur Respir Rev. 2015 Dec;24(138):630-41.

 

Pulmonary arterial hypertension (PAH) is a severe disease characterised by increased pulmonary vascular resistance, which leads to restricted pulmonary arterial blood flow and elevated pulmonary arterial pressure. In patients with PAH, pulmonary concentrations of prostacyclin, a prostanoid ...

Last Updated: 1 Dec 2015

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Symptoms, Diagnosis, and Treatment

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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 Bosentan
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension; Idiopathic Pulmonary Fibrosis

 

Last Updated: 19 Feb 2008

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