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

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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 1117 free, full-text research articles on human participants. First 3 results:

[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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Case 1/2016--56-Year-Old Male with Atrial Septal Defect, Pulmonary Arterial Hypertension, Hospitalized Due to Eisenmenger Syndrome.
 

Author(s): Carolina Santana, Antonio Augusto B Lopes, Antonio Fernando Lins de Paiva, Luiz Alberto Benvenuti

Journal: Arq. Bras. Cardiol.. 2016 Mar;106(3):250-8.

 

Last Updated: 31 Mar 2016

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CXCL13 in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
 

Author(s): Karen M Olsson, Sandra Olle, Jan Fuge, Tobias Welte, Marius M Hoeper, Christian Lerch, Lavinia Maegel, Hermann Haller, Danny Jonigk, Lena Schiffer

Journal:

 

Chemokine CXC ligand 13 (CXCL13) has been implicated in perivascular inflammation and pulmonary vascular remodeling in patients with idiopathic pulmonary artery hypertension (IPAH). We wondered whether CXCL13 may also play a role in chronic thromboembolic pulmonary hypertension (CTEPH) ...

Last Updated: 1 Mar 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 277 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

There are currently no related results available in Genetics Home Reference.

 
 
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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: 6 Jul 2016

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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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