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

Potential hemodynamic biomarkers of pulmonary arterial hypertension measured with an aid of three-dimensional cine phase contrast MR imaging.
 

Author(s): Masaki Terada, Yasuo Takehara, Haruo Isoda, Takashi Kosugi, Marcus Alley

Journal: Nihon Hoshasen Gijutsu Gakkai Zasshi. 2014 Oct;70(10):1198-200.

 

Assessment of pulmonary arterial hypertension (PAH) is drawing more and more attention from respiratory and cardiovascular clinicians. Pulmonary artery flow velocities, flow volume and their derived parameters, such as acceleration time (AT), acceleration volumes (AV) and maximum ...

Last Updated: 20 Oct 2014

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[Efficacy and safety of sildenafil in the treatment of high altitude heart disease associated with severe pulmonary arterial hypertension in children: a preliminary evaluation].
 

Author(s): Yan-Liang Xia, Wei-Xiao Yan, Hong Chen

Journal: Zhongguo Dang Dai Er Ke Za Zhi. 2014 Jul;16(7):745-8.

 

To observe the clinical efficacy and safety of sildenafil in the treatment of high altitude heart disease associated with severe pulmonary arterial hypertension (PAH) in children.

Last Updated: 10 Jul 2014

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Pulmonary function differences in patients with chronic right heart failure secondary to pulmonary arterial hypertension and chronic left heart failure.
 

Author(s): Wei-Hua Liu, Qin Luo, Zhi-Hong Liu, Qing Zhao, Qun-Ying Xi, Hai-Feng Xue, Zhi-Hui Zhao

Journal:

 

Pulmonary abnormalities are found in both chronic heart failure (CHF) and pulmonary arterial hypertension (PAH). The differences of pulmonary function in chronic left heart failure and chronic right heart failure are not fully understood.

Last Updated: 11 Jun 2014

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

Pathological function of Ca2+-sensing receptor in pulmonary arterial hypertension.
 

Author(s): Aya Yamamura

Journal: J Smooth Muscle Res. 2014 ;50():8-17.

 

Pulmonary arterial hypertension (PAH) is defined as an intractable disease characterized by a progressive elevation of pulmonary vascular resistance (PVR) and pulmonary arterial pressure (PAP), leading to right heart failure and premature death. The five-year survival rate after diagnosis ...

Last Updated: 28 Apr 2014

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Apelin-APJ signaling: a potential therapeutic target for pulmonary arterial hypertension.
 

Author(s): Jongmin Kim

Journal: Mol. Cells. 2014 Mar;37(3):196-201.

 

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by the vascular remodeling of the pulmonary arterioles, including formation of plexiform and concentric lesions comprised of proliferative vascular cells. Clinically, PAH leads to increased pulmonary arterial ...

Last Updated: 31 Mar 2014

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Meta-analysis of randomized controlled trials of bosentan for treatment of pulmonary arterial hypertension.
 

Author(s): Young Ho Lee, Gwan Gyu Song

Journal: Korean J. Intern. Med.. 2013 Nov;28(6):701-7.

 

We assessed the efficacy and safety of bosentan in patients with pulmonary arterial hypertension (PAH).

Last Updated: 5 Dec 2013

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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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