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 1282 free, full-text research articles on human participants. First 3 results:

Prostanoid EP₂ Receptors Are Up-Regulated in Human Pulmonary Arterial Hypertension: A Key Anti-Proliferative Target for Treprostinil in Smooth Muscle Cells.
 

Author(s): Jigisha A Patel, Lei Shen, Susan M Hall, Chabha Benyahia, Xavier Norel, Robin J McAnulty, Shahin Moledina, Adam M Silverstein, Brendan J Whittle, Lucie H Clapp

Journal:

 

Prostacyclins are extensively used to treat pulmonary arterial hypertension (PAH), a life-threatening disease involving the progressive thickening of small pulmonary arteries. Although these agents are considered to act therapeutically via the prostanoid IP receptor, treprostinil ...

Last Updated: 31 Dec 1969

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A case report of pulmonary arterial hypertension in pregnancy and complications of anticoagulation therapy.
 

Author(s): Yangfang Xiang, Jun Li, Yinxiang Sun

Journal: Medicine (Baltimore). 2018 Aug;97(32):e11810.

 

Treprostinil, a potent vasodilator, is the treatment of choice for severe pulmonary arterial hypertension (PAH) during pregnancy. Its inhibition of platelet aggregation increases the risk of hemorrhage. In addition, anticoagulation therapy is widely used in pregnancy with PAH due ...

Last Updated: 31 Dec 1969

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Factors Associated with Heritable Pulmonary Arterial Hypertension Exert Convergent Actions on the miR-130/301-Vascular Matrix Feedback Loop.
 

Author(s): Thomas Bertero, Adam L Handen, Stephen Y Chan

Journal:

 

Pulmonary arterial hypertension (PAH) is characterized by occlusion of lung arterioles, leading to marked increases in pulmonary vascular resistance. Although heritable forms of PAH are known to be driven by genetic mutations that share some commonality of function, the extent to ...

Last Updated: 31 Dec 1969

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

Endothelin-receptor antagonists in the management of pulmonary arterial hypertension: where do we stand?
 

Author(s): Michele Correale, Armando Ferraretti, Ilenia Monaco, Davide Grazioli, Matteo Di Biase, Natale Daniele Brunetti

Journal:

 

Pulmonary arterial hypertension, a disease largely neglected until a few decades ago, is presently the object of intense studies by several research teams. Despite considerable progress, pulmonary arterial hypertension remains a major clinical problem, because it is not always easy ...

Last Updated: 31 Dec 1969

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Survival benefits of oral anticoagulants in patients with pulmonary arterial hypertension: Protocol for a systematic review and meta-analysis.
 

Author(s): Hai-Chao Zhang, Na Wang, Wen Zhang, Zhi-Chun Gu, Xiao-Yan Liu

Journal: Medicine (Baltimore). 2018 Sep;97(36):e12203.

 

Pulmonary arterial hypertension (PAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for idiopathic pulmonary arterial hypertension (IPAH), the survival benefits are uncertain. Therefore, the present paper provides a protocol ...

Last Updated: 31 Dec 1969

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[Cardiopulmonary rehabilitation in pulmonary arterial hypertension].
 

Author(s): Astrid Von Oetinger, Luz María Trujillo, Sergio Villanueva, Mónica Zagolin

Journal: Rev Med Chil. 2018 May;146(5):627-635.

 

Cardiopulmonary rehabilitation is a promising therapy for Pulmonary arterial hypertension (PAH) whose survival does not exceed 65% at five years. We performed a literature search about rehabilitation on PAH in MEDLINE, LILACS and COCHRANE databases, considering articles from 2005 ...

Last Updated: 31 Dec 1969

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Symptoms, Diagnosis, and Treatment

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Physiopathology of Pulmonary Arterial Hypertension: Mechanistic Studies
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Arterial Hypertension

 

Last Updated: 8 Nov 2016

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Apabetalone for Pulmonary Arterial Hypertension: a Pilot Study
 

Status: Not yet recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 8 Nov 2018

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PARP-1 Inhibition in Pulmonary Arterial Hypertension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 8 Nov 2018

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