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.

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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General Support Organizations

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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

View Details

 

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

Macitentan and pulmonary arterial hypertension.
 

Author(s): Tomás Pulido, Lewis J Rubin, Gérald Simonneau

Journal: N. Engl. J. Med.. 2014 Jan;370(1):82-3.

 

Last Updated: 2 Jan 2014

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A novel channelopathy in pulmonary arterial hypertension.
 

Author(s): Wendy K Chung, Kevin S Sampson, Robert S Kass

Journal: N. Engl. J. Med.. 2013 Nov;369(22):2162.

 

Last Updated: 28 Nov 2013

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Macitentan and morbidity and mortality in pulmonary arterial hypertension.
 

Author(s): Tomás Pulido, Igor Adzerikho, Richard N Channick, Marion Delcroix, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Pavel Jansa, Zhi-Cheng Jing, Franck-Olivier Le Brun, Sanjay Mehta, Camilla M Mittelholzer, Loïc Perchenet, B K S Sastry, Olivier Sitbon, Rogério Souza, Adam Torbicki, Xiaofeng Zeng, Lewis J Rubin, Gérald Simonneau,

Journal: N. Engl. J. Med.. 2013 Aug;369(9):809-18.

 

Current therapies for pulmonary arterial hypertension have been adopted on the basis of short-term trials with exercise capacity as the primary end point. We assessed the efficacy of macitentan, a new dual endothelin-receptor antagonist, using a primary end point of morbidity and ...

Last Updated: 29 Aug 2013

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

Drug-induced pulmonary arterial hypertension: a recent outbreak.
 

Author(s): David Montani, Andrei Seferian, Laurent Savale, Gérald Simonneau, Marc Humbert

Journal: Eur Respir Rev. 2013 Sep;22(129):244-50.

 

Pulmonary arterial hypertension (PAH) is a rare disorder characterised by progressive obliteration of the pulmonary microvasculature resulting in elevated pulmonary vascular resistance and premature death. According to the current classification PAH can be associated with exposure ...

Last Updated: 2 Sep 2013

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Therapies for pulmonary arterial hypertension: where are we today, where do we go tomorrow?
 

Author(s): Andrei Seferian, Gérald Simonneau

Journal: Eur Respir Rev. 2013 Sep;22(129):217-26.

 

Pulmonary arterial hypertension (PAH) is a progressive disease characterised by remodelling of small pulmonary arteries leading to an increased pulmonary vascular resistance, right ventricular failure and death. Available treatments try to re-establish the equilibrium on three signalling ...

Last Updated: 2 Sep 2013

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A review of ongoing trials in exercise based rehabilitation for pulmonary arterial hypertension.
 

Author(s): Abraham Samuel Babu, Ramachandran Padmakumar, Arun G Maiya

Journal: Indian J. Med. Res.. 2013 May;137(5):900-6.

 

Exercise based rehabilitation for patients with pulmonary arterial hypertension (PAH) is a new treatment option for these patients to improve their functional capacity and quality of life. Despite the benefits seen in cardiopulmonary rehabilitation in various other conditions, it ...

Last Updated: 13 Jun 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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Hormonal, Metabolic, and Signaling Interactions in PAH
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Arterial Hypertension; Heritable Pulmonary Arterial Hypertension; Scleroderma Associated Pulmonary Arterial Hypertension; Appetite Suppressant Associate PAH

 

Last Updated: 3 Apr 2014

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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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