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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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

View Details

 

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

Two formulations of epoprostenol sodium in the treatment of pulmonary arterial hypertension: EPITOME-1 (epoprostenol for injection in pulmonary arterial hypertension), a phase IV, open-label, randomized study.
 

Author(s): Kelly M Chin, David B Badesch, Ivan M Robbins, Victor F Tapson, Harold I Palevsky, Nick H Kim, Steven M Kawut, Adaani Frost, Wade W Benton, Jean-Christophe Lemarie, Frederic Bodin, Lewis J Rubin, Vallerie McLaughlin

Journal: Am. Heart J.. 2014 Feb;167(2):218-225.e1.

 

Epoprostenol sodium with arginine-mannitol excipients (epoprostenol AM; Veletri [Actelion Pharmaceuticals Ltd, Allschwil, Switzerland]) and epoprostenol sodium with glycine-mannitol excipients (epoprostenol GM; Flolan [GlaxoSmithKline, Triangle Park, NC]) are intravenous treatments ...

Last Updated: 20 Jan 2014

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EPITOME-2: An open-label study assessing the transition to a new formulation of intravenous epoprostenol in patients with pulmonary arterial hypertension.
 

Author(s): Olivier Sitbon, Marion Delcroix, Emmanuel Bergot, Anco B Boonstra, John Granton, David Langleben, Pilar Escribano Subías, Nazzareno Galiè, Thomas Pfister, Jean-Christophe Lemarié, Gérald Simonneau

Journal: Am. Heart J.. 2014 Feb;167(2):210-7.

 

Continuous infusion of epoprostenol is the treatment of choice in patients with pulmonary arterial hypertension in functional classes III to IV. However, this treatment's limitations include instability at room temperature. A new epoprostenol formulation offers improved storage conditions ...

Last Updated: 20 Jan 2014

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

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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Update on pediatric pulmonary arterial hypertension. Differences and similarities to adult disease.
 

Author(s): Tsutomu Saji

Journal: Circ. J.. 2013 ;77(11):2639-50.

 

Children and adults with pulmonary arterial hypertension (PAH) have similarities and differences in their background characteristics, hemodynamics, and clinical manifestations. Regarding genetic background, mutations in BMPR2-related pathways seem to be pivotal; however, it is likely ...

Last Updated: 28 Oct 2013

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"Nocturnal seizures" in idiopathic pulmonary arterial hypertension.
 

Author(s): Anthony Izzo, Julia McSweeney, Thomas Kulik, Umakanth Khatwa, Sanjeev V Kothare

Journal:

 

The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to ...

Last Updated: 15 Oct 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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