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

[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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Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry.
 

Author(s): Pavel Jansa, Jiri Jarkovsky, Hikmet Al-Hiti, Jana Popelova, David Ambroz, Tomas Zatocil, Regina Votavova, Pavel Polacek, Jana Maresova, Michael Aschermann, Petr Brabec, Ladislav Dusek, Ales Linhart

Journal:

 

Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Epidemiological data from national registries are growing worldwide, but are still unavailable in Eastern Europe.

Last Updated: 21 Mar 2014

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First-in-man experience with cryoplasty during graded balloon atrial septostomy to reduce spontaneous closure in a patient with severe pulmonary arterial hypertension.
 

Author(s): Mayra Guerrero, Hector Cajigas, Rana Awdish, Adam Greenbaum, Akshay Khandelwal, Julio Sandoval

Journal: EuroIntervention. 2014 Feb;9(10):1235-6.

 

Last Updated: 24 Feb 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 200 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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Pathogenesis of pulmonary arterial hypertension: lessons from cancer.
 

Author(s): Christophe Guignabert, Ly Tu, Morane Le Hiress, Nicolas Ricard, Caroline Sattler, Andrei Seferian, Alice Huertas, Marc Humbert, David Montani

Journal: Eur Respir Rev. 2013 Dec;22(130):543-51.

 

Although the causal pathomechanisms contributing to remodelling of the pulmonary vascular bed in pulmonary arterial hypertension (PAH) are still unclear, several analogous features with carcinogenesis have led to the emergence of the cancer-like concept. The major similarities concern ...

Last Updated: 2 Dec 2013

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Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives.
 

Author(s): Loïc Guillevin, Iain Armstrong, Rino Aldrighetti, Luke S Howard, Henrik Ryftenius, Aryeh Fischer, Sandra Lombardi, Sean Studer, Pisana Ferrari

Journal: Eur Respir Rev. 2013 Dec;22(130):535-42.

 

Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH ...

Last Updated: 2 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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