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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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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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 1327 free, full-text research articles on human participants. First 3 results:

Investigation of safety and efficacy of the new more thermostable formulation of Flolan (epoprostenol) in Japanese patients with pulmonary arterial hypertension (PAH)-An open-label, single-arm study.
 

Author(s): Kazuko Mihara, Aiko Ogawa, Hiromi Matsubara, Takumi Terao, Yoshitaka Ichikawa

Journal:

 

This study was conducted to evaluate the safety and efficacy of a new more thermostable Flolan (epoprostenol) solution prepared with the reformulated pH 12.0 diluent in Japanese patients with pulmonary arterial hypertension (PAH) receiving higher doses of Flolan than those typically ...

Last Updated: 31 Dec 1969

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Association between six-minute walk distance and long-term outcomes in patients with pulmonary arterial hypertension: Data from the randomized SERAPHIN trial.
 

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

Journal:

 

Patients with pulmonary arterial hypertension who achieve a six-minute walk distance of 380-440 m may have improved prognosis. Using the randomized controlled trial of macitentan in pulmonary arterial hypertension (SERAPHIN), the association between six-minute walk distance and long-term ...

Last Updated: 31 Dec 1969

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Hypoxemia in patients with idiopathic or heritable pulmonary arterial hypertension.
 

Author(s): Ghaleb Khirfan, Tawfeq Naal, Batool Abuhalimeh, Jennie Newman, Gustavo A Heresi, Raed A Dweik, Adriano R Tonelli

Journal:

 

The prevalence and prognostic implications of hypoxemia either at rest or during six-minute walk test (6MWT) in patients with idiopathic or heritable pulmonary arterial hypertension (IPAH or HPAH) have not been systemically studied.

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

The safety of endothelin receptor antagonists in the treatment of pulmonary arterial hypertension: Protocol for a systemic review and network meta-analysis.
 

Author(s): Zhi-Chun Gu, Yi-Jing Zhang, Mang-Mang Pan, Chi Zhang, Xiao-Yan Liu, An-Hua Wei, Ying-Jie Su

Journal: Medicine (Baltimore). 2018 Mar;97(11):e0122.

 

Pulmonary arterial hypertension (PAH) is a progressive disease and ultimately leads to right heart failure. Endothelin receptor antagonists (ERAs) have been demonstrated to significantly improve prognosis in PAH. However, ERAs-induced side effects can result in poor patient tolerance. ...

Last Updated: 31 Dec 1969

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The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease: A systematic review and meta-analysis.
 

Author(s): Hong-Yu Kuang, Yu-Hao Wu, Qi-Jian Yi, Jie Tian, Chun Wu, We Nian Shou, Tie-Wei Lu

Journal: Medicine (Baltimore). 2018 Mar;97(10):e0075.

 

Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute ...

Last Updated: 31 Dec 1969

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Exertional dyspnoea in pulmonary arterial hypertension.
 

Author(s): Daniel Dumitrescu, Olivier Sitbon, Jason Weatherald, Luke S Howard

Journal:

 

Dyspnoea is a principal presenting symptom in pulmonary arterial hypertension (PAH), and often the most distressing. The pathophysiology of PAH is relatively well understood, with the primary abnormality of pulmonary vascular disease resulting in a combination of impaired cardiac ...

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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REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 13 Jun 2018

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PARP-1 Inhibition in Pulmonary Arterial Hypertension
 

Status: Not yet recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 6 Mar 2018

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