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

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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 1024 free, full-text research articles on human participants. First 3 results:

A giant right atrial myxoma with pulmonary arterial hypertension.
 

Author(s): Ihsen Zairi, Khadija Mzoughi, Zouhayer Jnifene, Sana Fennira, Fathia Ben Moussa, Sofiene Kammoun, Mohamed Mounir Dhiab, Sondos Kraiem

Journal:

 

Here we report a case of a right atrial mass that morphology mimicking myxoma, in a young patient with no past medical history. The mass was pathologically confirmed to be symptomatic and surgical removal was successfully done.

Last Updated: 30 Oct 2015

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Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL).
 

Author(s): Ioana R Preston, Kari E Roberts, Dave P Miller, Ginny P Sen, Mona Selej, Wade W Benton, Nicholas S Hill, Harrison W Farber

Journal: Circulation. 2015 Dec;132(25):2403-11.

 

Long-term anticoagulation is recommended in idiopathic pulmonary arterial hypertension (IPAH). In contrast, limited data support anticoagulation in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). We assessed the effect of warfarin anticoagulation ...

Last Updated: 24 Dec 2015

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A Biochemical Approach to Understand the Pathogenesis of Advanced Pulmonary Arterial Hypertension: Metabolomic Profiles of Arginine, Sphingosine-1-Phosphate, and Heme of Human Lung.
 

Author(s): Yidan D Zhao, Lei Chu, Kathleen Lin, Elise Granton, Li Yin, Jenny Peng, Michael Hsin, Licun Wu, Amy Yu, Thomas Waddell, Shaf Keshavjee, John Granton, Marc de Perrot

Journal:

 

Pulmonary arterial hypertension (PAH) is a vascular disease characterized by persistent precapillary pulmonary hypertension (PH), leading to progressive right heart failure and premature death. The pathological mechanisms underlying this condition remain elusive. Analysis of global ...

Last Updated: 31 Aug 2015

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

Epigenetic modulation as a therapeutic approach for pulmonary arterial hypertension.
 

Author(s): Jun-Dae Kim, Aram Lee, Jihea Choi, Youngsook Park, Hyesoo Kang, Woochul Chang, Myeong-Sok Lee, Jongmin Kim

Journal:

 

Pulmonary arterial hypertension (PAH) is a rare but progressive and currently incurable disease, which is characterized by vascular remodeling in association with muscularization of the arterioles, medial thickening and plexiform lesion formation. Despite our advanced understanding ...

Last Updated: 3 Aug 2015

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The molecular targets of approved treatments for pulmonary arterial hypertension.
 

Author(s): Marc Humbert, Hossein-Ardeschir Ghofrani

Journal: Thorax. 2016 Jan;71(1):73-83.

 

Until recently, three classes of medical therapy were available for the treatment of pulmonary arterial hypertension (PAH)--prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 (PDE5) inhibitors. With the approval of the soluble guanylate cyclase stimulator riociguat, ...

Last Updated: 18 Dec 2015

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Determinants of an elevated pulmonary arterial pressure in patients with pulmonary arterial hypertension.
 

Author(s): Seiichiro Sakao, Norbert F Voelkel, Nobuhiro Tanabe, Koichiro Tatsumi

Journal:

 

Given the difficulty of diagnosing early-stage pulmonary arterial hypertension (PAH) due to the lack of signs and symptoms, and the risk of an open lung biopsy, the precise pathological features of presymptomatic stage lung tissue remain unknown. It has been suggested that the maximum ...

Last Updated: 7 Jul 2015

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

 
 
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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 Bosentan
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension; Idiopathic Pulmonary Fibrosis

 

Last Updated: 19 Feb 2008

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