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

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 1117 free, full-text research articles on human participants. First 3 results:

Implication of overexpression of dishevelled-associated activator of morphogenesis 1 (Daam-1) for the pathogenesis of human Idiopathic Pulmonary Arterial Hypertension (IPAH).
 

Author(s): Shun Yanai, Megumi Wakayama, Haruo Nakayama, Minoru Shinozaki, Hisayuki Tsukuma, Naobumi Tochigi, Tetsuo Nemoto, Tsutomu Saji, Kazutoshi Shibuya

Journal:

 

Idiopathic pulmonary arterial hypertension (IPAH) is a rare, fatal disease of unknown pathogenesis. Evidence from our recent study suggests that IPAH pathogenesis is related to upregulation of the Wnt/planar cell polarity (Wnt/PCP) pathway. We used microscopic observation and immunohistochemical ...

Last Updated: 14 Mar 2017

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The Correlation of Decreased Heart Rate Recovery and Chronotropic Incompetence with Exercise Capacity in Idiopathic Pulmonary Arterial Hypertension Patients.
 

Author(s): Changwei Wu, Jian Guo, Hui Liu, Bigyan Pudasaini, Wenlan Yang, Qinhua Zhao, Lan Wang, Jinming Liu

Journal: Biomed Res Int. 2017 ;2017():3415401.

 

We show by this study that a decrease in HRR1 in IPAH patients is associated with severe limitation of exercise capacity. HRR1 < 16 beats and CI just after completion of a CPET could be an indicator of poor prognosis.

Last Updated: 13 Mar 2017

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Reverse Right Ventricular Remodeling After Lung Transplantation in Patients With Pulmonary Arterial Hypertension Under Combination Therapy of Targeted Medical Drugs.
 

Author(s): Toshihiro Sarashina, Kazufumi Nakamura, Satoshi Akagi, Takahiro Oto, Hiroki Oe, Kentaro Ejiri, Koji Nakagawa, Nobuhiro Nishii, Hiromi Matsubara, Motomu Kobayashi, Hiroshi Morimatsu, Shinichiro Miyoshi, Hiroshi Ito

Journal: Circ. J.. 2017 Feb;81(3):383-390.

 

Patients with pulmonary arterial hypertension (PAH) are currently treated with combination therapy of PAH-targeted drugs. Reverse right ventricular (RV) remodeling after lung transplantation (LTx) in patients with end-stage PAH despite combination therapy of PAH-targeted drugs has ...

Last Updated: 19 Jan 2017

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

Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature.
 

Author(s): Sébastien Sanges, Cécile M Yelnik, Olivier Sitbon, Olivier Benveniste, Kuberaka Mariampillai, Mathilde Phillips-Houlbracq, Christophe Pison, Christophe Deligny, Jocelyn Inamo, Vincent Cottin, Luc Mouthon, David Launay, Marc Lambert, Pierre-Yves Hatron, Laurence Rottat, Marc Humbert, Eric Hachulla

Journal: Medicine (Baltimore). 2016 Sep;95(39):e4911.

 

Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French ...

Last Updated: 30 Sep 2016

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Comparative Effectiveness of Oral Medications for Pulmonary Arterial Hypertension.
 

Author(s): Ataru Igarashi, Sachie Inoue, Tomonori Ishii, Kiichiro Tsutani, Hiroshi Watanabe

Journal: Int Heart J. 2016 Jul;57(4):466-72.

 

Pulmonary arterial hypertension (PAH) is a disease that imposes a significant burden on patients. Although multiple treatment options for PAH are available, head-to-head comparisons are difficult to conduct. Network meta-analysis (NMA) can be a useful alternative for direct comparison ...

Last Updated: 28 Jul 2016

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Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension.
 

Author(s): Jane A Leopold, Bradley A Maron

Journal:

 

Pulmonary arterial hypertension (PAH) is a devastating disease that is precipitated by hypertrophic pulmonary vascular remodeling of distal arterioles to increase pulmonary artery pressure and pulmonary vascular resistance in the absence of left heart, lung parenchymal, or thromboembolic ...

Last Updated: 24 May 2016

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Clinical Trial Information This information is provided by ClinicalTrials.gov

REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 16 Jan 2017

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Physiopathology of Pulmonary Arterial Hypertension: Mechanistic Studies
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Arterial Hypertension

 

Last Updated: 8 Nov 2016

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Aerobic Exercise Training in Pulmonary Arterial Hypertension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 13 May 2015

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