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

View Details
Logo
PHCentral

Pulmonary Hypertension Information, Support and Advocacy Since 1999

Last Updated: 19 Dec 2012

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

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
Logo
PHCentral

Pulmonary Hypertension Information, Support and Advocacy Since 1999

http://www.phcentral.org

Last Updated: 19 Dec 2012

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

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 1148 free, full-text research articles on human participants. First 3 results:

Serotonin Signaling Through the 5-HT1B Receptor and NADPH Oxidase 1 in Pulmonary Arterial Hypertension.
 

Author(s): Katie Y Hood, Kirsty M Mair, Adam P Harvey, Augusto C Montezano, Rhian M Touyz, Margaret R MacLean

Journal: Arterioscler. Thromb. Vasc. Biol.. 2017 Jul;37(7):1361-1370.

 

Serotonin can induce human pulmonary artery smooth muscle cell (hPASMC) proliferation through reactive oxygen species (ROS), influencing the development of pulmonary arterial hypertension (PAH). We hypothesize that in PASMCs, serotonin induces oxidative stress through NADPH-oxidase-derived ...

Last Updated: 5 May 2017

Go To URL
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

Go To URL
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

Go To URL

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

Survival in Idiopathic Pulmonary Fibrosis: Perspectives from Pulmonary Arterial Hypertension.
 

Author(s): Toby M Maher, Fred Dejonckheere, Steven D Nathan

Journal: J Manag Care Spec Pharm. 2017 Mar;23(3-b Suppl):S3-S4.

 

Maher has received grants, consulting fees, and speaker fees from GlaxoSmithKline and UCB and grants from Novartis. He has also received consulting fees and speaker fees from AstraZeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Cipla, Lanthio, InterMune International AG (a wholly ...

Last Updated: 13 Mar 2017

Go To URL
Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience.
 

Author(s): Olivier Sitbon, Anton Vonk Noordegraaf

Journal:

 

Epoprostenol was the first therapy to be approved for the treatment of pulmonary arterial hypertension (PAH). In the 20 years since the introduction of this prostacyclin analogue, the outlook for patients with PAH has improved, with survival rates now double those from the era before ...

Last Updated: 18 Jan 2017

Go To URL
A Review of Clinical Trial Endpoints of Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension and How They Relate to Patient Outcomes in the United States.
 

Author(s): Christine Divers, David Platt, Edward Wang, Jay Lin, Melissa Lingohr-Smith, Stephen C Mathai

Journal: J Manag Care Spec Pharm. 2017 Jan;23(1):92-104.

 

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are subgroups of pulmonary hypertension and are considered rare diseases. Understanding how endpoints of clinical trials (and patient registry studies) of patients with PAH and CTEPH are ...

Last Updated: 27 Dec 2016

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

 
 
Top

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: 19 May 2017

Go to URL
Physiopathology of Pulmonary Arterial Hypertension: Mechanistic Studies
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Arterial Hypertension

 

Last Updated: 8 Nov 2016

Go to URL
Aerobic Exercise Training in Pulmonary Arterial Hypertension
 

Status: Recruiting

Condition Summary: Pulmonary Arterial Hypertension

 

Last Updated: 3 Jul 2017

Go to URL