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

Pulmonary arterial hypertension associated with tetralogy of Fallot.
 

Author(s): Jun Yasuhara, Hiroyuki Yamagishi

Journal: Int Heart J. 2015 ;56 Suppl():S17-21.

 

Pulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD). Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and ...

Last Updated: 19 Mar 2015

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Ankylosing spondylitis associated with pulmonary arterial hypertension.
 

Author(s): Yao-Min Hung, Chin-Chang Cheng, Shue-Ren Wann, Shoa-Lin Lin

Journal: Intern. Med.. 2015 ;54(4):431-4.

 

Pulmonary arterial hypertension (PAH) is a frequent complication of connective tissue diseases, such as systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. However, the occurrence of PAH in a patient with ankylosing spondylitis (AS) has not been previously reported ...

Last Updated: 9 Mar 2015

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Rescue balloon pulmonary angioplasty under veno-arterial extracorporeal membrane oxygenation in a patient with acute exacerbation of chronic thromboembolic pulmonary hypertension.
 

Author(s): Makiko Nakamura, Osahiko Sunagawa, Hiroyuki Tsuchiya, Takafumi Miyara, Yoji Taba, Takashi Touma, Hiroshi Munakata, Tadao Kugai, Yutaka Okita

Journal: Int Heart J. 2015 ;56(1):116-20.

 

We describe a case of a 41-year-old woman with acute exacerbation of chronic thromboembolic pulmonary hypertension (CTEPH) complicated by rapidly progressive respiratory failure and right heart failure with cardiogenic shock. A computed tomography (CT) showed thrombi in the right ...

Last Updated: 6 Mar 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 212 free, full-text review articles on human participants. First 3 results:

When to offer genetic testing for pulmonary arterial hypertension.
 

Author(s): Wendy K Chung, Eric D Austin, D Hunter Best, Lynette M Brown, C Gregory Elliott

Journal: Can J Cardiol. 2015 Apr;31(4):544-7.

 

Genetic testing is poised to play a greater role in the diagnosis and management of pulmonary arterial hypertension (PAH). Physicians who manage PAH should know the heritable PAH phenotypes, inheritance patterns, and responsible genes. They also should know indications, potential ...

Last Updated: 4 Apr 2015

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Advanced imaging tools rather than hemodynamics should be the primary approach for diagnosing, following, and managing pulmonary arterial hypertension.
 

Author(s): Mario Gerges, Christian Gerges, Irene M Lang

Journal: Can J Cardiol. 2015 Apr;31(4):521-8.

 

Pulmonary hypertension (PH) is currently defined based on invasive measurements: a resting pulmonary artery pressure ≥ 25 mm Hg. For pulmonary arterial hypertension, a pulmonary arterial wedge pressure ≤ 15 mm Hg and pulmonary vascular resistance > 3 Wood units are also required. ...

Last Updated: 4 Apr 2015

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Pulmonary arterial hypertension associated with bronchopulmonary dysplasia and congenital heart disease in preterm infants. A case report of a preterm infant with recurrent pulmonary hypertension after corrective cardiac surgery and review of the literature.
 

Author(s): Jun Muneuchi, Ayako Kuraoka, Mamie Watanabe, Yoshie Ochiai, Kunitaka Joo

Journal: Int Heart J. 2015 ;56 Suppl():S22-5.

 

In preterm infants with congenital heart disease, concomitant bronchopulmonary dysplasia (BPD) is associated with relatively poor clinical outcomes because of the increased pulmonary vascular resistance and adverse effects of inflammation on the damaged lungs, even after surgery. ...

Last Updated: 19 Mar 2015

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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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