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 1204 free, full-text research articles on human participants. First 3 results:

Decreased haptoglobin levels inversely correlated with pulmonary artery pressure in patients with pulmonary arterial hypertension: A cross-sectional study.
 

Author(s): Hiroyuki Nakamura, Masaru Kato, Toshitaka Nakaya, Michihiro Kono, Shun Tanimura, Takahiro Sato, Yuichiro Fujieda, Kenji Oku, Hiroshi Ohira, Toshiyuki Bohgaki, Shinsuke Yasuda, Ichizo Tsujino, Masaharu Nishimura, Tatsuya Atsumi

Journal: Medicine (Baltimore). 2017 Oct;96(43):e8349.

 

We investigated the serum haptoglobin levels in patients with pulmonary arterial hypertension (PAH) based on the hypothesis that haptoglobin levels would reflect subclinical hemolysis due to microangiopathy in pulmonary arterioles.This cross-sectional study included 3 groups of patients ...

Last Updated: 25 Oct 2017

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Prognostic Value of Pulmonary Artery Compliance in Patients with Pulmonary Arterial Hypertension Associated with Adult Congenital Heart Disease.
 

Author(s): Xiao-Ling Cheng, Zhi-Hong Liu, Qing Gu, Xin-Hai Ni, Qin Luo, Zhi-Hui Zhao, Jian-Guo He, Chang-Ming Xiong

Journal: Int Heart J. 2017 Oct;58(5):731-738.

 

In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart ...

Last Updated: 2 Oct 2017

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The impact and financial burden of pulmonary arterial hypertension on patients and caregivers: results from a national survey.
 

Author(s): Zhenguo Zhai, Xia Zhou, Shuai Zhang, Wanmu Xie, Jun Wan, Tuguang Kuang, Yuanhua Yang, Huan Huang, Chen Wang

Journal: Medicine (Baltimore). 2017 Sep;96(39):e6783.

 

Pulmonary arterial hypertension (PAH) is a chronic progressive devastating disease. Symptom burden might impair health-related quality of life of patients. Furthermore, treatment on this disease brings significant financial burden to patients' families. Both physiological and psychological ...

Last Updated: 27 Sep 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 298 free, full-text review articles on human participants. First 3 results:

Novel strategies for treatment of pulmonary arterial hypertension.
 

Author(s): Magdalena Jasińska-Stroschein, Daria Orszulak-Michalak

Journal:

 

Pulmonary hypertension (PH) is a rare disorder associated with abnormally elevated pulmonary pressures that, if untreated, leads to right heart failure and premature death. Special population include patents with pulmonary arterial hypertension (PAH). A greater understanding of the ...

Last Updated: 9 Aug 2017

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Prognostic factors in pulmonary arterial hypertension: Literature review.
 

Author(s): Emilia Stępnowska, Ewa Lewicka, Alicja Dąbrowska-Kugacka, Paweł Miękus, Grzegorz Raczak

Journal: Adv Clin Exp Med. ;26(3):549-553.

 

Pulmonary arterial hypertension is a disease that has a bad influence on the patient's prognosis. Recently, the possibility of therapy has dramatically changed. Nowadays, the treatment of this disease is concerned mainly with the pathophysiological target. In clinical practice, it ...

Last Updated: 9 Aug 2017

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A systematic review of genetic mutations in pulmonary arterial hypertension.
 

Author(s): Gerardo Garcia-Rivas, Carlos Jerjes-Sánchez, David Rodriguez, José Garcia-Pelaez, Victor Trevino

Journal:

 

Pulmonary arterial hypertension (PAH) is a group of vascular diseases that produce right ventricular dysfunction, heart failure syndrome, and death. Although the majority of patients appear idiopathic, accumulated research work combined with current sequencing technology show that ...

Last Updated: 3 Aug 2017

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Symptoms, Diagnosis, and Treatment

 
 
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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: 19 May 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: 7 Sep 2017

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