This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|80%-99% of people have these symptoms|
|Elevated right atrial pressure||0005168|
|Increased pulmonary vascular resistance||0005317|
|Right ventricular failure||0001708|
|Right ventricular hypertrophy||0001667|
|30%-79% of people have these symptoms|
Abnormal blood clot
|Edema of the lower limbs||
Fluid accumulation in lower limbs
Tiredness[ more ]
Missed heart beat
Skipped heart beat[ more ]
|Pulmonary arterial medial hypertrophy||0004964|
|Pulmonary artery vasoconstriction||0005308|
|Pulmonary aterial intimal fibrosis||0005312|
|5%-29% of people have these symptoms|
|Abnormality of the tricuspid valve||0001702|
|Congestive heart failure||
Heart failure[ more ]
|Recurrent respiratory infections||
Frequent respiratory infections
Multiple respiratory infections
respiratory infections, recurrent
Susceptibility to respiratory infections[ more ]
|Sudden cardiac death||
Premature sudden cardiac death
|1%-4% of people have these symptoms|
|Pulmonary arterial hypertension||0002092|
|Percent of people who have these symptoms is not available through HPO|
|Arterial intimal fibrosis||0011353|
People with pulmonary arterial
Treatment of serious or life threatening PAH may involve continuous IV epoprostenol. Other treatment options, include treprostinil, iloprost, bosentan, ambrisentan, sildenafil, and tadalafil. Many of these treatments can be administered in various forms, such as by shot, IV, or inhalation. A small number of people with PAH respond well to long term oral calcium channel blockers. Blood thinners, diuretics, and supplemental oxygen may be prescribed as needed.
Many drugs can be harmful to people with PAH. The following should be avoided: appetite suppressants, cocaine, amphetamines (and related compounds), low oxygen environments (such as high altitudes), and possibly
Learn more orphan products.
The average survival of a person with pulmonary arterial
Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
New NCATS Rare Diseases Research Video
December 27, 2017
Rare Disease Day at NIH on March 1, 2018
December 19, 2017
Strategic Planning for Pulmonary Vascular Disease Research Monday, March 8, 2010 -
Tuesday, March 9, 2010
Location: Bethesda, Maryland
Description: Formulation of strategy for advancing clinical research in pulmonary arterial hypertension (PAH) was a high priority of this conference. Plans are to make participants' recommendations available via publication in a peer-reviewed journal. Initiatives on future research were considered.
Contact: Timothy M. Moore, M.D., Ph.D., Tim.Moore@nih.govTim.Moore@nih.gov
Co-funding Institute(s): National Heart, Lung, and Blood Institute, Office of Rare Diseases Research
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My daughter has been diagnosed with familial pulmonary arterial hypertension & I was wondering if there is a cure & if not, how long will she have to live? Thank you. See answer