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Idiopathic pulmonary hypertension

Other Names for this Disease
  • Idiopathic pulmonary arterial hypertension
  • PAH
  • PPH
  • Primary pulmonary hypertension
  • Pulmonary arterial hypertension
More Names
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Overview


Idiopathic pulmonary hypertension 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 idiopathic pulmonary hypertension occur in individuals with no known 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.[1]

References

  1. Pulmonary arterial hypertension. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/pulmonary-arterial-hypertension. Accessed September 28, 2011.
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General Information

  • You can obtain comprehensive information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States. Click on the link to read information on this condition.
  • Genetics Home Reference (GHR) contains information on Idiopathic pulmonary hypertension. Click on the link to go to GHR and review the information.
  • MedlinePlus, a Web site designed by the National Library of Medicine to help you research your health questions, provides more information about this topic. Click on the link to view this information.
  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The National Heart, Lung, and Blood Institute (NHLBI) provides leadership for a national program in diseases of the heart, blood vessels, lung, and blood; blood resources; and sleep disorders. Since October 1997, the NHLBI has also had administrative responsibility for the NIH Woman's Health Initiative. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.  Click on the link to read information on this topic.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Idiopathic pulmonary hypertension. Click on the link to view a sample search on this topic.
  • The American Heart Association has an information page on pulmonary hypertension. You can access this information by clicking on the link above.
  • The American Lung Association has an information page on pulmonary hypertension. To access this information, click on the link above.
  • The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Idiopathic pulmonary hypertension. Click on the link to go to OMIM and review these resources.

Press Releases

  • The U.S. Food and Drug Administration (FDA) approved a new orphan drug called Letairis for the treatment of pulmonary arterial hypertension. To read the news release regarding this drug, click on the link.