Idiopathic Pulmonary Arterial Hypertension
(PPH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Pulmonary Arterial Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension; Primary Pulmonary Hypertension)
|Heart and Lungs|
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- Autoimmune diseases
- Exposure to certain drugs or chemicals
- Genetic defects
- Liver cirrhosis
- Portal hypertension
- HIV infection
- Family history of PPH
- Use of appetite suppressants (diet pills)
- Cocaine use
- Shortness of breath (when you are active or at rest)
- Abnormally rapid, deep breathing—hyperventilation
- Progressive weakness
- Fainting spells
- Coughing up blood
- Bluish tint to the lips and skin—cyanosis
- Swelling of the legs and hands
- Chest pain
- Lack of appetite
- Cold hands and feet
- Low blood pressure
- Swelling of the veins in your neck
- Enlarged liver and swollen abdomen
- An abnormal sound in the heart—heart murmur
- Blood tests
- Pulse oximetry to evaluate how much oxygen is in your blood
- Electrocardiogram (EKG)—to test your heart’s electrical activity
- Pulmonary function tests—non-invasive tests, like blowing into a tube, that measure how well your lungs are working
- Cardiac catheterization—to detect problems with the heart and its blood supply
- Six minute walk to determine the amount of shortness of breath, an indirect measure of the severity of PHH
- Calcium channel blockers
- Endothelin receptor antagonists
- Phosphodiesterase-5 inhibitors
Lung Transplant or Heart-Lung Transplant
National Heart, Lung, and Blood Institute http://www.nhlbi.nih.gov
Pulmonary Hypertension Association http://www.phassociation.org
Canadian Lung Association http://www.lung.ca
Heart and Stroke Foundation of Canada http://www.heartandstroke.com
Nuclear lung scan. Cedars-Sinai Medical Center website. Available at: http://www.cedars-sinai.edu/Patients/Programs-and-Services/Imaging-Center/For-Patients/Exams-by-Procedure/Nuclear-Medicine/Nuclear-Lung-Scan.aspx. Accessed September 25, 2014.
Primary pulmonary hypertension. American Lung Association website. Available at: http://www.lung.org/lung-disease/primary-pulmonary-hypertension. Accessed September 25, 2014.
Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 25, 2014.
Pulmonary arterial hypertension (PAH). EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated June 30, 2014. Accessed September 25, 2014.
Rich S. The current treatment of pulmonary arterial hypertension: time to redefine success. Chest. 2006;130:1198-1202.
What is pulmonary hypertension? American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension%5FUCM%5F301792%5FArticle.jsp. Updated August 12, 2014. Accessed September 25, 2014.
Zamanian RT, Haddad F, et al. Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med. 2007;35:2037-2050.
- Reviewer: Michael J. Fucci, DO
- Review Date: 09/2015 -
- Update Date: 10/09/2015 -