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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- Immune system disease
- Exposure to certain drugs or chemicals
- Genetic defects
- Female between the ages of 30 and 40
- Liver cirrhosis
- Portal hypertension
- HIV infection
- Family history of PPH
- Use of appetite suppressants—diet drugs (eg, fenfluramine)
- Cocaine use
- Shortness of breath, especially when you are active or at rest
- Hyperventilation—abnormally rapid, deep breathing
- Progressive weakness
- Fainting spells
- Coughing up blood
- Cyanosis—a bluish tint to the lips and skin
- 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
- A heart murmur —an abnormal sound in the heart
- Blood tests—liver function, coagulation tests, test for HIV, autoantibody
- Blood gases to determine the oxygen status
- Electrocardiogram (ECG, EKG) —a test that records the heart’s electrical activity
- Chest x-ray —a test that uses radiation to take a picture of structures inside the body and can show if the heart is enlarged
- CT scan of the chest—a type of x-ray that uses a computer to make pictures of structures inside the chest
- Echocardiogram —a test that uses high-frequency sound waves (ultrasound) to examine the size, shape and motion of the heart
- 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
- Pulmonary arteriogram—a type of x-ray to detect blood clots and other blockages
- Nuclear lung scan—a special camera takes pictures of the lungs, to look for blockages in the blood vessels
- Six minute walk to determine the amount of shortness of breath, an indirect measure of the severity of PHH
- Calcium channel-blocking drugs ( Nifedipine , diltiazem , and amlodipine )—to relax the muscles in the blood vessels in the lungs
- Protacylin drugs ( epoprostenol , treprostinil ) and synthetic prostacylins ( iloprost , cisaprost)—to relax the muscles in the blood vessels in the lungs
- Digoxin —to improve the ability of the heart to pump blood
- Anticoagulants—to decrease the chance of blood clots in the lungs
- Diuretics—to reduce the amount of fluid in the body and in turn reduce strain on the heart
- Vasodilators—to help reduce blood pressure in the blood vessels in the lungs
- Endothelin receptor antagonists (Bosentan)—to relax muscles in blood vessels of lungs
- Phosphodiesterase-5 inhibitors ( sildenafil )—to relax muscles in blood vessels of lungs
Lung Transplant or Heart-Lung Transplant
PPH Cure Foundation http://www.pphcure.org/
Pulmonary Hypertension Association http://www.phassociation.org/
Heart and Stroke Foundation of Canada http://www.heartandstroke.com/
The Canadian Lung Association http://www.lung.ca/
American Heart Association. Primary or unexplained pulmonary hypertension. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=4752 . Accessed November 10, 2010.
American Lung Association. Primary pulmonary hypertension (PPH) fact sheet. American Lung Association website. Available at: http://www.lung.org/lung-disease/primary-pulmonary-hypertension/ . Accessed September 12, 2005.
Cedars-Sinai Medical Center. Imaging–nuclear lung scan. Cedars-Sinai Medical Center website. Available at: http://www.csmc.edu/pf%5F2760.html. Accessed September 21, 2005.
Cincinnati Children’s Hospital Medical Center. Primary pulmonary hypertension. Cincinnati Children’s Hospital Medical Center website. Available at: http://www.cincinnatichildrens.org/health/p/pulmonary-hypertension/ . Accessed November 10, 2010.
DynaMed Editorial Team. Primary pulmonary hypertension. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated September 29, 2010. Accessed November 10, 2010.
Rich S. the current treatment of pulmonary arterial hypertension: time to redefine success. Chest . 2006;130:1198-1202.
Zamanian RT, Haddad F, Doyle RL, Weinacker AB. 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: 09/2013 -
- Update Date: 09/30/2013 -
This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.
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