Primary Pulmonary Hypertension
(PPH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Pulmonary Arterial Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension)
Pronounced: PRY-mair-ee PUL-mo-nair-ee hi-per-TEN-shunEn Español (Spanish Version)
Primary pulmonary hypertension (PPH) is a rare disease that causes high blood pressure in the blood vessels of the lungs. A person with PPH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them. So, the right side of the heart, which pumps blood to the lungs, has to work harder. This additional strain can eventually lead to heart failure.
PPH is a serious condition that requires care from your doctor.
Heart and Lungs
© 2008 Nucleus Medical Art, Inc.
The cause of PPH is unknown, but several factors may contribute to the development of the disease, including:
- Immune system disease
- Exposure to certain drugs or chemicals
- Genetic defects
A risk factor is something that increases your chances of getting a disease or condition. The following factors increase your chances of developing PPH:
- 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
Initial symptoms of PPH may be minor, and will get progressively worse. If you experience any of these symptoms do not assume it is due to PPH. These symptoms may be caused by other, less serious health conditions. If you experience any one of them, see your physician.
- Shortness of breath, especially when you’re active or at rest
- Hyperventilation – abnormally rapid, deep breathing
- Fatigue – tiredness
- 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
A physical exam by your doctor may show:
- Swelling of the veins in your neck
- Enlarged liver and swollen abdomen
- A heart murmur —an abnormal sound in the heart
Diagnosis of PPH may be delayed because it is hard to detect until symptoms worsen. Evaluation should take place at a medical center with expertise in pulmonary hypertension.
Your doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include the following:
- Blood tests—liver function, coagulation tests, test for HIV, autoantibody
- Pulmonary function tests
- Blood gases to determine the oxygen status
- Electrocardiogram (ECG, EKG) —a test that records the heart’s activity by measuring electrical currents through the heart muscle.
- Chest x-ray —a test that uses radiation to take a picture of structures inside the body. A chest x-ray 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 —a tube-like instrument inserted into the heart through a vein or artery (usually in the arm or leg) to detect problems with the heart and its blood supply.
- Pulmonary arteriogram—a type of x-ray that takes pictures of the blood vessels in the lungs, 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
There is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best treatment plan for you. Treatment options include the following:
- Calcium channel-blocking drugs—to relax the muscles in the blood vessels in the lungs
- Protacylin drugs (epoprostenol, treprostinil, lioprost)—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
Delivered through a mask or tubes inserted into the nostrils, if breathing becomes difficult.
Lung Transplant or Heart-Lung Transplant
Defective lungs and/or heart are replaced with donor organs. This option is used only in severe cases of PPH.
American Heart Association
American Lung Association
PPH Cure Foundation
Pulmonary Hypertension Association
Heart and Stroke Foundation of Canada
The Canadian Lung Association
Imaging–nuclear lung scan. Cedars-Sinai Medical Center website. Available at: http://www.csmc.edu/pf_2760.html . Accessed September 21, 2005.
Primary or unexplained pulmonary hypertension. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=4752 . Accessed September 12, 2005.
Primary pulmonary hypertension. Cincinnati Children’s Hospital Medical Center website. Available at: http://www.cincinnatichildrens.org/health/heart-encyclopedia/disease/pph.htm . Accessed September 12, 2005.
Primary pulmonary hypertension. US National Library of Medicine and the US National Institutes of Health Medline Plus website. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/000112.htm . Accessed September 12, 2005.
Primary pulmonary hypertension (PPH) fact sheet. American Lung Association website. Available at: http://www.lungusa.org/site/pp.asp?c=dvLUK9O0E&b=35699 . Accessed September 12, 2005.
Rich S: the current treatment of pulmonary arterial hypertension: time to redefine success. Chest . 2006;130:1198-202.
Zamanian RT, Haddad F, Doyle RL, Weinacker AB: Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med . 2007;35:2037-50.
Last reviewed January 2008 by David Juan, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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