• 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)


    Primary pulmonary hypertension (PPH) is a rare disease. It is 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. As a result, the right side of the heart has to work harder to push blood to the lungs. This additional strain can eventually lead to heart failure.
    PPH is a serious condition. It requires care from your doctor.
    Heart and Lungs
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    Copyright © Nucleus Medical Media, Inc.


    The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
    • Autoimmune diseases
    • Exposure to certain drugs or chemicals
    • Genetic defects

    Risk Factors

    PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:
    • Liver cirrhosis
    • Portal hypertension
    • HIV infection
    • Family history of PPH
    • Use of appetite suppressants (diet pills)
    • Cocaine use


    Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause:
    • Shortness of breath (when you are active or at rest)
    • Abnormally rapid, deep breathing—hyperventilation
    • Fatigue
    • Progressive weakness
    • Fainting spells
    • Lightheadedness
    • 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


    Your doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis of PPH may be delayed. It is hard to detect until symptoms worsen.
    A physical exam by your doctor may show:
    • Swelling of the veins in your neck
    • Enlarged liver and swollen abdomen
    • An abnormal sound in the heart—heart murmur
    Tests may include:
    • 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
    Imaging tests evaluate the lungs and surrounding structures. These may include:


    There is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best plan for you. Treatment options include the following:


    Medication can improve blood flow, decrease the risk of blood clots, and improve the ability of the heart to pump blood. These may include:
    • Calcium channel blockers
    • Protacylins
    • Digoxin
    • Anticoagulants
    • Diuretics
    • Vasodilators
    • Endothelin receptor antagonists
    • Phosphodiesterase-5 inhibitors

    Supplemental Oxygen

    If breathing becomes difficult oxygen may be given. It may be given through a mask or tubes inserted into the nostrils.

    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 or when other treatment methods fail.


    There are no current guidelines for preventing PPH because the cause is not known.


    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.

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