• 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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    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:


    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
    • ECG—to test your heart’s electrical activity
    • Pulmonary function tests—noninvasive 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 includes 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
    • Prostacylins
    • Digoxin
    • Anticoagulants
    • Diuretics
    • Vasodilators
    • Endothelin receptor antagonists
    • Phosphodiesterase-5 inhibitors

    Supplemental Oxygen

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


    If PPH is severe or other treatment methods fail, a lung transplant or heart-lung transplant may be needed.


    There are no current guidelines to prevent PPH because the cause is unknown.


    National Heart, Lung, and Blood Institute https://www.nhlbi.nih.gov

    Pulmonary Hypertension Association https://phassociation.org


    Heart and Stroke Foundation of Canada http://www.heartandstroke.ca

    The Lung Association https://www.lung.ca


    Explore pulmonary hypertension. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health/health-topics/topics/pah. Updated August 2, 2011. Accessed September 14, 2017.

    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 14, 2017.

    Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at: https://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 14, 2017.

    Pulmonary arterial hypertension (PAH). EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T115043/Pulmonary-arterial-hypertension-PAH. Updated June 14, 2017. Accessed September 14, 2017.

    Pulmonary hypertension. American Lung Association website. Available at: http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/pulmonary-hypertension. Accessed September 14, 2017.

    Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension%5FUCM%5F301792%5FArticle.jsp#.Wbr2xrKGNxA. Updated May 23, 2017. Accessed September 14, 2017.

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