• 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)

    Definition

    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.

    Causes

    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:

    Symptoms

    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

    Diagnosis

    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:

    Treatment

    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

    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.

    Transplantation

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

    Prevention

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

    RESOURCES

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

    Pulmonary Hypertension Association https://phassociation.org

    CANADIAN RESOURCES

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

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

    References

    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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