What Is Idiopathic Pulmonary Arterial Hypertension?

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  • What Is Pulmonary Hypertension?

    What Is Pulmonary Hypertension?

    Pulmonary hypertension (PH) is a condition that causes increased blood pressure in the pulmonary arteries and the right side of the heart. The pulmonary arteries carry blood from the heart and into the lungs.

    Five types of PH exist. One common type is called pulmonary arterial hypertension (PAH). It’s a severe disease that progresses rapidly and usually causes right heart failure. In many cases, PAH is fatal and leads to death.

  • Exploring a Relationship

    Exploring a Relationship

    The types of PH are named according to what causes them and what the treatment options are. When PAH is the result of a pre-existing disease, such as a heart or lung disorder, it’s called secondary PAH.

    Sometimes, however, PAH occurs without any identifiable cause. This type of PAH is called idiopathic pulmonary arterial hypertension (IPAH). IPAH cannot be explained by another condition.

  • How Common Is IPAH?

    How Common Is IPAH?

    More than 1,000 Americans are diagnosed with PAH each year. IPAH is much more rare.

    According to Wexner Medical Center at The Ohio State University, IPAH occurs in about one in every one million patients each year.

    The condition is more common in women, and young adults are most often diagnosed with it.

  • What Causes PAH?

    What Causes PAH?

    The pulmonary arteries carry blood from the heart to the lungs, where the blood picks up oxygen. In people with PAH and IPAH, the small arteries become narrowed and constricted. One or more of these three things typically causes the narrowed arteries:

    • Tiny blood clots form in the smallest arteries in the lungs and block blood flow.
    • Increased cell growth in the blood vessels causes abnormally thick artery walls.
    • Artery walls squeeze and make blood flow more difficult.
  • IPAH and Genes

    IPAH and Genes

    Some people with IPAH may have a genetic predisposition to the disease. In other words, an inherited gene may play a role in determining who develops unexplained PAH and who doesn’t. The first thing a doctor will do when they detect PAH is to search for a cause that explains the high blood pressure. But, people diagnosed with IPAH show no recognizable cause for pulmonary high blood pressure.

  • Symptoms of IPAH

    Symptoms of IPAH

    The most common symptoms of IPAH include:

    • shortness of breath when physically active
    • tiredness
    • dizziness
    • fainting
    • swelling in the extremities, including legs and ankles
    • chest pressure or pain
    • racing pulse
  • How Is IPAH Diagnosed?

    How Is IPAH Diagnosed?

    IPAH is a diagnosis of exclusion. When doctors can’t determine the cause of PAH, they classify it as IPAH.

    If you’re experiencing symptoms of PAH, your doctor may request several tests and procedures that can help identify the cause. A noninvasive procedure called an echocardiogram may detect PAH. This imaging test can gauge the blood pressure inside your pulmonary arteries.

    Once PAH is found, doctors will want to determine what’s causing it.

  • IPAH Is Hard to Treat

    IPAH Is Hard to Treat

    Unfortunately, IPAH management and treatment are difficult. Management strategies are limited because doctors and medical researchers understand very little about IPAH and why it develops. Treatments aim to slow the progression of the disease and ease symptoms, not reverse or cure it.

    Medications commonly prescribed to treat IPAH include:

    • calcium channel blockers
    • endothelin receptor antagonists
    • vasodilators
  • Life Expectancy with IPAH

    Life Expectancy with IPAH

    Ultimately, IPAH and most forms of PAH are fatal. Treatments, including medicines and surgeries, can only slow the progression of the aggressive disease, not stop it.

    If you’ve been diagnosed with IPAH, work with your doctor and healthcare professionals to create a disease management plan. This plan should include treatment options, exercise regimens, and lifestyle changes that can make living with IPAH more comfortable. Together, you and your healthcare team can make dealing with IPAH less burdensome and make your days more fulfilling.