Familial Pulmonary Arterial Hypertension

What is familial pulmonary arterial hypertension?

Familial pulmonary arterial hypertension (FPAH) is a condition in which the pressure in your pulmonary artery is increased. The pulmonary artery is the large blood vessel that brings blood from your heart to your lungs.

What causes FPAH?

Familial means that this condition can be passed from parents to children. For example, if you have a close family member with FPAH, you are more likely to have it. FPAH is caused by any medical condition that increases the pressure on your pulmonary artery. Connective tissue diseases, such as scleroderma, lupus, or rheumatoid arthritis, can cause FPAH. Weight loss medicines and illegal drugs can cause FPAH. Ask your caregiver about other conditions that can cause FPAH.

What are the signs and symptoms of FPAH?

  • Weakness and tiredness

  • Weight gain or lack of appetite

  • Joint pain

  • Shortness of breath with exercise

  • Abdominal swelling

  • Chest pain or heart palpitations (strong, fast heartbeats)

  • Dizziness or feeling faint

How is FPAH diagnosed?

Your caregiver will ask about your medical history and examine you. You may need any of the following tests:

  • Blood tests: You may need blood taken from your hand, arm, or IV. This is done to help caregivers find out what is causing your FPAH.

  • Blood vessel test: This is done to see if the small arteries in your lungs will widen (dilate) when you are given a medicine. The pressure in your pulmonary artery will be measured before and after the medicine is given.

  • ECG: This is also called an EKG. An ECG is done to check for damage or problems in your heart. A short period of electrical activity in your heart is recorded.

  • Cardiac catheterization: A thin, bendable tube inserted into an arm, neck, or groin vein is moved into your heart. Your caregiver may use an x-ray to guide the tube to the right place. Dye may be put into your vein so the pictures show up better on a monitor.

  • Chest x-ray or echocardiogram: A chest x-ray is a picture of your lungs and heart. An echocardiogram is a type of ultrasound. Sound waves are used to show the structure, movement, and blood vessels of your heart.

  • CT scan: This test is also called a CAT scan. An x-ray uses a computer to take pictures of your heart and lungs. You may be given dye before the pictures are taken to help caregivers see the pictures better. Tell caregivers if you are allergic to iodine or seafood. You may also be allergic to the dye.

  • MRI: This scan uses powerful magnets and a computer to take pictures of your heart and lungs. You may be given dye to help the pictures show up better. Tell caregivers if you are allergic to iodine or seafood. You may also be allergic to the dye. Do not enter the MRI room with anything metal. Metal can cause serious injury. Tell caregivers if you have any metal in or on your body.

  • Pulmonary function tests: Pulmonary function tests (PFTs) help caregivers learn how well your body uses oxygen. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time. PFTs help your caregivers decide the best treatment for you.

  • V/Q Scan: This is a ventilation (V) and perfusion (Q) test. This test is also called a VP scan. A V/Q scan is a two-part test which takes pictures of your lungs to look for certain lung problems.

    • During the perfusion part of the test, radioactive dye is put into your vein (blood vessel). The blood carries the dye to the blood vessels in your lungs. Pictures are taken to see how blood flows in your lungs.

    • During the ventilation part of the test, you breathe in special gas. Pictures are taken to see how well your lungs take in oxygen.

How is FPAH treated?

There is no known cure for FPAH. The goals of treatment are to improve your condition and stop it from getting worse. You may have any of the following:

  • Medicines:

    • Blood thinners: These prevent blood clots. They may make you bruise or bleed more easily. Use a soft toothbrush and an electric shaver to prevent bleeding.

    • Diuretics: These help your body get rid of extra fluid and protect your heart from more damage. You may urinate more often while you are taking diuretics.

    • Vasodilators: These improve blood flow by making the vessels in your heart and lungs wider. These may be given as a pill, an inhaler, or in your IV.

  • You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your healthcare provider before you take off the mask or oxygen tubing.

  • Surgery:

    • Shunt: This is surgery to help blood flow from one part of the heart to another using a tube called a shunt. This increases the blood flow and oxygen delivery to your body.

    • Transplant: This is surgery to remove and replace a damaged organ with a healthy organ from a donor. You may need a lung or heart transplant if other treatments do not work and your condition is severe.

How can I manage my FPAH?

  • Eat less salt: You may need to limit the amount of sodium you eat. Check labels to find low-sodium or no-salt-added foods. Some low-sodium foods use potassium salts for flavor. Too much potassium can also cause health problems. Ask your caregiver how much sodium and potassium salt you should eat each day.



  • Limit liquids: You may need to drink less fluids to help balance your fluid level. Ask how much liquid you should drink each day.

  • Get regular exercise: Exercise may help decrease your symptoms and improve your heart function. Exercise also helps with weight control. Never start an exercise program before you talk with your caregiver.

  • Special positions while sleeping: You may have trouble breathing when lying down. Sleeping in a position with your upper body raised may help you breathe easier. You can use foam wedges or elevate the head of your bed. There are many devices that you can buy to help raise your upper body while in bed. Use a device that will tilt your whole body, or bend your body at the waist. The device should not bend your body at the upper back or neck.

What are the risks of FPAH?

  • You may lose too much blood during surgery, or get an infection. Transplant surgery may lead to organ rejection, which is when the transplanted organ does not work well or at all.

  • Without treatment, the pressure may get worse and damage other organs. Over time, your heart may fail, which can be life-threatening.

When should I contact my caregiver?

Contact your caregiver if:

  • You have a fever.

  • Your symptoms keep you from doing your daily activities.

  • Your joints are painful and swollen.

  • Your fingers or toes are clubbed (the ends are round and thick).

  • You have questions or concerns about your condition or care.

When should I seek immediate care?

Seek care immediately or call 911 if:

  • You have shortness of breath at rest, especially when you lie down.

  • Your legs or ankles are swollen.

  • You are vomiting and cannot eat or drink.

  • You are confused or feel like you are going to faint.

  • You have chest pain or heart palpitations (strong, fast heartbeats).

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2014 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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