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Familial Pulmonary Arterial Hypertension
WHAT YOU SHOULD KNOW:
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.
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.
- 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.
WHILE YOU ARE HERE:
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Keep the head of your bed raised to help you breathe easier. You can also raise your head and shoulders up on pillows or rest in a reclining chair. If you feel short of breath, let caregivers know right away.
A pulse oximeter is a device that measures the amount of oxygen in your blood. A cord with a clip or sticky strip is placed on your finger, ear, or toe. The other end of the cord is hooked to a machine. Never turn the pulse oximeter or alarm off. An alarm will sound if your oxygen level is low or cannot be read.
- 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.
- 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.
- An echocardiogram is a type of ultrasound. Sound waves are used to show the structure and function 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 a 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.
- 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.
- 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.
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.