Familial Pulmonary Arterial Hypertension

WHAT YOU SHOULD KNOW:

• Familial pulmonary arterial hypertension is also called FPAH. This is a condition where the pressure in your pulmonary artery, connecting your lungs and heart, is increased. It may be caused by problems with your genes that may have been passed down to you by your parents. A gene is a little piece of information that tells the body what to do or what to make. Hypertension occurs when the small arteries in your lungs get smaller, making it harder for blood to flow through your lungs. While you may have no symptoms at first, over time you may get weak and more tired than the usual, have chest pain or trouble breathing, and you may faint.
  
  
• Your caregiver will ask about medicines you have taken, and diseases or conditions you have had, and their treatment. He will examine you, and do blood and imaging tests. The imaging tests may include a computed tomography (CT) scan, magnetic resonance imaging (MRI), or echocardiography. Treatment may include oxygen and medicines, such as blood thinners, diuretics ("water pills"), and ones to widen your arteries. If your condition worsens even with medicines, you may need to have surgery. You may need to have a shunt (special tube) placed in your heart, or one or both of your lungs may need to be replaced with healthy lungs from a donor. You may need to have both lungs and your heart replaced. There is no cure for FPAH, but with treatment, your condition may stay the same, or improve.

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.

RISKS:

Risks of FPAH treatment include allergic reactions to the medicines used for treatment. You may lose too much blood during surgery, or get an infection. Transplant surgery may lead to organ rejection. This is when the transplanted body organ does not work well or at all. If you do not get treated for FPAH, your symptoms may get worse and damage other organs. Over time, your heart may fail and you may die. Talk to your caregiver if you are worried or have concerns about FPAH, or your medicine or care.

WHILE YOU ARE HERE:

Informed consent:

A consent form is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

Rest:

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.

IV:

An IV (intravenous) is a small tube placed in your vein that is used to give you medicine or liquids.

Medicines:

You may be given the following medicines:

• Blood thinners: This medicine helps prevent clots from forming in the blood. Clots can cause strokes, heart attacks, and death. Blood thinners make it more likely for you to bleed or bruise. Use an electric razor and soft toothbrush to help prevent bleeding.
  
  
• Diuretics: This medicine is given to decrease edema (excess fluid) that collects in a part of your body, such as your legs. Diuretics can also remove excess fluid from around your heart or lungs and decrease your blood pressure. It is often called water pills. You may urinate more often when you take this medicine.
  
  
• Heart medicine: You may need medicine to increase the force of your heart when it pumps. This drug causes the heart to squeeze harder when it beats. When this happens, the heart may be able to move more blood with every beat.
  
  
• Vasodilators: These medicines dilate (widen) your blood vessels to help improve blood flow in your lungs. This also may decrease the strain on your heart in working hard to get blood into your lungs. You may be able to take these medicines by mouth or by inhaling them. You may need medicines that are given through an intravenous (IV) tube placed in a vein (blood vessel). You may need to be on a constant dose of IV medicine that uses an IV pump to deliver it.

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 caregiver before you take off the mask or oxygen tubing.

Pulse oximeter:

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.

Tests:

You may also need to have any of the following:

• Blood gases: This is also called an arterial blood gas, or ABG. Blood is taken from an artery (blood vessel) in your wrist, arm, or groin. Your blood is tested for the amount of oxygen and carbon dioxide in it. The results can tell caregivers how well your lungs are working.
  
  
• Blood tests: You may need blood taken to give caregivers information about how your body is working. The blood may be taken from your hand, arm, or IV.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
  
  
• Heart monitor: This test is also called an EKG or ECG. Sticky pads are placed on your skin to record your heart's electrical activity. An EKG gives information about how your heart is working. Lie as still as possible during the test.
  
  
• Imaging tests: Dye may be used in certain tests to make pictures show up better. Tell your caregiver if you are allergic to iodine or shellfish (lobster, crab, or shrimp), as you may also be allergic to this dye.
  
  
  • Computed tomography scan: This is also called a CT scan. A special x-ray machine uses a computer to take pictures of your heart and lungs. It may also be used to look at your blood vessels.
    
    
  • Magnetic resonance imaging: This test is also called MRI. During the MRI, pictures are taken of your chest. An MRI may be used to look at organs, such as the heart and lungs, and tissues, such as blood vessels. You will need to lie still during an MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal objects. This may cause serious injury. Tell your caregiver if you have any metal implants in your body.

Treatment options:

• Surgery:
  
  
  • Shunts: This is surgery to help blood flow from one part of the heart to another using a shunt (special tube). The tube is placed in the left ventricle (large chamber) of your heart. This decreases the work of the right ventricle, and allows more blood flow to the rest of your body. When there is greater blood flow, the delivery of oxygen to your body also increases. This surgery may also be done to prepare you for a lung, or heart and lung, transplant.
    
    
  • Transplants: This is surgery done to remove and replace a damaged organ with a healthy organ from a donor. You may have one or both lungs transplanted, or both lungs and your heart may be replaced. Transplants may be done for people whose conditions worsen even after treatment.
  
  

Vital signs:

Caregivers will check your blood pressure, heart rate, breathing rate, and temperature. They will also ask about your pain. These vital signs give caregivers information about your current health.

Copyright © 2012. Thomson Reuters. All rights reserved. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.

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.

Learn more about Familial Pulmonary Arterial Hypertension (Inpatient Care)