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Familial Pulmonary Arterial Hypertension

GENERAL INFORMATION:

What is familial pulmonary arterial hypertension? Familial pulmonary arterial hypertension is also called FPAH. This is a condition where 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. Hypertension occurs when small arteries in your lungs get narrow. This decrease in size makes it harder for blood to flow through your lungs. When this happens, the pressure in your pulmonary artery increases. This leads to increased pressure in the right ventricle (large chamber) of your heart. Over time, these changes cause the affected blood vessels to scar and your heart to work harder.

What causes FPAH? Familial means that this is a genetic condition that runs in families. A familial genetic condition is a problem in the genes 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. Most people who have this genetic problem do not get FPAH. You may be at a higher risk if a close family member has FPAH. You may be concerned about having this condition or passing the genetic problem on to your children. Ask your caregivers about genetic counseling if you have these concerns.

What are the signs or symptoms of FPAH? You may have any of the following in the early stages of this condition:

• Body weakness or fatigue (getting tired easily).
  
  
• Trouble breathing when you exercise or do other activities.

The following signs and symptoms may develop if your condition gets worse:

• Abdominal (stomach) swelling or fullness.
  
  
• Chest pain and discomfort, or heart palpitations (strong, fast heartbeats).
  
  
• Dizziness, fainting (loss of consciousness), or light-headedness.
  
  
• Lack or loss of appetite, or weight gain.
  
  
• Swelling of your legs, joint pain, or clubbed fingers or toes. Clubbing happens when the ends of the fingers and toes become round and thick.
  
  
• Trouble breathing even while at rest, especially when lying down or sleeping.

How is FPAH diagnosed? Your caregiver will ask you about your medical and drug history. He will ask about the medicines you take and have taken in the past. He will do a physical exam. Tell your caregiver if you have skin diseases or if you are HIV positive. Tell him if you have used any medicine to decrease your appetite. 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.

You may need the following tests:

• Blood tests: You may need blood taken for tests. The blood can be taken from a blood vessel in your hand, arm, or the bend in your elbow. It is tested to see how your body is doing. It can give your caregivers more information whether this is a genetic condition or if other conditions are causing your symptoms. You may need to have blood drawn more than once.
  
  
• Blood vessel testing: This is called vasodilator testing. It is done to see if the small arteries in your lungs will dilate (widen) when you are given a medicine. The pressure in your pulmonary artery will be measured before and after the medicine is given. Caregivers will look to see if your pulmonary artery pressure goes down after the medicine is given. If it goes down, it means the small arteries in your lungs have dilated. This test may help your caregivers decide what medicine to treat you with. This test may also help caregivers understand how bad your condition may become.
  
  
• Heart tests:
  
  
  • ECG testing: You may have a 12-lead ECG (also called an EKG) test done to help caregivers look for problems in your heart. Sticky pads are placed you that have wires hooked up to a machine or TV-type screen. A short period of electrical activity in your heart muscle is recorded. Caregivers will look closely for certain problems or changes in how your heart is working. You may need this test more than once. You may also have other ECG tests done. This includes having you ride an exercise bike or walk on a treadmill while you are hooked up to the ECG monitor. If you are too ill for this test, you may instead be given medicine to increase your heart rate. Caregivers will see with the ECG how your heart responds to this stress.
    
    
  • Cardiac catheterization: This is a test, also called a heart cath, to see how well your heart is working. Your arteries (blood vessels) may also be checked to see if they are blocked. A special tube is threaded into your heart through a blood vessel in your leg or arm. X-ray dye is used and special x-ray pictures are taken to see your blood vessels and your heart chambers.
    
    
  • Echocardiogram:
    
    
    • This test is also called an echo. It is a type of ultrasound, using sound waves to show pictures of the size and shape of your heart. An echo also looks at how your heart moves when it is beating. These pictures are seen on a TV-like screen.
      
      
    • This test is done while lying down on your back. Clear jelly will be squirted on your chest to help the ultrasound sensor slide easily. The sensor will be rubbed across your chest to see your heart from different angles. You may hear a barking or whooshing noise, which is the sound of your blood flow. Caregivers may ask you to pedal a bike during the test (exercise echo) or you may get medicine before the test to increase blood flow to your heart muscle (stress echo). This test can tell how well your heart is pumping. An echo can also find problems, such as fluid around the heart or problems with your heart valves.
  
  
• Imaging tests: You may have a chest x-ray to first look at how your heart and lungs are doing. You may also have a computed tomography scan (CT scan) or a magnetic resonance imaging (MRI) test done. These are x-ray tests that use computers to take very detailed pictures of your chest. With these tests, caregiver can better see your heart, lungs, and other structures such as blood vessels. You need to lie very still during these tests. You will receive safety instructions before having an MRI to keep you from being injured by the very strong magnet in the MRI machine. These instructions include not bring metal items into the MRI room, including regular oxygen tanks.
  
  
• Lung and breathing tests: You may have a sleep study test. This test is done to see if your symptoms are caused by sleep apnea (periods of not breathing while you sleep). This test is done in a sleep lab, where you will spend the night. You will be attached to monitors to track your heart activity and oxygen level while you sleep. You caregivers may need to know more about how FPAH is affecting you and may do other tests. These may include pulmonary function tests (PFTs) to check how well your lungs are working. You may also have a ventilation (V) and perfusion (Q) test, also called a V/Q or VP scan. This test shows the blood flow in your lungs and how well your lungs pick up 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:

• Low salt diet: You may need to limit or stop using salt when cooking or eating. Using too much salt can cause your body to have edema (extra fluid in your blood and tissues). Having edema can cause your heart to have to work harder to pump your blood. You may need to decrease the amount of liquids that you drink. Ask your caregiver how much liquid you should drink each day.
  
  
• Medicines: If you have a respiratory tract infection, have it treated with medicines as early as possible. You may also need to have a shot of vaccine to help improve your immune system. Ask your caregiver for the type of vaccine that you may need. Other medicines you may need include:
  
  
  • Blood thinners: These medicines help prevent clots from forming in the blood. Clots can cause strokes, heart attacks, and death.
    
    
  • Heart medicines: You may need a diuretic medicine, often called "water pills". Diuretics help your body get rid of extra fluid to decrease how hard your heart has to work to pump blood. This medicine may also decrease your blood pressure. You may urinate more often when taking diuretics. You may also need medicine to increase the force of the heart when it pumps. This medicine causes the heart to squeeze harder when it beats.
    
    
  • 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 to help you breathe easier. It may be given through a plastic mask over your mouth and nose. It may be given through a nasal cannula. A nasal cannula is a pair of short, thin tubes that rest just inside your nose. Tell your caregiver if your nose gets dry, or if the mask or cannula bothers you. Ask your caregiver before taking off your oxygen. Never smoke or let anyone else smoke in the same room while your oxygen is on. This can help prevent fire from starting. Carry oxygen with you when you travel, especially if you are going on an airplane.
  
  
• Surgery: You may need to have a shunt (special tube) put in your heart. This surgery helps your 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 lung, or heart and lung, transplant. Transplant 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.

Where can I find more information? Contact any of the following for more information:

• American Lung Association
  61 Broadway, 6th floor
  New York City, NY 10006
  Phone: 1-800-586-4872
  Web Address: http://www.lungusa.org
  

• National Heart, Lung and Blood Institute
  Health Information Center
  P.O. Box 30105
  Bethesda, MD 20824-0105
  Phone: 1-301-592-8573
  Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
  

CARE AGREEMENT:

You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

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