Idiopathic Pulmonary Arterial Hypertension

GENERAL INFORMATION:

What is idiopathic pulmonary arterial hypertension? Idiopathic pulmonary arterial hypertension is also called IPAH. This is a condition where the pressure in your pulmonary artery is increased. The pulmonary artery is the 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, your body increases the pressure in your pulmonary artery. This lead to a backwards flow of blood, and increased pressure in the right ventricle of your heart.

What may cause IPAH? Idiopathic means that the cause is unknown. Certain conditions, such as tumors pressing on the pulmonary artery, may cause this condition. Problems in your heart, lungs, or blood vessels may also cause IPAH. Ask your caregiver about other conditions that may cause IPAH.

What are the signs or symptoms of IPAH? You may have any of the following:

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

• Abdominal (stomach) swelling or fullness.
  
  
• Chest pain and discomfort, or 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 IPAH diagnosed? Your caregiver will ask you about diseases or conditions that you have had and their treatments. You will get a physical exam. Tell your caregiver if you have skin diseases or if you are HIV positive. Tell him if you use 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:

• Cardiac catheterization: This is a test 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.
  
  
• 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.
  
  
• 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 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.
  
  
• 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.
  
  
• Pulmonary function tests: Pulmonary function tests, also called PFTs, help caregivers learn how well your lungs work. PFTs may also help your caregivers decide on the best treatment for you. During the tests, 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. This helps caregivers to see how well your lungs are moving and working.
  
  
• 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 IPAH treated? There is no known cure for IPAH. The goals of treatment are to improve your condition and stop it from getting worse. You may have any of the following:

• Medicine:
  
  
  • Blood thinners: These medicines help prevent clots from forming in the blood. Clots can cause strokes, heart attacks, and death.
    
    
  • Diuretics: This medicine is often called "water pills". Diuretics help your body get rid of extra fluid (edema) in your legs and ankles. This medicine may also help get rid of extra fluid in your lungs or around your heart. It may also decrease your blood pressure. You may urinate more often when taking diuretics.
    
    
  • Vasodilators: These medicines widen (make bigger) the diameter of your blood vessels and help improve blood flow.
  
  
• 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. Doing this can help prevent fires from starting. Carry oxygen with you when you travel, especially if you are going on an airplane.
  
  
• 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 (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.
  
  
• Others:
  
  
  • 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.
    
    
  • You may need to limit or stop using salt when cooking or eating. Too much salt is not good for your heart. You may need to decrease the amount of liquids that you drink. Ask your caregiver how much liquid you should drink each day.

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.

Copyright © 2008 Thomson Healthcare Inc. 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.

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