Associated Pulmonary Arterial Hypertension

What is associated pulmonary arterial hypertension?

Associated pulmonary arterial hypertension is also called APAH. 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 will lead to increased pressure in the large right chamber (ventricle) of your heart. Over time, these changes cause the affected blood vessels to scar and your heart to work harder.

What causes APAH?

APAH is caused by other conditions that affect your heart, lungs, or blood vessels. These are conditions that interfere with blood flow to the lungs or increase the pressure in the heart:

• Connective tissue diseases: These diseases are the most common cause of APAH, with the most frequent being scleroderma. Other diseases of this type that also cause APAH include lupus (SLE), rheumatoid arthritis, and Sjogren's syndrome.
  
  
• Heart birth defects: Some people are born with heart problems (defects). These defects can interfere with the normal path of blood flow through the body. These are usually a small hole in the wall between right and left sides of the heart. The hole can be between the upper (ASD) or lower (VSD) chambers of the heart, or both. These defects can also include the large blood vessels that attach to the heart. These blood vessels may be connected in a way that prevents blood from traveling the normal path in and out of the heart. If untreated, these defects may affect the blood flow to the lungs to cause APAH.
  
  
• Liver disease: Severe (very bad) liver disease injures and scars the liver. When this happens, blood cannot easily flow through the liver. This increases pressure in the artery carrying blood to the liver. This in turn affects the pressure in the pulmonary artery, causing APAH.
  
  
• HIV infection: APAH occurs in a small number of people with HIV infections and how it is caused is unclear.
  
  
• Medicines, drugs, and toxins: Some medicines that were used for weight loss have been shown to cause APAH. Street drug, such as cocaine and "meth", can also cause APAH. Medicines used for treating cancer and other toxic substances, such as rapeseed oil, may also cause APAH.
  
  
• Other diseases:
  
  
  • Thyroid disorders.
    
    
  • Disorders that block or prevent blood flow in your arteries. These include disorders cause your blood to clot easily and ones where red blood cells break down too fast, such as sickle cell disease.
    
    
  • Surgical removal of your spleen and other conditions that affect your spleen.

What are the signs or symptoms of APAH?

You may have any of the following in the early stages of this condition:

• Body weakness or fatigue (getting tired easily).
  
  
• Trouble breathing which occurs 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 APAH 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 widen (dilate) 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 on 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 that carry blood away from the heart) may also be checked to see if they are blocked. A special tube is threaded into your heart through a blood vessel in your groin or arm. The groin is the area just above your leg. X-ray dye is used and special x-ray pictures (fluoroscopy) are taken to see your blood vessels and your heart chambers.
    
    
  • Echocardiogram: This test is a type of ultrasound. Sound waves are used to show the structure, movement, and blood vessels of your heart.
  
  
• 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 bringing 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 APAH 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 APAH treated?

There is no known cure for APAH. 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 extra fluid in your blood and tissues (edema). Having extra fluid 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 widen (dilate) 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. The tube is placed in the left ventricle 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
  1301 Pennsylvania Ave. NW
  Washington , DC 20004
  Phone: 1- 202 - 785-3355
  Phone: 1- 800 - 548-8252
  Web Address: www.lung.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. 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.

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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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