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Pulmonary Hypertension

Pulmonary arterial hypertension (PAH) is also known as high blood pressure of the lungs. It is a disease that affects the heart and lungs.


The heart pumps blood throughout the body. Here’s how it works:

1. Blood from the body enters the right side of the heart.
2. The right side of the heart pumps blood to the lungs through the pulmonary (lung) artery.
3. In the lungs, the blood picks up oxygen that you breathe in and travels to the left side of the heart. The left side of the             heart then pumps this blood to the rest of the body.
4. Blood then returns to the right side of the heart, and the process starts over.


The effects of PAH on the heart and lungs:

When someone has PAH, the pulmonary arteries become narrow or blocked. This means the heart has to work harder to push the blood through the lungs. Over time, the heart cannot keep up. Less blood flows through the lungs to pick up oxygen. This results in PAH symptoms such as trouble breathing, dizziness, or feeling tired often.


Who Gets PAH, and What Causes the Disease?

PAH is a rare disease. Yet it is found in men and women of all races and ages. It is more common among women between the ages of 20 and 40. No one knows exactly why people get PAH. Doctors are learning more and more every day. Sometimes the cause of PAH is not clear. It may run in families – however – if someone in your family has PAH, that doesn’t mean you will get it.


PAH can also be caused by:
• Heart disease
• Liver disease
• Scleroderma, a disease that affects tissue and skin


PAH is also found in people who have used certain diet pills or illegal drugs like cocaine.


Getting Diagnosed

What the doctor will do?
The first thing the doctor will do is ask you about any symptoms you may have. Then you will be given a physical exam. During the exam the doctor will:
• Listen to your heart
• Check for other signs, such as swollen legs or ankles


The doctor may also order tests to confirm that you have PAH.
In order for the doctor to be sure you have PAH, the following test may be taken:
• Right Heart Catheterization – Measures blood pressure in your heart and arteries. A thin tube is inserted into an arm, leg,        or neck vein and then threaded into the heart


The doctor may also order the following tests to help make a diagnosis of PAH:
• Echocardiogram – Looks at your heart by making pictures of it with sound waves. This test checks for heart function, size,        and blood flow
• Blood Tests – Can detect other conditions or illnesses that can cause PAH
• Chest X-ray – Shows if there is any enlargement of the heart. X-rays may also show other conditions that have similar              symptoms, such as emphysema
• Computed Tomography, or CT/CAT Scan – A computerized x-ray machine that looks at your lungs. This tests for lung tissue    that is scarred or destroyed
• Magnetic Resonance Imaging (MRI) – Makes images of the human body without the use of x-rays. MRI uses a large magnet,    radio waves, and a computer to produce these images
• Pulmonary Function Tests – Measures how much air your lungs can hold and how well you can breathe


Treatment Options

There is currently no cure for PAH. However, some treatments may help you feel better. Your doctor will decide on a treatment that is right for you. He or she should then talk to you about the overall goals of your treatment.

Your doctor will choose what treatment is right for you based on a number of things, including:
• How advanced your PAH is
• Whether you are on any other medications
• If you have any other conditions that would affect your PAH treatment


Your doctor may also talk to you about changing certain lifestyle habits, such as smoking or drug use. Such habits can worsen PAH and get in the way of treatment.


Treatments for PAH include:
• Medicines that prevent blood clots – These medicines are called anticoagulants. They work by making it less possible for          the blood to clot so it flows more freely
• Medicines that help the heart – These medicines, such as digoxin, can help the heart work better
• Medicines that reduce swelling – These medicines include diuretics. They help the body get rid of excess fluid, which can          help the heart work better
• Oxygen therapy – This is used if breathing gets too difficult. A mask is placed over the nose and mouth, and patients                breathe in the extra oxygen through the nose
• PAH-specific therapies – Such as sildenafil, bosentan, epoprostenol, treprostinil, and iloprost

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