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What is Pulmonary Hypertension?

In some people, the blood vessels in their lungs become narrowed, causing high blood pressure in the lungs. This is a complex health problem called pulmonary hypertension or PH. Pulmonary refers to the lungs. Hypertension is the name for high blood pressure. PH may also scar the blood vessels in the lungs. For the heart to push blood through smaller, scarred blood vessels, the heart’s right side must work harder. Over time, the heart can enlarge and weaken. This may lead to congestive heart failure.

Four Main Types of PH

  • Pulmonary Arterial Hypertension results from thickening and narrowing of the arteries that carry blood from the heart’s right side to the lungs. It may be primary or secondary. When the cause is not known, it is called idiopathic pulmonary hypertension (IPH). Another name is primary pulmonary hypertension (PPH). When another health problem is the cause, it is called secondary pulmonary hypertension. Some possible causes are: collagen vascular disease, such as lupus, scleroderma, and rheumatoid arthritis; some heart defects that are present at birth; severe liver disease; certain weight loss medicines; and HIV infection
  • Pulmonary Venous Hypertension from diseases of the left side of the heart
  • Pulmonary Hypertension from Respiratory Disease, such as emphysema, sleep apnea, interstitial lung disease, and long-term high altitude exposure
  • Pulmonary Hypertension from chronic blood clotting in the lungs

Symptoms of PH

The symptoms of PH may be similar to symptoms of other illnesses. This can make it difficult for doctors to identify PH. Common symptoms of PH include:

  • Shortness of breath
  • Extreme tiredness (fatigue)
  • Fast heart beat (palpitations)
  • Chest pain
  • Fainting
  • Swelling in the legs

How is PH detected and evaluated?

To decide on the best treatment for you, the doctor must find the exact cause of your PH. Below is a list of tests that you may have:

  • Blood tests: To detect other illnesses that can cause PH.
  • Echocardiogram: To look at your heart by making pictures of it with sound waves. This test checks the heart for size, ability to function and blood flow.
  • CT scan: To look at your lungs by computerized X-rays of the chest. The test checks for lung tissue that is scarred or destroyed.
  • Ventilation-perfusion scan: To look at your lungs after injecting dye through an IV (intravenous line). This test checks how well air and blood go through your lungs and if there are blood clots in your lungs.
  • Pulmonary function test: To measure how much air and how fast you can breathe into a machine. The test checks for lung diseases such as asthma or emphysema.
  • Heart catheterization: To measure exact blood pressure in your heart and arteries. A thin, flexible tube is inserted into an arm, leg or neck vein, and then threaded into the heart and lung artery. A dye may be injected into the heart arteries to look for blockages. This test can show if you have PH, how severe it is and what is causing it.
  • Cardiac MRI: To look at the anatomy of the heart and lungs. In this test, you lie flat on a table while a large magnet moves around your chest, taking pictures. This cannot be done if you have any metal implants in your body (for example, a metal plate from a broken bone)
  • Sleep study: To check oxygen levels in your blood while you sleep. The test shows if you have periods when you stop breathing for a few seconds during the night.

How is PH treated?

Several recent advances have been made in treatments for PH. This section tells you about the main types of treatment.

Medicines
(The generic name of each medicine is given first; the brand name is second)

Medications for blood pressure in the lungs:

  • Bosentan (Tracleer): A pill taken twice a day to help reduce pressure in your lungs, enable you to exercise more and slow down the disease. You must have blood tests every month to check your liver, because this medicine may harm the liver. If a liver problem develops, your liver should return to normal if you stop the medicine or take a lower dose. You should avoid pregnancy while on this medicine because it can harm an unborn child.
  • Ambrisentan (Letairis): A pill taken once a day to help reduce pressure in your lungs, enable you to exercise more and slow down the disease. You must have blood tests every month to check your liver, because this medicine may harm the liver. If a liver problem develops, your liver should return to normal if you stop the medicine or take a lower dose. You should avoid pregnancy while on this medicine because it can harm an unborn child.
  • Sildenafil (Revatio): A pill taken three times a day to help reduce pressure in your lungs, enable you to exercise more and slow down the disease. Blood tests are usually not needed for this type of pill.
  • Epoprostenol (Flolan): An IV (intravenous line) medicine that you receive non-stop. It requires a permanent IV catheter inserted into a vein and a portable pump. This medicine is usually given to patients with more serious illness. It can help to lessen your symptoms, enable you to exercise more and reduce blood pressure in your lungs. It may prolong life. Some side effects may occur, such as headache, diarrhea, facial redness (flushing), jaw pain, muscle pain and infection at the catheter site.
  • Treprostinil (Remodulin): A medicine given through a catheter into the bloodstream through an IV, or into the fatty tissue on your belly. The benefits of this medicine are similar to those of epoprostenol. For the form injected into the belly, a smaller pump the size of a pager is worn on the belt. The most common side effect is pain where the medicine goes into the skin. Infection is much less common with this medicine.
  • Calcium channel blockers: A pill to help relax narrowed blood vessels in the lungs. This medicine works only for a small number of patients.

Medications for heart failure and fluid build-up:

  • Digoxin: A pill to help the right side of the heart work better.
  • Diuretics: A pill to help reduce fluid build-up. These medicines are also called “water pills.”

Other Therapies

  • Warfarin (Coumadin): A pill taken once a day to prevent blood clots.
  • Oxygen therapy: Inhaled through the nose by a tube that rests just under the nose. Oxygen is used if the levels of oxygen in your blood are low.
  • Transplantation: When medicines are not able to help enough, a lung transplant or a heart-and-lung transplant may be considered.

For More Information

Some useful resources can be found at www.phassociation.org, the Web site of the Pulmonary Hypertension Association. The Pittsburgh Patient Support Group is another helpful resource. For more information, contact Sharren Yamron, Chairperson, at 412.829.0069 or sher351@adelphia.net .
 

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