Pulmonary hypertension is a condition which leads to abnormally high blood pressure in the blood vessels of the lungs. In the normal circulatory system, de-oxygenated blood from the right side of the heart travels through the pulmonary artery to the lungs, where carbon dioxide and waste gases are exchanged for fresh oxygen. The oxygenated blood is then sent back to the heart, then pumped through the aorta and on to the rest of the body.

In patients with pulmonary hypertension, the muscles in the walls of the pulmonary arteries tighten, narrowing the passageway inside the affected portion of the blood vessels. Increased pressure of blood as it flows through the arteries can damage the walls and cause them to scar and thicken. This process further narrows the affected arteries. In time, blood clots may form or become trapped in a narrowed portion of the vessel, blocking the flow of blood.

Symptoms of pulmonary hypertension vary by the stage of the disease. Initially, patients may become short of breath while exercising. As the disease progresses, breathlessness occurs with moderate activity. Eventually, patients become short of breath even with minimal activity. Some other signs include: fatigue, chest pain, dizziness, fainting, swelling in the legs or ankles, palpitations and a bluish tint to the skin and lips.

Pulmonary hypertension causes the right side of the heart to pump harder to get blood flow to the lungs. The overworked chambers of the heart can weaken and lose their ability to effectively pump enough blood to meet the needs of the body. This is called right-sided heart failure.

The exact incidence of pulmonary hypertension isn't known. However, the American Heart Association estimates up to 1,000 new cases are diagnosed each year. The condition can occur at any age, but is more commonly diagnosed in women between 20 and 40. In 2000, about 3,065 Americans died of pulmonary hypertension.

For many patients, no cause can be found for pulmonary hypertension. This is called primary pulmonary hypertension. Known causes of pulmonary hypertension include: chronic heart or lung disease, blood clots in the lungs, scleroderma, sickle cell disease, HIV, liver disease and use of certain appetite suppressants.

Diagnosis and Treatment

Pulmonary hypertension may not be diagnosed until the disease has reached more advanced stages. That's because the disease is not very common and symptoms (like shortness of breath) are often associated with lack of conditioning or other health problems. Generally, diagnosis is made based on medical history and diagnostic tests, like echocardiography or ultrasound of the heart. Vallerie McLaughlin, M.D., Cardiologist with the University of Michigan Medical Center, says confirmation of pulmonary hypertension is made with a right heart catheterization.

There is no cure for pulmonary hypertension. However, there are some treatments to help ease the symptoms of the disease. Anticoagulants may help reduce the risk of blood clots. Calcium channel blockers help to relax the arteries and lower blood pressure. Oral and inhaled treatments may also be used. One of the most effective medications for pulmonary hypertension is epoprostenol. The drug is given though an IV line. Patients wear a special receptacle pump that continually delivers the medication into a vein through a catheter.

As pulmonary hypertension progresses, patients may require supplemental oxygen. Eventually, oxygen may be needed 24-hours/day. When standard medical therapy is no longer effective, a patient may be a candidate for a lung transplant.

Vallerie McLaughlin, M.D. (featured in story), Cardiologist, University of Michigan Medical Center, Department of Public Relations and Marketing Communications, 2901 Hubbard St., Suite 2400, Ann Arbor, MI 48109. (Media Contact: Katie Gazella, (734) 764-2220.)

For general information on pulmonary hypertension:

American Heart Association, or contact your local chapter

American Lung Association

National Heart, Lung and Blood Institute

Pulmonary Hypertension Association