Treatment:

There is no known cure for pulmonary hypertension. The goal of treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve disorders.

Many new treatment options for pulmonary arterial hypertension (IPAH) and other forms of pulmonary aterial hypertension are becoming available. Medicines used to treat pulmonary hypertension include:

• Ambrisentan (Letairis)
• Bosentan (Tracleer)
• Calcium channel blockers
• Diuretics
• Prostacyclin or similar medicines
• Sildenafil and similar medicines

Your doctor will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you are responding to the medication. Never stop taking medicines without talking to your doctor.

Some patients are put on blood thinners to reduce the risk of blood clots in leg veins and lung arteries.

People with low oxygen levels in the blood may need oxygen therapy at home.

As the illness gets worse, you will need to make changes in your home and get more help around the house.

Other important tips to follow:

• Avoid pregnancy
• Avoid heavy physical activities and lifting
• Avoid traveling to high altitudes
• Keep up-to-date with yearly flu and pneumococcal vaccines
• Stop smoking

If treatment with medicine does not work, a lung or heart-lung transplant may help some people.

Expectations (prognosis):

The long-term outlook has been poor, but new treatments may lead to better results. Some people with this condition may have heart failure that could lead to death.

It is not a good idea to get pregnant if you have this condition.

Calling your health care provider:

Call your health care provider if:

• You begin to develop shortness of breath when you are active
• Shortness of breath worsens
• You develop chest pain
• You develop other symptoms

Most patients with pulmonary arterial hypertension are treated at centers that specialize in the care of these patients.