How Is Pulmonary Arterial Hypertension Treated?

The goals of treatment for patients with pulmonary arterial hypertension (PAH) are to:

• Treat the underlying cause. This is the first priority in patients with secondary pulmonary arterial hypertension (SPAH). Sometimes this treatment can correct the PAH.
• Reduce symptoms and improve quality of life.
• Slow the growth of the smooth muscle cells and the development of blood clots.
• Increase the supply of blood and oxygen to the heart, while reducing its workload.

These treatments include:

• Medicines
• Oxygen
• Lung transplantation

Medicines

• Anticoagulants (AN-te-ko-AG-u-lants) reduce the formation of blood clots.
• Calcium channel blockers relax blood vessels and increase the supply of blood and oxygen to the heart, while reducing its workload. These drugs can be very helpful, but only for a small amount of patients. All patients that take them should be monitored carefully.
• Epoprostenol (e-poe-PROST-en-ole) is a type of medicine called a prostacyclin (pros-ta-SI-klin). It is currently considered the most effective therapy for primary pulmonary arterial hypertension (PPAH). It may also be helpful in treating some forms of severe SPAH. This medication widens the lung arteries and prevents blood clot formation. Until recently, it was given intravenously through a permanent tube, or catheter, placed in a vein in the neck and connected to a battery-powered pump. But in December 2004, a new form of the drug, iloprost, was approved for the treatment of PAH. This medicine can be inhaled through a nebulizer. This makes it more convenient and less painful to take. Plus the medicine goes directly to the lungs, where it is needed.
• Treprostinil, another prostacyclin, also relaxes blood vessels and increases the supply of blood to the lungs, reducing the workload of the heart. It can be given under the skin.
• Bosentan, a relatively new treatment, widens the lung arteries and reduces blood pressure. It also is available in pill form. Until doctors learn more about the long-term effects, all patients taking this drug should be monitored for complications, especially those involving the liver.
• Nitric oxide inhalation, which causes the pulmonary arteries to widen or open, is also being used by some doctors.
• Sildenafil (Viagra®), another drug that causes the pulmonary arteries to open, has recently been shown to improve the condition of PAH patients and is expected to be approved soon. This drug is available in pill form.
• Diuretics (water or fluid pills) may help ease symptoms and improve the heart's performance in some patients with PAH.

Oxygen

You may need oxygen therapy if the level of oxygen in your blood is low. Oxygen is usually given through nasal prongs or a mask. Over time, you may need oxygen around the clock.

Lung Transplantation

Surgery to replace one or both diseased lungs with healthy lungs from a human donor may help some patients. This procedure is usually recommended for patients for whom medical therapy is no longer effective. Complications include rejection by the body of the transplanted lung and infection. Transplant patients must take medicines for life to reduce the chances that their body will reject the transplanted lung.

Other Possible Treatments

Researchers also are studying whether stem cell transplantation combined with gene therapy may provide a cure for PAH in the not too distant future.