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.
- Lung transplantation
- Anticoagulants 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 (Flolan) is a type of medicine called a prostacyclin. 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 very 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.
- Iloprost (Ventavis) In December 2004, a new form of the prostacyclin drug, iloprost (Ventavis), 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 most needed.
- Remodulin (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 (Tracleer), 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.
- Diuretics (water or fluid pills) may help ease symptoms and improve the heart's performance in some patients with PAH.
- Revatio - Sildenafil is a medicine called a phosphodiesterase type 5 (PDE5) inhibitor with the same ingredients as Viagra. In studies, Revatio increased the distance people walked and decreased pressure in the pulmonary artery. Revatio was not studied in patients taking bosentan (a medicine used in patients with PAH) so consult with your doctor. Revatio works by controlling how narrow or wide the blood vessels in the lining of the lungs can be by helping the blood vessels relax so blood and oxygen can then flow more freely.
- Ambrisentan (Letairis) is a selective receptor antagonist being developed as an oral therapy for patients with pulmonary arterial hypertension. Advantages of the drug include low levels of liver toxicity, high potency allowing low doses, once a day dosing and lower incidence of interaction with other drugs. For more information, CLICK HERE.
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.
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 medication 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