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There is currently no cure for pulmonary hypertension, although researchers continue to investigate treatments in the hope of finding a cure. In the meantime, successful management of the disease is possible. In cases of secondary pulmonary hypertension, the underlying cause (e.g., heart disease or another lung disease) will be the main focus of treatment.
Lifestyle changes are important. People with primary hypertension are often asked to stop taking all diet pills (especially those that suppress appetite), to avoid strenuous physical activity and to avoid being around people with respiratory infections. Short periods of regular exercise are recommended, but only when alternated with periods of rest to avoid over-exerting the lungs and heart. Eating a healthy diet is also important, including limiting salt and increasing the intake of antioxidant vitamins such as vitamin C, vitamin E and the trace mineral selenium.
Many patients with pulmonary hypertension are also put on oxygen therapy, especially among patients with chronic obstructive pulmonary disease (COPD). Additionally, although firm studies are lacking, patients with pulmonary hypertension caused by right ventricular dysfunction and other causes may be put on oxygen therapy.
Medications may also be prescribed to treat pulmonary hypertension. Because the responses to medications vary, physicians may try their patients on a few different types before a long-term therapy is formulated. In some cases, different medications may be administered during a cardiac catheterization so the physician can see their effects on blood vessels. Medications that may be used in the treatment of pulmonary hypertension include:
- Epoprostenol. This medication is a potent vasodilator that is administered intravenously. Patients who do not respond to other medications (e.g., calcium channel blockers) may be outfitted with a permanent catheter and an intravenous pump to administer continuous epoprostenol. Studies have shown that this medication improves lung function among patients with both secondary and primary pulmonary hypertension.
- Anticoagulants. Medications that inhibit the formation of blood clots. These are used to prevent blood clots from forming in the lungs, where the blood flow may be sluggish. They may also be prescribed among patients with secondary pulmonary hypertension that is due to one or more blood clots.
- Calcium channel blockers. Medications that reduce the workload of the heart by blocking calcium ions from signaling the blood vessels to constrict or tighten. Calcium channel blockers are often prescribed for patients who respond to vasodilators. However, their effectiveness is highly variable among patients with pulmonary hypertension, and in some patients, they may worsen the condition.
- Cardiotonics. Medications that strengthen the contractions of the heart so the heart does not need to beat as often in order to circulate an adequate amount of blood for the body.
- Diuretics. Medications that promote the formation of urine by the kidneys, causing the body to flush out fluids and minerals. If the kidneys malfunction and do not rid the body of the excess fluid in the system, the increased volume of blood being pumped through the blood vessels can lead to high blood pressure (hypertension). Diuretics are usually prescribed for patients with advanced heart or lung disease as a result of their pulmonary hypertension, including right-sided heart failure.
- Endothelin receptor antagonists. Medications that help increase blood supply to the lungs and, in so doing, reduce the workload of the heart. Bosanten, a type of endothelin receptor antagonist, is approved for use in the treatment of pulmonary hypertension.
- Prostacyclins. Medications that both dilate blood vessels (vasodilators) and inhibit the formation of blood clots (anticoagulants). Traditionally used during kidney dialysis, prostacyclins have also become a useful tool in treating pulmonary hypertension. However, prostacyclins currently can be delivered only through a continuous intravenous infusion. Other methods of delivery, including an inhaled form, are being researched.
- Phosphodiesterase (PDE) inhibitors. Medications that work by blocking an enzyme called phosphodiesterase (PDE) and facilitating the dilation of the blood vessels. Sildenafil PO is a common PDE inhibitor used to treat pulmonary hypertension.
A combination of medications, lifestyle changes and treatment of underlying conditions is usually enough to bring pulmonary hypertension under control. Surgical interventions, such as transplantation, are only considered in extreme cases. When lung transplantation and/or heart transplantation is necessary, the patient’s damaged organs are surgically replaced by healthy donated organs.
An experimental procedure called dilation atrial septostomy is being investigated for use in patients with severe pulmonary hypertension who are waiting for a heart and/or lung transplant. Dilation atrial septostomy involves making a small hole in the heart and slowly enlarging it to relieve some of the pressure in the heart’s right side by shunting blood across the atrial septum and into the left side of the heart. This procedure is similar to balloon atrial septostomy in which a naturally occurring hole present at birth is enlarged to help children with certain congenital heart defects. More research is necessary before this procedure can be useful as a “bridge to transplant” for patients with pulmonary hypertension.
Other areas of research for the treatment of pulmonary hypertension include stem cell research and gene therapy. | 
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