Treatment

There's no cure for pulmonary hypertension, but treatment is available to help improve signs and symptoms and slow the progress of the disease.

It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.

When pulmonary hypertension is caused by another condition, treatment is given for the underlying cause whenever possible.

Medications

Many different types of medications are available to help improve pulmonary hypertension signs and symptoms and slow disease progression, including:

• Blood vessel dilators (vasodilators). Vasodilators relax and open narrowed blood vessels, improving blood flow. A commonly prescribed vasodilator for pulmonary hypertension is epoprostenol (Flolan, Veletri).

  This drug continuously flows through an IV attached to a small pump, which is worn in a pack on the belt or shoulder. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea, leg cramps, and pain and infection at the IV site.

  Other types of vasodilators, including treprostinil (Tyvaso, Remodulin, Orenitram), can be inhaled, injected or taken by mouth. The drug iloprost (Ventavis) is given while breathing in through a nebulizer, a machine that vaporizes the medication.

  Side effects associated with treprostinil include chest pain, often with headache and nausea, and breathlessness. Possible side effects of iloprost include headache, nausea and diarrhea.

• Guanylate cyclase (GSC) stimulators. This type of medicine increases nitric oxide in the body, which relaxes the pulmonary arteries and lowers pressure in the lungs. GSC stimulators include riociguat (Adempas). Side effects include nausea, dizziness and fainting. You should not take GSC stimulators if you're pregnant.
• Endothelin receptor antagonists. These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. Such drugs include bosentan (Tracleer), macitentan (Opsumit) and ambrisentan (Letairis). These drugs may improve energy level and symptoms. However, they can damage the liver. You may need monthly blood tests to check your liver function. Endothelin receptor antagonists shouldn't be taken during pregnancy.
• Sildenafil and tadalafil. Sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis, Alyq) are commonly used to treat erectile dysfunction. But they also open the blood vessels in the lungs and allow blood to flow through more easily. Side effects can include upset stomach, headache and vision problems.
• High-dose calcium channel blockers. These drugs help relax the muscles in the walls of your blood vessels. They include amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension improve while taking them.

• Warfarin (Jantoven). Warfarin is a blood thinner (anticoagulant). It may be prescribed to help prevent blood clots in the pulmonary arteries. Blood thinners increase the risk of bleeding, especially in those who are having surgery or an invasive procedure. If you take warfarin, talk to your doctor about whether you need to stop taking the medication before surgery and for how long.

  Many other drugs, herbal supplements and foods can interact with warfarin. Talk to your doctor about your diet and all the medications you take, including those bought without a prescription. People who take warfarin need occasional blood tests to check how well the medicine is working.

• Digoxin (Lanoxin). This medicine helps the heart beat stronger and pump more blood. It can help control irregular heartbeats (arrhythmias).
• Diuretics. Commonly known as water pills, these medications help the kidneys remove excess fluid from the body. This reduces the amount of work the heart has to do. Diuretics may also be used to reduce fluid buildup in the lungs, legs and abdomen.
• Oxygen therapy. Breathing pure oxygen is sometimes recommended as a treatment for pulmonary hypertension, especially for those who live at a high altitude or have sleep apnea. Continuous oxygen therapy may be needed.

Surgery and other procedures

If medications do not help control the signs and symptoms of pulmonary hypertension, surgery may be recommended. Surgeries to treat pulmonary hypertension include:

• Atrial septostomy. This open-heart surgery may be recommended if medications don't control pulmonary hypertension signs and symptoms. In an atrial septostomy, a surgeon creates an opening between the upper left and right chambers of the heart (atria) to relieve the pressure on the right side of the heart. Potential complications include heart rhythm problems (arrhythmias).

• Lung or heart-lung transplant. Sometimes, a lung or heart-lung transplant may be recommended, especially for younger people who have idiopathic pulmonary arterial hypertension.

  Major risks of any type of transplantation include rejection of the transplanted organ and serious infection. Immunosuppressant drugs must be taken for life to help reduce the chance of rejection.