Pulmonary hypertension can't be cured, but doctors can help you manage your condition. Treatment may help improve your symptoms and slow the progress of pulmonary hypertension.
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. Your doctor might also need to change your treatment if it's no longer effective.
When pulmonary hypertension is caused by another condition, your doctor will treat the underlying cause whenever possible.
Blood vessel dilators (vasodilators). Vasodilators open narrowed blood vessels. One of the most commonly prescribed vasodilators for pulmonary hypertension is epoprostenol (Flolan, Veletri). The drawback to epoprostenol is that its effects last only a few minutes.
This drug is continuously injected through an intravenous (IV) catheter via a small pump that you wear in a pack on your belt or shoulder. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea and leg cramps, as well as pain and infection at the IV site.
Another form of the drug, iloprost (Ventavis), can be inhaled six to nine times a day through a nebulizer, a machine that vaporizes your medication. Because it's inhaled, it goes directly to the lungs. Side effects associated with iloprost include chest pain — often accompanied by a headache and nausea — and breathlessness.
Treprostinil (Tyvaso, Remodulin, Orenitram), another form of the drug, can be given four times a day. It can be inhaled, taken as oral medication or administered by injection. It can cause side effects such as a headache, nausea and diarrhea.
Endothelin receptor antagonists. These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. These drugs may improve your energy level and symptoms. However, these drugs shouldn't be taken if you're pregnant. Also, these drugs can damage your liver and you may need monthly liver monitoring.
These medications include bosentan (Tracleer), macitentan (Opsumit), and ambrisentan (Letairis).
- Sildenafil and tadalafil. Sildenafil (Revatio, Viagra) and tadalafil (Cialis, Adcirca) are sometimes used to treat pulmonary hypertension. These drugs work by opening the blood vessels in the lungs to allow blood to flow through more easily. Side effects can include an 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 medications such as amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia, others). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension respond to them.
- Soluble guanylate cyclase (SGC) stimulator. Soluble guanylate cyclase (SGC) stimulators (Adempas) interact with nitric oxide and help relax the pulmonary arteries and lower the pressure within the arteries. These medications should not be taken if you're pregnant. They can sometimes cause dizziness or nausea.
Anticoagulants. Your doctor is likely to prescribe the anticoagulant warfarin (Coumadin, Jantoven) to help prevent the formation of blood clots within the small pulmonary arteries. Because anticoagulants prevent normal blood coagulation, they increase your risk of bleeding complications.
Take warfarin exactly as prescribed, because warfarin can cause severe side effects if taken incorrectly. If you're taking warfarin, your doctor will ask you to have periodic blood tests to check how well the drug is working. Many other drugs, herbal supplements and foods can interact with warfarin, so be sure your doctor knows all of the medications you're taking.
- Digoxin. Digoxin (Lanoxin) can help the heart beat stronger and pump more blood. It can help control the heart rate if you experience arrhythmias.
- Diuretics. Commonly known as water pills, these medications help eliminate excess fluid from your body. This reduces the amount of work your heart has to do. They may also be used to limit fluid buildup in your lungs.
- Oxygen. Your doctor might suggest that you sometimes breathe pure oxygen, a treatment known as oxygen therapy, to help treat pulmonary hypertension, especially if you live at a high altitude or have sleep apnea. Some people who have pulmonary hypertension eventually require continuous oxygen therapy.
Atrial septostomy. If medications don't control your pulmonary hypertension, this open-heart surgery might be an option. In an atrial septostomy, a surgeon will create an opening between the upper left and right chambers of your heart (atria) to relieve the pressure on the right side of your heart.
Atrial septostomy can have serious complications, including heart rhythm abnormalities (arrhythmias).
Transplantation. In some cases, a lung or heart-lung transplant might be an option, 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, and you must take immunosuppressant drugs for life to help reduce the chance of rejection.