It often takes some time to find the best treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care. Your doctor may 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). 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. This means that you'll learn to prepare your own medication mixture, operate the pump and care for the IV catheter. You'll need comprehensive follow-up care. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea, leg cramps, as well as pain and infection at the IV site.
Another form of the drug, iloprost (Ventavis), avoids many of these problems. Iloprost can be inhaled every three hours through a nebulizer, a machine that vaporizes your medication, making it far more convenient and less painful to use. And because it's inhaled, it goes directly to the lungs. Side effects associated with iloprost include chest pain — often accompanied by headache and nausea — and breathlessness.
- Endothelin receptor antagonists. These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. One of these medications, bosentan (Tracleer), may improve your energy level and symptoms. The drug isn't for pregnant women. If you take bosentan, you'll need monthly liver monitoring, because the drug can damage your liver. Ambrisentan (Letairis) is another medication that stops the narrowing of your blood vessels. This drug can cause serious liver damage if not taken appropriately, and it shouldn't be taken by pregnant women. Before taking the drug, tell your doctor about any liver condition you have.
- 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 include upset stomach, dizziness 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 (Adalat, Procardia). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension respond to them.
- 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.
- 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 also may be used to limit fluid buildup in your lungs.
- Oxygen. Your doctor may 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 with pulmonary hypertension eventually require constant oxygen therapy.
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- Atrial septostomy. If medications don't control your pulmonary hypertension, this open-heart surgery may be an option. In an atrial septostomy, a surgeon will create an opening between the left and right chambers of your heart 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 may be an option, especially for younger people who have idiopathic pulmonary 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.
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