How is pulmonary hypertension (PH) treated?

Appropriate diagnosis and analysis of the problem is necessary before starting any treatment. Treatment varies by patient, based on the different underlying causes, but generally includes:

  • Taking medications
  • Making lifestyle and dietary changes
  • Having surgery, if necessary
  • Seeing your doctor regularly

Listed below are medication, lifestyle and dietary suggestions, and surgical treatment approaches.

Medications

Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depend on the underlying cause of pulmonary hypertension, how severe the pulmonary hypertension is, how likely it is to progress, and a patient’s drug tolerance.

  • Oxygen — replaces the low oxygen in your blood.
  • Anticoagulants or "blood thinners" such as warfarin sodium (Coumadin®) — decreases blood clot formation so blood flows more freely through blood vessels. Note: When taking anticoagulant medications, it is important for you to monitor bleeding complications and have regular lab work to monitor the level of medication in your bloodstream.
  • Diuretics or "water pills" [such as furosemide (Lasix®), spironolactone (Aldactone®)] — removes extra fluid from the tissues and bloodstream, which reduces swelling and makes breathing easier.
  • Potassium (such as K-dur®) — replaces potassium (an essential nutrient) that may be lost with increased urination when taking diuretics.
  • Inotropic agents (such as digoxin) — improves the heart's pumping ability.
  • Vasodilators [such as nifedipine (Procardia®) or diltiazem (Cardizem®)] — lowers pulmonary blood pressure and may improve the pumping ability of the right side of the heart.
  • Bosentan (Tracleer®), ambrisentan (Letairis®), macitentan (Opsumit) — helps block the action of endothelin, a substance that causes narrowing of lung blood vessels. Bosentan requires monthly lab work to monitor liver function.
  • Epoprostenol (Veletri, Flolan®), treprostinil sodium (Remodulin®, Tyvaso®, Orenitram ® ), iloprost (Ventavis®) — dilates (widens) pulmonary arteries and helps prevent blood clots from forming.
  • Sildenafil (Revatio®), tadalafil (Adcirca®) — relaxes pulmonary smooth muscle cells, which leads to dilation of the pulmonary arteries.
  • Riociguat (Adempas®)
  • Selexipag (Uptravi ®)

Medication guidelines

  • Know the names of your medications and how they work. Keep a list with you.
  • Take your medications at the same time every day. If you forget a dose, do not take two doses to make up for the dose you missed.
  • Do not take any over-the-counter drugs unless you ask your doctor or pharmacist first. Some drugs such as decongestants (including Benadryl®, Dimetapp®, Sudafed® and other drugs containing ephedrine or pseudoephedrine) and nonsteroidal anti-inflammatory agents (such as Advil®, Motrin®, and Indocin®) can cause problems in people who have heart failure. Also avoid any over-the-counter medications whose labels state that caution is to be used if you have high blood pressure.
  • Do not stop taking or change your medications unless you first talk with your doctor.
  • Avoid herbal products because of their uncertain effects when combined with medications used for pulmonary hypertension.

Dietary changes

  • Eat foods that are high in nutrients like potassium (e.g., dried fruits, bananas, oranges) and magnesium (e.g., peanuts, tofu, broccoli) and vitamins.
  • Limit your total daily calories, if necessary, to achieve your ideal body weight.
  • Limit foods that contain refined sugar, saturated fats, and cholesterol.
  • Eat foods that are high in fiber, including whole grains, brans, fruits, and vegetables.
  • Reduce your sodium intake:
    • Buy foods that are low in sodium. Choose foods with labels that read "low salt" and "low sodium."
    • Avoid table salt and seasoning salt.
    • Avoid smoked, cured, salted, and canned meat products.
    • Avoid fast food and limit prepared foods; they usually have a high sodium content.
  • Monitor your fluid intake. Cut down intake of fluids if you have become more short of breath or if you notice swelling.

Lifestyle changes

  • Monitor your weight: weigh yourself at the same time each day and record your weight in a diary or on a calendar. If you notice a rapid weight gain of 2 pounds in one day or 5 pounds over one week, inform your doctor.
  • Lose weight if you are overweight (follow your doctor’s recommendations).
  • Have annual health check-ups with your primary care physician.
  • Activity guidelines:
    • Restrict lifting, pushing, or shoving to less than 20 pounds, since these activities increase the pressure in your arteries and lungs.
    • Walking is important to keep your muscles strong and improve circulation. However, more strenuous forms of aerobic exercise should not be done regularly since your lungs may not be able to keep up with the oxygen demands placed on your body during these activities. Any activities that cause shortness of breath, dizziness, or chest pain should be stopped immediately. Contact your doctor if any of these symptoms occur.
  • Do not smoke or chew tobacco.
  • Avoid or reduce alcohol intake.
  • Avoid pregnancy; use contraceptives to prevent pregnancy.
  • Maintain close contact with your healthcare providers.

Surgical therapies

  • Pulmonary thromboendarterectomy: If present, blood clots in the pulmonary artery may be surgically removed to improve blood flow and lung function.
  • Lung transplantation: Currently, this is the only cure for pulmonary hypertension that is caused by chronic blood clots. Transplantation is reserved for advanced pulmonary hypertension that does not respond to medical therapy. The right side of the heart will generally return to normal after the lung/lungs have been transplanted. About 1,000 lung transplants are performed every year in the United States. Many people are on the waiting list, yet a shortage of donors is the major limiting factor. Your healthcare provider will discuss transplantation if it is an appropriate treatment option for your condition.
  • Heart/lung transplantation: This type of double organ transplant is very rare but is necessary for all patients who have combined lung and left heart failure.

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