Young woman sitting on a couch with her hand on her chest
With pulmonary hypertension, the arteries in your lungs become narrow or blocked.

What You Should Know About Pulmonary Hypertension

If you have been diagnosed with pulmonary arterial hypertension (PAH), you should be aware that it is a life-threatening condition.

Over time, this condition may get worse – but with treatment, you can have a good quality of life despite the condition. Many people can get back to their normal activities if they follow the treatment guidelines.

If you have pulmonary hypertension, it means you have high blood pressure in arteries that connect your heart and lungs.

Pulmonary hypertension is not the same as high blood pressure. However, it bears some similarities.

What Causes Pulmonary Hypertension?

Researchers believe that family history or genetics may play a role, or there may be another condition that's causing the problem, including:

  • Diet drug fen-phen increases risk 23-fold, possibly years later
  • Rheumatoid arthritis, lupus, scleroderma and other autoimmune diseases
  • Smoking and emphysema, chronic bronchitis, or pulmonary fibrosis
  • Obesity and sleep apnea
  • Heart defect you have had since birth
  • Family history of congestive heart failure
  • Liver disease (cirrhosis of the liver)
  • Illegal drug use (cocaine or methamphetamine)
  • Living at a high altitude (low-oxygen condition)

Why Is Pulmonary Hypertension So Serious?

With pulmonary hypertension, the arteries in your lungs become narrow or blocked.

These changes can reduce blood flow through the blood vessels, or block blood flow entirely. This blockage in blood flow raises blood pressure in the pulmonary arteries.

Your heart will have to work harder to pump blood through the arteries. Over time, the heart muscle gets weak and may lead to heart failure.

What Are the Signs of Pulmonary Hypertension?

In the early stages, there often aren't symptoms that anything is wrong. Years might go by before you realize you have a medical condition. You may find you are short of breath when active. Over time, you feel short of breath even when you are resting.

Additional symptoms may include:

  • Dizziness
  • Fainting
  • Chest pain
  • Fatigue
  • Swelling in ankles and legs
  • Bluish skin and lips
  • Racing pulse
  • Heart palpitations

Complications of Pulmonary Hypertension

When your heart's right ventricle becomes enlarged, it has to pump harder to move blood through the narrowed pulmonary arteries and may lead to heart enlargement and heart failure.

At first, the heart will try to adapt, so the heart's walls get thicker and expand to hold more blood. However, over time the heart will suffer from the strain.

You may develop:

  • Blood clots
  • Irregular heartbeat (arrhythmia)
  • Heart palpitations
  • Dizziness or fainting
  • Coughing up blood

These are potentially fatal complications.

How Is Pulmonary Hypertension Treated?

Every patient has a different treatment based on the underlying cause.

Treatment may include the following:

  • Medications
  • Change lifestyle and diet
  • Surgery
You May Also Like

Your doctor has medications to treat pulmonary hypertension.

The choice will depend on the severity of your condition, if it worsens and how well you tolerate the drug because you have low levels of oxygen in your blood, a tendency to form blood clots, and water retention in tissues, your treatments will address those problems.

Therapies and medications may include:

  • Oxygen therapy – Replaces oxygen in your blood.
  • Anticoagulants (blood thinners) – Reduces blood clot formation and improve blood flow in arteries and blood vessels.
  • Diuretics (water pills) – Removes extra fluid from tissues and bloodstream, to ease swelling and improve breathing. However, increases urination.
  • Potassium – Replaces potassium lost when taking diuretics.
  • Inotropic agents – Improves the heart's pumping ability.
  • Vasodilators – Lowers pulmonary blood pressure and can improve the heart's pumping ability.
  • Medications to prevent narrowing of lung blood vessels.
  • Medication to dilate (widen) pulmonary arteries and prevent the formation of blood clots.
  • Medication to promote dilation of the pulmonary arteries.

Dietary Changes

By changing your diet and starting to exercise, you can help improve your condition. Limiting salt will help reduce fluid retention. Limiting calories – and increasing exercise – will help lower your weight.

  • Eat foods high in potassium (bananas, oranges, dried fruits, bananas, oranges) and magnesium (peanuts, broccoli)
  • Limit total daily calories to lose weight
  • Limit refined sugar, cholesterol, saturated fats
  • Eat high-fiber foods – vegetables, fruits, whole grains

Reduce sodium/salt intake:

  • Reduce sodium in canned/packaged food
  • Avoid table salt and seasoning salt
  • Avoid canned, salted, smoked and cured meat products
  • Remove fast food

Monitor the fluids you drink. Cut back on fluids if shortness of breath gets worse or swelling in ankles starts to occur.

Lifestyle Changes

  • Monitor your weight
  • Lose weight if necessary
  • See your doctor annually

Activity Guidelines

  • Don't lift anything over 20 pounds
  • Walk to keep muscles strong and improve blood circulation
  • Don't risk more strenuous aerobic activities (causing shortness of breath, chest pain or dizziness)
  • Don't smoke or use chew tobacco
  • Avoid alcohol intake

If you experience symptoms of pulmonary hypertension, contact your doctor right away, and always stay in close contact with your doctor.

Surgical Treatment

If blood clots have formed in the pulmonary artery, your doctor may advise having a surgery called pulmonary thromboendarterectomy. In this procedure, the blood clots are surgically removed to improve blood flow and lung function.

A lung transplant is the only cure for advanced pulmonary hypertension when medications have not improved the condition sufficiently. Either one or both lungs will be replaced, which will allow the heart to return to normal function. Lung transplant is a relatively rare procedure, although very successful.

A shortage of donors results in a long waiting list. Heart and lung transplantation is very rare but is necessary when lungs and heart have failed.