What is Pulmonary Hypertension

Pulmonary hypertension is a long-term (chronic) condition in which there is high blood pressure in the arteries in the lungs (pulmonary arteries).

This condition occurs when pulmonary arteries become narrow and tight, making it harder for blood to flow through the lungs.

This in turn makes the heart work harder to pump blood through the lungs, making it harder for you to breathe.

Over time, pulmonary hypertension can weaken and damage the heart muscle, specifically the right side of the heart. Pulmonary hypertension is a serious condition that can be life-threatening.

What are the causes?

This condition may be caused by different medical conditions. It can be categorized by cause into five groups:

• Group 1: Pulmonary hypertension that is caused by abnormal growth of small blood vessels in the lungs (pulmonary arterial hypertension). The abnormal blood vessel growth may have no known cause, or it may be:
  • Passed from parent to child (hereditary).
  • Caused by another disease, such as a connective tissue disease (including lupus or scleroderma), congenital heart disease, liver disease, or HIV.
  • Caused by certain medicines or poisons (toxins).
• Group 2: Pulmonary hypertension that is caused by weakness of the left chamber of the heart (left ventricle) or heart valve disease.
• Group 3: Pulmonary hypertension that is caused by lung disease or low oxygen levels. Causes in this group include:
  • Emphysema or chronic obstructive pulmonary disease (COPD).
  • Untreated sleep apnea.
  • Pulmonary fibrosis.
  • Long-term exposure to high altitudes in certain people who may already be at higher risk for pulmonary hypertension.
• Group 4: Pulmonary hypertension that is caused by blood clots in the lungs (pulmonary emboli).
• Group 5: Other causes of pulmonary hypertension, such as sickle cell anemia, sarcoidosis, tumors pressing on the pulmonary arteries, and various other diseases.

What are the signs or symptoms?

Symptoms of this condition include:

• Shortness of breath. You may notice shortness of breath with:
  • Activity, such as walking.
  • Minimal activity, such as getting dressed.
  • No activity, like when you are sitting still.
• A cough. Sometimes, bloody mucus from the lungs may be coughed up (hemoptysis).
• Tiredness and fatigue.
• Dizziness, lightheadedness, or fainting, especially with physical activity.
• Rapid heartbeat, or feeling your heart flutter or skip a beat (palpitations).
• Veins in the neck getting larger.
• Swelling of the lower legs, abdomen, or both.
• Bluish color of the lips and fingertips.
• Chest pain or tightness in the chest.
• Abdominal pain, especially in the upper abdomen.

How is this diagnosed?

This condition may be diagnosed based on one or more of the following tests:

• Chest X-ray.
• Blood tests.
• CT scan.
• Pulmonary function test. This test measures how much air your lungs can hold. It also tests how well air moves in and out of your lungs.
• 6-minute walk test. This tests how severe your condition is in relation to your activity levels.
• Electrocardiogram (ECG). This test records the electrical impulses of the heart.
• Echocardiogram. This test uses sound waves (ultrasound) to produce an image of the heart.
• Cardiac catheterization. This is a procedure in which a thin tube (catheter) is passed into the pulmonary artery and used to test the pressure in your pulmonary artery and the right side of your heart.
• Lung biopsy. This involves having a procedure to remove a small sample of lung tissue for testing. This may help determine an underlying cause of your pulmonary hypertension.

How is this treated?

There is no cure for this condition, but treatment can help to relieve symptoms and slow the progress of the condition. Treatment may include:

• Cardiac rehabilitation. This is a treatment program that includes exercise training, education, and counseling to help you get stronger and return to an active lifestyle.
• Oxygen therapy.
• Medicines that:
  • Lower blood pressure.
  • Relax (dilate) the pulmonary blood vessels.
  • Help the heart beat more efficiently and pump more blood.
  • Help the body get rid of extra fluid (diuretics).
  • Thin the blood in order to prevent blood clots in the lungs.
• Lung surgery to relieve pressure on the heart, for severe cases that do not respond to medical treatment.
• Heart-lung transplant, or lung transplant. This may be done in very severe cases.

Follow these instructions at home:

Eating and drinking

• Eat a healthy diet that includes plenty of fresh fruits and vegetables, whole grains, and beans.
• Limit your salt (sodium) intake to less than 2,300 mg a day.

Lifestyle

• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Avoid secondhand smoke.

Activity

• Get plenty of rest.
• Exercise as directed. Talk with your health care provider about what type of exercise is safe for you.
• Avoid hot tubs and saunas.
• Avoid high altitudes.

General instructions

• Take over-the-counter and prescription medicines only as told by your health care provider. Do not change or stop medicines without checking with your health care provider.
• Stay up to date on your vaccines, especially yearly flu (influenza) and pneumonia vaccines.
• If you are a woman of child-bearing age, avoid becoming pregnant. Talk with your health care provider about birth control.
• Consider ways to get support for anxiety and stress of living with pulmonary hypertension. Talk with your health care provider about support groups and online resources.
• Use oxygen therapy at home as directed.
• Keep track of your weight. Weight gain could be a sign that your condition is getting worse.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• Your cough gets worse.
• You have more shortness of breath than usual, or you start to have trouble doing activities that you could do before.
• You need to use medicines or oxygen more frequently or in higher dosages than usual.

Get help right away if:

• You have severe shortness of breath.
• You have chest pain or pressure.
• You cough up blood.
• You have swelling of your feet or legs that gets worse.
• You have rapid weight gain over a period of 1–2 days.
• Your medicines or oxygen do not provide relief.

Summary

• Pulmonary hypertension is a chronic condition in which there is high blood pressure in the arteries in the lungs (pulmonary arteries).
• Pulmonary hypertension is a serious condition that can be life-threatening. It can be caused by a variety of illnesses.
• Treatment may involve taking medicines and using oxygen therapy. Severe cases may require surgery or a transplant.