What is pulmonary hypertension?

Pulmonary hypertension (PH) is a type of high blood pressure that affects the arteries of the lungs and the right side of the heart. These are the blood vessels that carry oxygen-poor blood from your heart to your lung. The disrupted flow of blood through your heart and lungs makes the right side of the heart work harder than normal.

Over time, PH damages your heart and causes problems throughout your body. Early diagnosis and treatment can help you enjoy a better quality of life. It can be fatal without treatment.

Alternative names

Pulmonary arterial hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Cor pulmonale - pulmonary hypertension

What causes pulmonary hypertension?

Pulmonary hypertension can develop on its own or be caused by another disease or condition. It commonly affects people who have heart or lung conditions.

Changes in the cells that line the lung arteries can cause the artery walls to become narrow, stiff or swollen. As a result, these changes may slow down or stop blood flow through the lungs.

Pulmonary hypertension is classified into five groups by the World Health Organization (WHO), depending on the cause:

• Group 1: Pulmonary arterial hypertension (PAH)
  • Unknown cause, called idiopathic pulmonary arterial hypertension
  • Changes in gene passed through families
  • Autoimmune or connective tissue diseases
  • Use of medicines or illegal drugs
  • Heart problems present at birth or other health conditions
• Group 2:
  • Most common form of pulmonary hypertension
  • Left heart failure
  • Left-sided heart valve disease such as mitral valve or aortic valve diseases
• Group 3:
  • Certain lung problems such as pulmonary fibrosis, chronic obstructive pulmonary disease (COPD) or sleep apnea
• Group 4:
  • Chronic blood clots (Chronic thromboembolic pulmonary hypertension)
  • Tumors that block the pulmonary artery
• Group 5:
  • Blood disorders
  • Sarcoidosis
  • Inflammatory disorders
  • Metabolic disorders
  • Kidney disease

Symptoms

The symptoms of PH tend to develop slowly and get worse as the disease progresses.

Shortness of breath or lightheadedness during activity is often the first symptom. Fast heart rate, or palpitations, may also be present.

Other symptoms include:

• Ankle and leg swelling
• Bluish color of the lips or skin (cyanosis)
• Chest pain or pressure, most often in the front of the chest
• Dizziness or fainting spells
• Fatigue
• Increased abdominal size
• Weakness

People with pulmonary hypertension often have symptoms that come and go. They report good days and bad days.

Exams and tests

Your health care provider will perform a physical exam and ask about your symptoms. The exam may find:

• Abnormal heart sounds
• Feeling of a pulse over the breastbone
• Heart murmur on the right side of the heart
• Larger-than-normal veins in the neck
• Leg swelling
• Liver and spleen swelling
• Normal breath sounds if pulmonary hypertension is idiopathic or due to congenital heart disease
• Abnormal breath sounds if pulmonary hypertension is from other lung disease

In the early stages of the disease, the exam may be normal or almost normal. The condition may take several months to diagnose. Asthma and other diseases may cause similar symptoms and must be ruled out.

Tests that may be ordered include:

• Blood tests
• Cardiac catheterization
• Chest x-ray
• CT scan of the chest
• Echocardiogram
• ECG
• Lung function tests
• Nuclear lung scan
• Pulmonary arteriogram
• Six-minute walk test
• Sleep study
• Tests to check for autoimmune problems
• Genetic test

Treatment

There is no cure for PAH. The goal of pulmonary hypertension treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension such as obstructive sleep apnea, lung conditions and heart valve problems.

Many treatment options for pulmonary arterial hypertension are available. If you are prescribed medicines, they may be taken by mouth (oral), received through the vein (intravenous, or IV), injected into the stomach or thigh (subcutaneous or intramuscular) or breathed in (inhaled).

Your provider will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you are responding to the medicine.

Other treatments may include:

• Blood thinners to reduce the risk of blood clots, especially for some forms of pulmonary hypertension due to blood clots
• Oxygen therapy at home
• Lung transplant or, in some cases, heart-lung transplant if medicines do not work

Other tips to follow:

• Avoid pregnancy: PH can be life threatening for the mother and developing baby
• Avoid heavy physical activities and lifting
• Avoid traveling to high altitudes
• Get a yearly flu vaccine as well as other vaccines such as the pneumonia vaccine and the COVID vaccine
• Stop smoking

When to contact a medical professional

Contact your provider if:

• You begin to develop shortness of breath when you are active
• Shortness of breath gets worse
• You develop chest pain
• You develop other symptoms
• You feel lightheaded or dizzy
• You have persistent palpitations

Patient information

• The Pulmonary Hypertension Association