Symptoms, Diagnosis and Treatment



The signs and symptoms of pulmonary hypertension might not be noticeable for months or even years. As the disease progresses, symptoms become worse. Pulmonary hypertension symptoms include:

  • Shortness of breath
  • Fatigue
  • Dizziness or fainting spells
  • Chest pressure or pain
  • Swelling in your ankles and legs
  • Bluish color to your lips and skin (cyanosis)
  • Racing pulse or heart palpitations

Severe pulmonary hypertension can lead to heart failure. Currently, there is no cure for pulmonary hypertension, but medications are available to help treat the disease and manage symptoms.






Pulmonary hypertension can be hard to diagnose early because it's not often detected during a routine physical exam. Even when the condition is more advanced, its signs and symptoms resemble those of other more common heart and lung conditions. Many people with pulmonary hypertension are not diagnosed until the disease has become more advanced.


To make an accurate diagnosis, your doctor may review your medical and family history, discuss your signs and symptoms and conduct a physical examination. One of the following tests may determine the severity of your condition as well as find out the cause of your condition.


Tests may include:


  • Cardiac Catheterization: This is a procedure that is used to diagnose and treat cardiovascular conditions. This test uses a thin, flexible tube called a catheter that is inserted into the heart and lungs through blood vessels to measure the pressure in the arteries of the lung and assess heart function.
  • Chest X-ray: A chest X-ray can show images of your heart, lungs and chest and can show enlargement of the right ventricle of the heart or the pulmonary arteries, which can occur in pulmonary hypertension. This test can also be used to identify other conditions that may be causing pulmonary hypertension.
  • Computer Tomography (CT) Scan: A CT scanner takes a series of pictures inside your body while you rest on a scanning table, producing a series of detailed images, many of which would not show up on conventional X-rays. For some scans, an IV contrast will be injected into your vein to outline blood vessels or organs so that they can be seen more easily.
  • Echocardiogram: An echocardiogram (also called an echo) is a type of ultrasound test that uses high-pitched sound waves that are sent through a device called a transducer. The device picks up echoes of the sound waves as they bounce off the different parts of your heart. These echoes are turned into moving pictures of your heart that can be seen on a video screen.
  • Electrocardiogram (ECG/EKG): This test shows your heart's electrical patterns and can detect abnormal rhythms. Doctors may also be able to see signs of right heart enlargement or strain.
  • Six-Minute Walk Test: This test evaluates the capacity for exercise including the amount of feet walked in 6 minutes.  It also evaluates oxygen levels at rest and while walking to determine if supplemental oxygen is needed.
  • Nuclear Medicine Lung Scan: A lung scan is a type of nuclear scanning test. It is most often used to find a blood clot in the lungs (pulmonary embolism). Blood clots prevent normal blood flow in the lung.
    • Two types of lung scans are usually done together:
      • Ventilation scan: During this scan, you inhale a radioactive tracer gas or mist. Pictures from this scan can show areas of the lungs that are not getting enough air or that hold too much air.
      • Perfusion scan: During this scan, a radioactive tracer is injected into a vein in your arm. It travels through your blood and into the lungs. Pictures from this scan can show areas of the lungs that are not getting enough blood.
    • If the lungs are working as they should, the results of the two scans will match, meaning the blood and air both get into all of the regions of the lung.  If the scan results do not match, you may have a blood clot in the lung.
  • Pulmonary Function Test: This test measures how much air your lungs can hold, and the airflow in and out of your lungs. During the test, you will blow into a simple instrument called a spirometer.
  • Blood Work: Initial evaluation includes electrolyte levels, kidney function, liver function, blood counts for anemia, heart enzyme, HIV test and lupus/rheumatoid arthritis tests.





Although there is no cure for pulmonary hypertension, there are many treatment options available that can improve the quality of life for people living with pulmonary hypertension.


Pulmonary hypertension is first treated with medications. These medications can be used alone or in combination with another medication, and may:

  • Lower blood pressure
  • Open blood vessels
  • Help the heart pump blood more effectively
  • Reduce fluid throughout the body

If patients are found to have low oxygen levels, supplemental oxygen may prescribed at rest, with exertion and/or while sleeping.

For patients who have pulmonary hypertension due to blood clots in the lungs, your doctor may recommend evaluation for surgical removal of the clots. Blood thinner medications are also recommended.


Patients with severe pulmonary hypertension who do not respond to medical treatment may require a double lung transplant. During a lung transplant the diseased lungs are replaced with donor lungs.