Pulmonary Hypertension

When blood pressure in your lungs’ arteries is high, your heart must work harder to get blood to your lungs. The harder your heart works, the more difficult it may be to breathe. Over time, your heart may become weaker, too.

We classify pulmonary hypertension into one of five groups based on its cause:

• Group 1 results from abnormal growth of the inner and middle layers of the arteries in the lungs leading to obstruction of blood flow.  This makes the right side of the heart have to work harder to try to pump the blood through the lungs to the left side of the heart.  This condition can occur on its own but sometimes is related to other conditions such as connective tissues disease such as scleroderma or lupus, liver disease, congenital heart disease, or exposure to some drugs.
• Group 2 is related to problems with the left side of the heart if from weakness, stiffening, or problems with the valves. This is the most common form of pulmonary hypertension.
• Group 3 is linked to other lungs diseases such as interstitial lung disease (pulmonary fibrosis) or chronic obstructive pulmonary disease (COPD).
• Group 4 is for patients with chronic thromboembolic pulmonary hypertension (CTEPH), a rare condition where prior blood clots block blood flow in the lungs, sometimes without the patient even knowing blood clots had occurred in the past. CTEPH causes high blood pressure in the lungs, strains the right side of the heart, and results in symptoms like swelling, fatigue, shortness of breath, and difficulty doing activities.
• Group 5 is due to other causes. Examples include tumors or sickle cell anemia, a condition in which oddly shaped red blood cells block blood flow. 

Symptoms of pulmonary hypertension may include:

• Shortness of breath
• Swelling in the legs
• Dizziness, lightheadedness or a feeling you might faint 

Pulmonary hypertension can be hard to diagnose. The symptoms are similar to many other conditions. If you or your doctor suspect the condition, it’s important to turn to experienced pulmonologists.

Tests that may help diagnose pulmonary hypertension include:

• Blood tests to evaluate organ function and check for a variety of conditions.
• Imaging tests, such as a chest X-ray, CT scan or pulmonary angiography, a test that uses special dye and X-ray to help doctors see your lungs’ blood vessels. Doctors may also order an echocardiogram (an ultrasound of your heart) to see how blood moves from your heart to your lungs or a ventilation-perfusion scan (V/Q scan). A V/Q scan measures how well air and blood flow through your lungs.
• Pulmonary function tests to measure your lungs’ size and ability to move air in and out.
• Right heart catheterization, which involves passing a thin, flexible tube through your blood vessels and into your pulmonary artery, on your heart’s right side. The test allows doctors to measure pressure in your heart and lungs. It’s key to getting an accurate diagnosis of pulmonary hypertension and making decisions about treatment.
• Six-minute walk test to evaluate your breathing during exercise.