Diagnosis

Pulmonary hypertension is 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 are similar to those of other heart and lung conditions.

To diagnose pulmonary hypertension, a doctor will perform a physical exam and review any signs and symptoms. You'll likely be asked questions about your medical and family history.

Blood and imaging tests done to help diagnose pulmonary hypertension may include:

• Blood tests. Blood tests can help determine the cause of pulmonary hypertension or detect signs of complications.
• Chest X-ray. A chest X-ray creates pictures of the heart, lungs and chest. It can show enlargement of the right ventricle or the pulmonary arteries. A chest X-ray may also be used to check for other lung conditions that can cause pulmonary hypertension.
• Electrocardiogram (ECG). This noninvasive test shows the heart's electrical patterns and can detect abnormal heartbeats. An ECG may also reveal signs of right ventricle enlargement or strain.

• Echocardiogram. Sound waves are used to create moving images of the beating heart. An echocardiogram lets a doctor see how well the heart and its valves are working. It can show the size and thickness of the right ventricle and the pressure in the pulmonary arteries.

  Sometimes, an echocardiogram is done while exercising on a stationary bike or treadmill to understand how well the heart works during activity. If you have this test, you may be asked to wear a mask that checks how well the heart and lungs use oxygen and carbon dioxide.

  An echocardiogram may also be done after diagnosis to determine how well treatments are working.

• Right heart catheterization. If an echocardiogram reveals pulmonary hypertension, you'll likely have a right heart catheterization to confirm the diagnosis.

  During this procedure, a cardiologist places a thin, flexible tube (catheter) into a vein, usually in the groin. The catheter is gently guided into the right ventricle and pulmonary artery.

  Right heart catheterization allows a doctor to directly measure the pressure in the main pulmonary arteries and right ventricle of the heart. The test may also be used to determine how well pulmonary hypertension medications are working.

If you're diagnosed with pulmonary hypertension, your doctor might also order one or more of the following tests to check the condition of your lungs and pulmonary arteries and further determine the cause of the disease:

• Computerized tomography (CT). This imaging test creates cross-sectional pictures of the bones, blood vessels and soft tissues inside the body. A CT scan can show the size of the heart and any blockages in the pulmonary arteries. This test may be used to look more closely for lung diseases that might lead to pulmonary hypertension such as COPD or pulmonary fibrosis. A dye (contrast material) may be injected into a blood vessel before the CT scan to help the arteries show up more clearly on the images.
• Magnetic resonance imaging (MRI). An MRI scan uses a magnetic field and pulses of radio wave energy to make pictures of the body. A doctor may order this test to see blood flow in the pulmonary arteries and determine how well the right ventricle is working.
• Lung (pulmonary) function test. This noninvasive test measures how much air the lungs can hold and the airflow in and out of the lungs. The test involves blowing into an instrument called a spirometer.
• Sleep study (polysomnogram). This test measures brain activity, heart rate, blood pressure, oxygen levels and other factors during sleep. It can help diagnose a sleep disorder such as obstructive sleep apnea, which can cause pulmonary hypertension.
• Ventilation/perfusion (V/Q) scan. In this test, a tracer is injected into a vein in the arm. The tracer shows blood flow and airflow to the lungs. A V/Q scan can determine whether blood clots are causing symptoms of pulmonary hypertension.
• Open-lung biopsy. Rarely, a doctor might recommend an open-lung biopsy to check for a possible cause of pulmonary hypertension. An open-lung biopsy is a type of surgery in which a small sample of tissue is removed from the lungs.

Genetic testing

If a family member has had pulmonary hypertension, screening for genes that are linked with disease may be recommended. If you test positive, your doctor might recommend that other family members also be screened.

Pulmonary hypertension severity classification

Once a diagnosis of pulmonary hypertension is confirmed, a doctor will classify the severity of the disease into one of following categories:

• Class I. Pulmonary hypertension is diagnosed, but there are no symptoms during activity.
• Class II. There are no symptoms at rest, but symptoms such as fatigue, shortness of breath or chest pain occur with activity.
• Class III. It's comfortable at rest, but symptoms occur during physical activity.
• Class IV. Symptoms occur at rest and during physical activity.