Pulmonary hypertension may be considered by your medical provider if you are feeling short of breath, your ability to exercise has decreased, or you are having increased lightheadedness, dizziness, fatigue, or swelling.
Many different tests may suggest the possibility of PH but cannot conclusively make the diagnosis. If PH is suspected based on these tests, further testing should be performed to confirm the diagnosis..
- Electrocardiogram (EKG/ECG): This checks the electrical impulses of the heart through electrodes that are attached to the skin. Certain patterns on the EKG can suggest PH, although it cannot definitively rule in or out PH.
Chest x-ray or chest CT scan of the lungs: Enlarged pulmonary arteries, which can be seen in PH, can sometimes be noted on CT or x-ray imaging of the chest. Other contributing pulmonary diseases, such as emphysema, interstitial lung disease, or chronic blood clots can also be seen on these tests.
Pulmonary Function Testing: This test measures how much air your lungs can hold and how much air moves in and out of your lungs. One portion of this test measures how efficiently the lungs transfer the oxygen you breathe into the blood vessels (called the diffusing capacity or DLCO), which may be low in PH. By showing abnormal patterns (such as obstruction or restriction), pulmonary function testing can help identify if you have a lung disease (e.g. COPD) causing your PH.
Cardiopulmonary exercise testing (CPET): This is a specialized exercise test performed and interpreted only at certain centers to determine the cause of exercise intolerance. Certain patterns in the CPET results can be seen in PH.
Echocardiogram: This is a detailed ultrasound of the heart, which provides a picture of the heart's size and pumping function. The echocardiogram can estimate the pressures in the lung arteries; however, it may either over or underestimate the true pressure. Other signs of PH are enlarged right-sided chambers of the heart and decreased pumping function of the right side of the heart. This test is also useful to see whether there is significant left heart disease (e.g. weak pump, stiff heart, or valve problems) that is contributing to or causing PH. Most people who are told they have PH are told so because of the results of this test.
Sleep study or nocturnal oximetry: This test assesses how well you sleep and whether your oxygen levels drop at night. It screens for sleep disorders, like sleep apnea, which can cause or exacerbate PH.
Nuclear Ventilation/Perfusion Scan (V/Q Scan): This is a specialized chest image in which a small dose of radioactive material is breathed in while another dose is injected into the blood. It allows for the identification of the presence of new or old blood clots in the lungs.
Cardiac MRI: This test provides pictures of the heart that can be used to look for abnormal heart structures that may lead to PH. It can also provide information on the function of your heart and the severity of PH.
Right heart catheterization: The only test that confirms PH is a right heart catheterization. During this test, a long thin plastic tube is passed from a vein (inserted in the neck, arm, or groin) into the heart and lung arteries to directly measure the pressures from inside the lungs. This procedure sometimes requires sedation, and it is often performed as an outpatient procedure. Depending on the results, you may be tested with 100% oxygen and/or a drug called nitric oxide, epoprostenol, or adenosine during the procedure. This so-called ‘vasodilator challenge’ can help guide what therapies may work best. ..
The right heart catheterization confirms whether PH is present and whether it is caused by increased pressures from the left side of the heart or by changes in the lungs and lung blood vessels.
When it is determined that the problem is in the blood vessels within the lung, this is called precapillary PH. There are a number of different tests used to determine the cause of precapillary PH, and provide information on the severity of the condition.
Bloodwork: Your physician will check a variety of blood tests to look for potential causes of PH (liver function, HIV, drug screen, autoimmune labs, thyroid function) as well as other tests to gauge baseline levels of kidney function, blood counts,, and a test called brain natriuretic peptide (BNP) which helps assess the “stretch” on the heart muscles and is used to monitor response to treatment.
Genetic testing: If no underlying cause is detected on your evaluation or there is concern for family history of PH, genetic testing may be part of your diagnostic workup. The most common mutation is in the BMPR2 gene. The cost of testing may be covered by insurance. For more information on genetic testing, click here.
