Diagnosis

Pulmonary hypertension may be considered by your medical provider if you are feeling more short of breath, your ability to exercise has decreased or you are having increased dizziness, fatigue or swelling.

Many different tests that may suggest the possibility of pulmonary hypertension but cannot conclusively make the diagnosis. If pulmonary hypertension is suspected based on these tests, you should have further testing performed.

1. Electrocardiogram (EKG/ECG): Electrocardiogram (EKG/ECG): This checks the electrical impulses of the heart through electrodes which are attached to the skin. Certain patterns on the EKG can be seen in pulmonary hypertension, although none can definitively rule in or out PH.


2. Chest x-ray or CT scan of the lungs: Enlarged pulmonary arteries, which can be seen in pulmonary hypertension, can sometimes be noted on these imaging studies. Additionally, other contributing pulmonary diseases such as emphysema or interstitial lung disease can be seen on these tests.


3. 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) may be low in pulmonary hypertension.


4. 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 pulmonary hypertension.


5. Echocardiogram: This is a detailed ultrasound of the heart, which provides a picture of the general size and pumping function of the heart. Most people who are told they have pulmonary hypertension are told so because of the results of this test. The echocardiogram can provide an estimate of the pressures in the lungs; however, it may either over or underestimate the true pressure. Other signs on an echocardiogram that you might have pulmonary hypertension are an enlarged right-sided chamber 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 disease on the left side of the heart such as a weak pump, stiff heart, or valve disease.


6. Sleep study or nocturnal oximetry: This is used to assess how well you are sleeping, and if your oxygen levels drop at night. This test screens for sleep disorders, like sleep apnea, that can cause or exacerbate pulmonary hypertension.


7. Nuclear Ventilation/Perfusion Scan (V/Q Scan): This is a specialized chest image where a small dose of radioactive material is breathed in while another dose is injected into the blood, allowing us to identify the presence of new or old blood clots in the lungs.


8. Cardiac MRI: This test can provide pictures of the heart that can be used to look for abnormal structures that can lead to pulmonary hypertension. It can also be used to provide information on the function of your heart and the severity of pulmonary hypertension.


9. Right heart catheterization: The only test that will confirm if you have pulmonary hypertension is a right heart catheterization, which directly measures the pressure in your lungs. This procedure sometimes requires sedation and it most often is performed as an outpatient procedure. After administering a local anesthetic (lidocaine) in the skin, the doctor will place a large IV in the vein in the neck, arm, or groin. They will then pass a long, a thin, plastic catheter through that IV into the chambers of your heart and capture measurements in the right-sided chambers and the pulmonary artery. Depending on the numbers, you may be asked to breathe in 100% oxygen and/or a drug called nitric oxide or receive medication such as epoprostenol or adenosine to see if that intervention helps reduce the pressure in the lungs.
   
   The right heart catheterization confirms if pulmonary hypertension is present and if it is caused by increased pressures from the left side of the heart or if it is coming from changes in the lungs and lung blood vessels.
   
   If it is determined to be from the lungs it is oftentimes referred to as precapillary pulmonary hypertension. There are a number of different tests used to figure out the cause of precapillary pulmonary hypertension, as well as provide information on the severity of the condition.


10. Bloodwork: Your physician will check a variety of blood tests to look for potential causes of pulmonary hypertension (liver function, HIV, drug screen, autoimmune labs, thyroid function) as well as other tests to gauge baseline levels of kidney function, complete blood counts of your heart health, 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.


11. Genetic testing: If no underlying cause is detected on your evaluation or there is concern for a family history of your of pulmonary hypertension, 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.


12. Pulmonary Function Test: This can help identify if you have lung disease causing your pulmonary hypertension and can show changes that are seen in lung diseases like chronic obstructive pulmonary disease or pulmonary fibrosis.


13. CT Scan: This study provides a high resolution picture of the lungs and can also help identify underlying lung diseases.