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Diagnostic Tests

How will I be followed/monitored?

How you are followed or monitored will likely depend on how you are doing clinically in terms of your medications and symptoms. If you require adjustments to your medications or close monitoring because of changes in your symptoms, you will be seen more frequently. If you are stable on your medications and not having new symptoms, you will need to go to your doctor less frequently.

In addition, different physicians have different practice styles, but your physician will likely see you in the office on a regular basis (such as every 3 or 6 months) to monitor your symptoms, perform a physical exam, and possibly do a 6-minute walk test. He or she will also periodically use some or all of the tests described in this section to monitor changes in your PAH and follow your response to therapy. As an example, you may have a repeat TTE (or even a right heart catheterization) every year or two to determine how your PA pressures have changed.

One other common way in which many patients are followed and their overall condition is described is called their "Functional Class". This is a four-tiered system that doctors use for describing your overall ability to do activities of daily living. The rating system is based on a similar system used originally by the New York Heart Association (and therefore sometimes abbreviated as NYHA Class I-IV) to classify patients with another heart problem called congestive heart failure (when the pumping function of the heart muscle is weakened). There are no strict criteria (such as the distance you walk on a six-minute walk test) for each of the levels, but generally we think of patients who are Class I as being relatively or nearly symptom-free with activities of daily living and exertion. Class II patients tend to get symptoms with maximal or near maximal activity. Class III patients get symptoms of their PAH with more routine activities of daily living, and Class IV patients generally get symptoms with almost any activity or even at rest.

You may also need to undergo testing when you have new symptoms or worsening of previous symptoms. It will always be important to make sure that new or increased shortness of breath or other respiratory symptoms are not due to problems other than PAH. For example, even though you have pulmonary arterial hypertension, other problems such as pneumonia or bronchitis can occur and cause symptoms. In this case, a chest x-ray or chest CT scan may be obtained to rule out other causes of shortness of breath.

As mentioned previously, these tests are also a very important way to monitor your response to a change in medical therapy. For example, if you were started on a new medication, your physician would likely want to follow your response in terms of how you felt, how your physical exam may have changed, and also how your 6-minute walk test distance or PA pressures (as measured by TTE) may have changed as well.