There is currently no cure for PAH. PAH is a progressive disease (meaning that it gets worse over time).
Often a diagnosis may be delayed for months or even years. Patients with undiagnosed PAH who do not receive
treatment may experience more rapid worsening of symptoms or physical condition than patients
who are on a doctor-prescribed treatment plan.
Early and late symptoms of PAH may be easily confused with those of other conditions, such as
asthma or other lung diseases. That's why a series of tests must be performed to rule out other conditions
and confirm PAH.
Making a diagnosis
Once symptoms are detected, there is an array of diagnostic testing tools that your healthcare
team may want to use. For an accurate diagnosis to be made, certain tests may be performed
either to rule out other conditions and/or to confirm PAH.
| If PAH is suspected, the following tests are often used |
If PAH is present, the tests may indicate |
| Electrocardiogram (ECG) |
May show changes in heart rhythm and structure which may indicate damage to the heart. |
| Chest X-rays |
May show enlarged pulmonary arteries. |
| Doppler Echocardiogram |
May detect abnormalities in pressure and flow rates in vessels of lungs and heart. May show right heart chamber enlargement, cardiac output reduction, and abnormal heart function. |
Confirming a definite diagnosis
A
right-heart catheterization is
the only test to confirm a definite diagnosis of PAH. This test may
also evaluate the severity of PAH by measuring pressure and blood flow changes in the pulmonary
arteries and right side of the heart.
1
It's an invasive procedure—involving the insertion of a thin flexible tube into the right side of
the heart—that takes 1 to 2 hours and often requires sedation. So your healthcare provider may first perform a
number of non invasive tests like the electrocardiogram, chest X-ray, and Doppler echocardiogram
mentioned above.
1
Your healthcare provider might order other kinds of tests to learn more about your condition or to
rule out other diseases. These tests include:
- Lung scan to rule out a pulmonary embolism or lung disease
- Pulmonary function tests to rule out other lung diseases
- Formal sleep study to rule out sleep apnea
- Lab tests to rule out other conditions
Classifying PAH symptoms
Once a diagnosis of PAH has been established, your healthcare provider needs to determine your WHO
Functional Class. This is a rating of your ability to perform ordinary physical activities, such as
walking, without shortness of breath. Placing patients into WHO Functional Classes helps the healthcare
team make decisions about your treatment.
The WHO Functional Class scale is divided into 4 categories and considers how symptoms such as tiredness,
shortness of breath, chest pain or light-headedness limit physical activity.
2 The lower the WHO
Functional Class, the less severe the symptoms of PAH are. One of the goals of treatment is to improve
your Functional Class.
| The WHO Functional Class Scale2 |
Functional Class I:
No limitation in usual physical activity (ordinary activity does not increase symptoms).
|
Functional Class II:
Some limitation in physical activity (no discomfort at rest, but normal activity increases symptoms).
|
Functional Class III:
Significant limitation in physical activity (no discomfort at rest, but less than normal activity
increases symptoms).
|
Functional Class IV:
Symptoms are present with any activity or even at rest.
|
Tracleer is approved for patients with pulmonary arterial hypertension (PAH) WHO Class II, III, IV.
The 6-Minute Walk Test
The
6-Minute Walk Test
is a tool to evaluate exercise capacity. For the test, you walk as far
as possible in 6 minutes. Before, during, and after the test, you will be asked to report how short of breath
you are. If you are diagnosed with PAH, your healthcare provider may ask you to take the 6-MWT before you
begin and several times during your treatment.
IMPORTANT SAFETY INFORMATION
Because of the risks associated with Tracleer, this medication may be prescribed by your doctor only
through the Tracleer Access Program.
Liver damage: Tracleer can cause serious liver damage, including in rare cases liver
failure and, in situations with regular liver testing, unexplained liver cirrhosis. So you must have a
blood test to check your liver function before you start Tracleer and each month after that. Call your
doctor right away if you have any of these symptoms of liver problems: nausea, vomiting, fever, unusual
tiredness, abdominal (stomach area) pain, or yellowing of the skin or the whites of your eyes (jaundice).
Serious birth defects: Tracleer is very likely to cause serious birth defects. It is
important not to be or to become pregnant while taking Tracleer or one month after stopping treatment
and to have a pregnancy test before you start the drug and each month thereafter. It is very important
for you to use reliable forms of birth control while taking Tracleer. Hormonal contraceptives should
not be used alone because they may not be effective in patients taking Tracleer.
Do not take Tracleer with glyburide because it may increase your chance of liver injury. Do
not take Tracleer with cyclosporine-A because it may increase your chance of side effects.
Please see
Additional Safety Information.
Please see full
Prescribing Information, including
BOXED WARNING and
Medication Guide.
REFERENCE:
- McLaughlin VV. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009;53:1573-619.
- Barst, RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(Suppl S):40S–47S.
