The following information consists of research funded by the Pulmonary Hypertention Association.  To reach the PHA:  PHAssociation.org

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I hope this information regarding nailing down the proper diagnosis may be helpful to someone suffering with this frequently 'challenging' disease.  Perhaps a portion of the point of the following article is that when assigned a PH diagnosis, punch your way through the occasionally unpleasant medical tests, until you're absolutely sure what you're dealing with...

With my Best Regards.......Reluctant survivor

          Confirming a Diagnosis (PAH)

PH is often not diagnosed in a timely manner because its early symptoms can be confused with those of many other conditions.  Some of the symptoms of PH include:

* Breathlessness or shortness of breath, especially with activity

* Feeling tired all the time

* Lightheadedness, especially when climbing stairs or upon standing up

* Swollen ankles, legs or abdomen

* Chest pain, especially during physical activity

* Fainting.

To establish a diagnosis of PH, a series of tests are performed that show how well a person's heart and lungs are working.  These tests may include:

* Echocardiogram (ultrasound of the heart)

* Electrocardiogram (ECG,a recording of the electrical current in the heart)

* Right heart chatheterization (direct measurement of pressure in the pulmonary arteries and determination of how well the heart is pumping blood to the body)

* Six-minute walk test to assess exercise capacity.

If these tests show that a person definitely he PH, other kinds of diagnostic tests may be ordered to aid in identifying the cause, including:

* Ventilation-perfusion (V/Q) scan to rule out a pulmonary embolism (blood clot in pulmonary arteries)

* A computed tomography (CT or CAT) scan to rule out abnormalities of the lung tissue and, in some cases, to assess for a pulmonary embolism

* A pulmonary function test to rule out diseases of the lung tissue such as emphysema or pulmonary fibrosis

* A formal sleep study to rule out sleep apnea

* Laboratory tests to rule out hepatitis, collagen disease, HIV, or other conditions.

When no cause can be found, the diagnosis is idiopathic PAH.