What are the symptoms of Pulmonary Hypertension?

The initial onset is characterized by fairly general symptoms such as shortness of breath, tiredness, and fainting. Often the patient describes themselves as "being out of shape," which often delays treatment. As the disease progresses there may be bluish lips and skin, swelling of the ankles/lower legs and chest pain. Patients often complain of a "racing heart". The more acute the symptoms, the more advanced the stage of PH. Early detection is important and people with a family history of PH should routinely be checked.

How is PH diagnosed?

PH is a rare disease and is usually found only after a physician has excluded every other possible explanation of the symptoms. The diagnosis is usually made in the process of trying to rule out other explanations. Most PH cases are are secondary pulmonary hypertension-that is the condition is related to another disease process (more info>>)

Tests that are done include:
Echocardiogram
Cardiac Catherization
Pulmonary Function Tests
Lung Scan (ventilation-perfusion or "VQ" scan)
CAT Scan
Blood Tests
Sleep Study
MRI scan

The MRI is very important, replacing many of the other tests and scans. However, it is not always used because of the scarcity of the machines and the high costs of the scans. It is the most important tool for diagnosis of PH.

What is a PPH Functional Classification?

Once PPH is diagnosed, doctors classify the disease based on how well a patient can perform daily life tasks and functions. Created by the New York Heart Association, there are four classes:

Class 1- Patients who have no symptoms such as tiredness, palpitations, shortness of breath, chest pains.

Class 2- Patients who are comfortable when resting but experience tiredness, palpitations, shortness of breath, chest pains when performing basic life tasks.

Class 3- Patients who are comfortable when resting but experience tiredness, palpitations, shortness of breath, chest pains with less than ordinary life tasks.

Class 4- Patients who experience tiredness, palpitations, shortness of breath, chest pains even at rest.


Once diagnosed with either PH or PPH, what is the life-expectancy and prognosis?

In the early 1990's patients could expect a three year life expectancy. However, the development of Flolan treatment in 1994 provided a viable way to treat the disease and raised life expectancy to 5 or more years. These prognosis are not good and much work needs to be done in developing a cure and effective treatments for PH.

 

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