Pulmonary Hypertension is considered by doctors to be a rare and elusive disease. The rarity of the disease and its common symptoms often lead to a late diagnosis. The first symptoms are general and usually nonspecific, with the person experiencing fatigue. Additionally, even if the doctor suspects a medical condition, PPH is diagnosed after a long process of elimination. A confirmed PPH diagnosis will require a series of invasive tests to conclusively confirm the origin and cause of the elevated pulmonary artery pressure. Thus, doctors and patients usually remain unaware of the disease until the symptoms worsen, leading to a delayed diagnosis. In fact, the average time from the onset of the first symptoms to an accurate diagnosis is 2 years.

Today, by far the biggest risk factor is a history of diet drug use including Fen Phen, Pondimin and Redux.

If you have taken diet drugs for any length of time and experience dyspnea (shortness of breath), fainting (syncope), swollen ankles, rapid pulse, increased heart rate, chest discomfort and pain, dizziness, general weakness and fatigue over weeks or months you should see a doctor and describe your concerns. In general, women display more of the symptoms than men. Although both sexes are at risk, PPH occurs more often in younger woman for unknown reasons.

Testing for PPH

The disease acts on both lung and heart functions and a diagnosis is made by excluding all other possible explanations. Thus a PPH diagnosis is made after high blood pressure in the lungs is found, that cannot be attributed to any other cause or causes.
The initial tests are designed to evaluate the overall performance of the lungs and heart. If PPH is suspected the following diagnostic tests will be done:

Chest radiograph (X-Rays): These will generally be the first test a doctor orders. X Rays can reveal a wide variety of more common lung disorders and the films can rule out a number of causes of elevated pulmonary artery pressure. Unfortunately, X Rays do not reveal the cause or etiology of the pulmonary hypertension.

Echocardiogram: This test is used to look at the heart using sound waves to create an image. In PPH diagnosis, the test is used to look at the right and left ventricles and how they are functioning. A doctor can use this test to estimate pulmonary arterial pressure. An Echocardiogram can also rule out congenital anomalies, other heart conditions and valvular disease.

Ventilation Perfusion Lung Scan: A type of high-resolution chest scan. Doctors may order this test to rule out alternative causes such as interstitial lung disease caused by smoking, asbestos or other pollutants. It may also be used to rule out blood clots and other lung diseases.

Pulmonary angiogram: This test may be needed definitively rule out blood clotting ( thromboembolic disease). This test is invasive and may be high risk in people with high pulmonary arterial pressures and is usually used only if the ventilation-perfusion scan is inconclusive. This test involves injecting a dye into the lung arteries. It provides the best image of what is going on and gives doctors a detailed picture of the blood vessels in the lungs and how blood is flowing through them.

Electrocardiogram (ECG / EKG): This is basically an electrical recording of the heart.
People with PPH, may have irregular EKG patterns, because the disease damages the right ventricle, and may result in atrial enlargement, which doctors can measure in the sound waves. However, some people with PPH have regular ECG findings; so ECG testing cannot eliminate a PPH diagnosis.

Definitive Diagnosis

Once all other possible explanations have been ruled out, a doctor may order a Cardiac Catheterization.

Cardiac Catheterization (Right Heart):
This test is used to make a definitive diagnosis by direct examination of the pressure inside of the heart. A doctor places a catheter, a thin plastic tube into an artery or vein usually in an arm or leg. After it is inserted, the catheter is pushed into the heart and coronary arteries. This test measures the blood pressure in the heart and the amount of oxygen in the blood. The test can also measure the pumping ability of the heart muscle. In PPH patients the doctors will look at the right ventricle for signs of damage. The doctor is able to do this by measuring the volume of blood the heart pumps out of the right side of the heart with each heartbeat.

After a right heart cardiac catheterization, a positive PPH diagnosis may be made.

If you or a loved one has been diagnosed with PPH and have used Fen Phen or related diet drugs, you may be entitled to substantial compensation.
Contact us to find out more about your medical and legal rights.

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