What is Pulmonary Arterial Hypertension (PAH)?

PAH is high blood pressure that occurs in the pulmonary arteries, the arteries that carry blood from the heart to the lungs. With PAH, the blood vessels that branch off from the arteries have tightened and narrowed, making it difficult for the blood to flow to the lungs and gather oxygen. When this happens, the heart has to work harder to push the blood through the vessels, resulting in high blood pressure in the arteries.

Though PAH occurs rarely, it is a serious disease; left untreated, damage to the heart can occur, which can lead to heart failure. That is why early diagnosis is so important.

What are the symptoms of PAH?

Common symptoms of PAH include:

• Breathlessness, or feeling short of breath
• Chronic fatigue, or always feeling tired
• Chest pain, especially when you are doing some physical activity
• Swelling, particularly in the legs and ankles
• Dizziness, often upon climbing stairs or standing up, though sometimes when just sitting
• Fainting, or passing out

Because these symptoms could be due to other diseases, it is important that a correct diagnosis is made.

How is PAH diagnosed?

Your physician will order tests to confirm that you have PAH, and also to rule out other causes of your symptoms. Tests which may be performed include:

• Chest x-ray
• Laboratory tests, such as blood tests, for example to check the oxygen level in your blood, or to determine if other conditions related to PAH may be present
• Doppler Echocardiogram, which uses sound waves to determine if certain abnormalities related to the heart are present which might indicate the presence of PAH
• Six minute walk test, to determine limitations in functioning
• Pulmonary function tests, to determine if certain kinds of lung diseases are present
• Cat scan of the chest, to evaluate blood clots in the lungs

Based on your medical history and the results of tests performed, your physician may order other diagnostic procedures to assist in the diagnosis. To confirm a diagnosis of PAH, a right heart cardiac catheterization, an accurate and frequently-used procedure, might be ordered.

What causes PAH?

In some incidences, the cause of PAH is unknown. Some persons, however, have a genetic predisposition to developing the disease, while for others PAH may be associated with other conditions. Other conditions incude, but are not limited to:

• Connective tissue diseases such as lupus and scleroderma
• Congenital heart and lung diseases, for example heart defects
• Portal hypertension, or high blood pressure in the liver, for which a common cause is cirrhosis
• HIV Infection
• Drugs or toxins, among which are street drugs like cocaine, or diet pills that work by suppressing the appetitie
• Sickle cell anemia

What is the treatment for PAH?

PAH is a complicated disease and requires multiple approaches to treatment. There are several drug therapies that are used and your physician may also advise you to make certain lifestyle changes, such as diet or physical activity.

Drug therapies may include, but are not limited to:

• Calcium Channel Blockers
• Endothelin Receptor Antagonists
• Nitric Oxide and Arginine
• Phosphodiesterase Inhibitors
• Prostanoids
• Diuretics
• Digoxin
• Oxygen

The various treatment options are aimed at avoiding the serious complications which may result from PAH, thereby imporving your heart function and quality of life. For eligible patients, lung transplantation may be advised if medical therapies do not work.