What is Pulmonary Hypertension (PH)?
Pulmonary hypertension (PH) is a rare blood disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life threatening. It is a disease that affects people of all ages and ethnic backgrounds.
What are the symptoms of PH?
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:
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. Shortness of breath (dyspnea) especially with activity
. Fatigue
. Chest pain (angina)
. Dizziness
. Fainting spells (syncope)
. Ankle swelling (edema)
. Heart throbbing (palpitations) |
What are different types of PH?
PH occurs due to disease in the large or the small pulmonary arteries, but in nearly all cases, the small pulmonary arteries are eventually affected. In 2003, World Health Organization (WHO) sponsored committee proposed that the disease be divided into five categories
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1. Pulmonary arterial hypertension (PAH)
2. Pulmonary venous hypertension (PVH)
3. Pulmonary hypertension associated with disorders of respiratory systems and/orhypoxia
4. Pulmonary hypertension associated with thrombotic and/or embolic disease
5. Miscellaneous |
What tests are done to diagnose PH?
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:
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. Echocardiogram (ultrasound of the heart)
. Electrocardiogram (ECG, a recording of the electrical current in the heart)
. Right heart catheterization (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 has PH, other kinds of diagnostic tests may be ordered to aid in identifying the cause, including:
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. 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 disease in 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 |
What are available treatments for PH?
You may be treated with different types of medication alone or combined with others, such as:
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. Anticoagulants to prevent blood clots in the lungs
. Calcium channel blockers to relieve constriction in the pulmonary arteries
. Digoxin which may help the heart pump blood more effectively
. Diuretics to reduce fluid in the body
. Inhaled oxygen to increase the oxygen level in the blood and provide better oxygenation to the heart and other organs
. Bosentan (brand name Tracleer®) is an oral endothelin receptor
antagonist, or ERA. Bosentan is administered in simple pill form.
. Ambrisentan (Letairis®) is a selective endothelin receptor antagonist. Letairis is administered in simple pill form.
. Sildenafil (brand name Revatio®) is an oral phosphodiesterase (PDE) inhibitor. It prevents the breakdown of a substance made by the body called cyclic GMP which helps to open up constricted blood vessels in the lung, thereby enabling the heart to pump blood more effectively. Sildenafil is administered in a simple pill form.
. Epoprostenol (brand name Flolan®) is the synthetic salt of prostacyclin, a substance made by the body that is found in decreased amounts in patients with PH. This medication is delivered by a constant intravenous infusion.
. Treprostinil (brand name Remodulin) is a prostacyclin analogue. It is administered through a continuous subcutaneous (under the skin) or intravenous infusion.
. Iloprost (brand name Ventavis®) is another prostacyclin analogue. It is administered by inhalation, 5 to 8 times daily. |
For patients who do not respond to any of these treatments, a lung transplant may be an option for selected patients.
How to schedule an appointment with PH physician?
To make an appointment call 251 471 7029.
During your appointment you will be evaluated by one of our physicians with special expertise in pulmonary hypertension. Once the testing and evaluation is completed, your physician will discuss various therapeutic options appropriate for your stage of disease and will determine the option most appropriate for your condition and make the decision with you.
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