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Pulmonary hypertension is a rare lung disorder in which the blood pressure in the pulmonary artery (the artery that goes from the heart to the lungs) rises far above normal levels, placing excess stress on the right side of the heart. Pulmonary hypertension is a long-term or chronic disease affecting both sexes; however, it is more common in women.
Very high blood pressure in the pulmonary arteries causes changes in the blood vessels in your lungs and prevents normal blood flow through these vessels. High blood pressure in your lung vessels causes your right ventricle and right atrium (heart chambers) to become enlarged and weak and to not pump as well.
Pulmonary hypertension is usually inherited, and sometimes its cause is unknown. Researchers believe the blood vessels are sensitive to certain internal and external factors and narrow when exposed to these factors, including immune system factors and sensitivity to drugs and other chemicals.
There are several types of pulmonary hypertension, and causes of the disease depend on the type:
Symptoms can be similar to congestive heart failure. In severe cases, fluid can back up into the abdomen and cause fullness, congestion of the liver and leg swelling. Other symptoms include:
In advanced stages of the disease, symptoms can include inability to perform even minimal activities; you can even experience symptoms when resting.
The Ohio State University Wexner Medical Center has specialists in pulmonology and cardiology who provide comprehensive evaluation and treatment of pulmonary hypertension. Although idiopathic pulmonary arterial hypertension, a particular type of pulmonary hypertension, is considered a rare condition, our experts have the experience to treat these patients.
If the pulmonary hypertension is related to clots in the lungs it can be cured by surgical procedures. The Ohio State University Wexner Medical Center is one of the few hospitals in the Midwest that performs pulmonary thromboendarterectomy, the surgical removal of blood clots in the pulmonary artery.
We’re also involved in several research studies for new medications and trials evaluating the use of FDA-approved combination therapy. This combination therapy involves combining various agents and therapies and is being evaluated in clinical trials.
If your physician suspects you have pulmonary hypertension, you will have a complete examination and diagnostic tests that may include:
Chest X-ray – A radiograph or picture of the heart and lungs including blood vessels, ribs and bones of the spine.
Electrocardiogram (EKG/ECG) – A test that records the electrical activity of the heart.
Echocardiogram (also called echo) – This test uses sound waves to check the heart. These ultrasound waves can produce pictures that show the function of the heart and valves. Pressures from inside of the heart and pulmonary arteries can be estimated from the waveforms.
Pulmonary function tests – Tests to measure the lungs’ ability to exchange oxygen and carbon dioxide appropriately.
Perfusion lung scan – A nuclear medicine procedure to detect blood clots in the vessels of the lung.
CT scan (computed tomography scan, also called CAT scan) – An imaging procedure that uses X-rays and computer technology to get more detailed pictures of the lungs and vessels.
Right heart catheterization – A procedure to measure the pressures in the pulmonary artery. A long thin tube is inserted in a vein either in the neck or groin, then threaded into the right side of the heart and out through the pulmonary artery to measure the pressures.
Exercise tolerance test – You are asked to walk for six minutes and then distance, heart rate and oxygen levels are measured. This test is sometimes performed on a treadmill.
While pulmonary hypertension is not curable, it is treatable. Treatment is aimed at lowering the pulmonary artery pressure and relieving symptoms. Treatment of idiopathic pulmonary arterial hypertension, the rarest form of pulmonary hypertension, varies according to the stage of the disease, and systematic evaluation is required to decide which therapy is best for an individual patient. Treatment may include:
There are various medications used to treat pulmonary hypertension, and new studies are underway to test the efficacy of new medicine.
If your pulmonary hypertension is caused by blood clots in your pulmonary artery, they may be surgically removed to improve blood flow. This is called a pulmonary thromboendarterectomy. The Ohio State University Wexner Medical Center is one of the few hospitals in the Midwest that performs this procedure.
Lung transplantation is performed for advanced stages of pulmonary hypertension. In severe cases in which there has been damage to the heart, combined heart-lung transplantation may be required.
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