Pulmonary hypertension is a type of high blood pressure that affects the pulmonary artery (the artery that goes from the heart to the lungs) and 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. Ohio State Wexner Medical Center’s vascular experts provide a variety of treatment options for those with pulmonary hypertension, right here in Columbus.

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 not pump as well.

Some forms of pulmonary hypertension are serious conditions that become progressively worse and sometimes fatal. Although some forms of this disease aren’t curable, treatment can help alleviate symptoms and improve quality of life.

Pulmonary hypertension causes

Pulmonary hypertension is usually inherited, and sometimes its cause is unknown. With pulmonary hypertension, changes in the cells that line your pulmonary arteries causes a rise in blood pressure, which then causes the artery walls to become stiff and thick. The blood vessels may also become inflamed and tight.

These changes in the arteries can reduce or block blood flow in the vessels, making it harder for blood to flow. This raises the blood pressure in the pulmonary arteries. 

There are several types of pulmonary hypertension, and causes of the disease depend on the type:

  • Pulmonary arterial hypertension (APAH) is associated with other diseases such as scleroderma, HIV, liver cirrhosis and congenital heart disease.
  • Secondary pulmonary hypertension is caused by another medical condition, such as heart disease, lung disease or clots in the lung.
  • Idiopathic pulmonary arterial hypertension (IPAH) is when the underlying cause for high blood pressure in the lungs can’t be found.

Pulmonary hypertension risk factors

Your risk of developing pulmonary hypertension may be greater if you have one or more of the following:

  • Cirrhosis
  • Congenital heart disease
  • Connective tissue disease
  • Family history of hypertension
  • Human immunodeficiency virus (HIV)
  • Sickle cell disease
  • Thromboembolic disease

Other risk factors include:

  • Being overweight
  • Using certain weight loss medications
  • Using illegal drugs, such as cocaine

Symptoms of pulmonary hypertension

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:

  • Bluish lips and skin
  • Chest pain
  • Difficulty breathing
  • Dizziness and fainting
  • Fatigue
  • Palpitations (rapid, strong or irregular heartbeat)
  • Racing pulse
  • Swelling in the legs and ankles
  • Trouble getting enough air
  • Unexplained dyspnea (shortness of breath)

In advanced stages of the disease, symptoms can include inability to perform even minimal activities; you can even experience symptoms when resting.

Diagnosis of pulmonary hypertension

If your physician suspects you have pulmonary hypertension, you’ll have a complete examination and diagnostic tests that may include:

  • Chest X-ray – This radiograph (picture of the heart and lungs) shows blood vessels, ribs and bones of the spine.
  • Electrocardiogram (EKG/ECG) – This test 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 – These tests measure the lungs’ ability to exchange oxygen and carbon dioxide appropriately.
  • Perfusion lung scan – This is a nuclear medicine test that can help detect blood clots in the blood vessels of the lung.
  • CT scan (computed tomography scan, or CAT scan) – This imaging procedure uses X-rays and computer technology to obtain more detailed pictures of the lungs and vessels.
  • Right heart catheterization – This procedure measures 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’ll be asked to walk for 6 minutes and then distance, heart rate and oxygen levels are measured. This test is sometimes performed on a treadmill.

Perfusion lung scan

A perfusion lung scan is a type of nuclear scan that uses a special camera and a radioactive tracer to detect abnormalities in the organs of the body. A perfusion test measures the amount of blood an organ receives through its vessels to supply it with nutrients and oxygen. Certain conditions prevent different areas of your lungs from receiving even amounts of oxygen. Physicians order perfusion lung scans to help diagnose blockages in the pulmonary arteries, also known as embolisms. Physicians also use this test to determine the severity of lung diseases, as well as before lung removal surgery.

What to expect during a perfusion lung scan

There are no special preparations for this test, although it’s important to tell your physician if you’re pregnant or breastfeeding. Your physician may order a chest X-ray before your perfusion lung scan test.

  • During your procedure: If you’re having a perfusion lung scan done, you’ll receive a small amount of radioactive tracer through an IV. If you’re having a ventilation lung scan, you’ll receive a small amount of radioactive tracer inhaled through a mask. For both tests, once the tracer circulates through your body, you’ll lie on a flat, movable table inside a round scanner. During the test, this scanner rotates around you, taking a series of images. You’ll be able to see and hear your technologist during this test, which takes about 45 minutes.
  • After your procedure: There are no special directions to follow after your perfusion lung scan, although your physician may give you specific instructions depending on your medical condition. Often, additional tests are necessary to adequately diagnose your lung condition.

Treatment of pulmonary hypertension

While pulmonary hypertension is not curable, it is treatable. The goal of treatment is to lower the pulmonary artery pressure and relieve symptoms. Treatment of idiopathic pulmonary arterial hypertension, the rarest form of pulmonary hypertension, varies according to the stage of the disease. Our specialists use testing and personalized assessment to decide which therapy is best for you. Treatment may include:

Medication: Our team uses various medications to treat pulmonary hypertension, and new studies are underway to test the efficacy of new medicine.

Surgery: If blood clots in your pulmonary artery have caused your pulmonary hypertension, your vascular specialist may recommend surgically removing the clots 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. We perform lung transplantation 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.

Why choose Ohio State for pulmonary hypertension treatment?

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 Food and Drug Administration-approved combination therapy. This combination therapy involves combining various agents and therapies and is being evaluated in clinical trials.

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