Pulmonary hypertension can be caused by other medical conditions, such as heart disease or lung disease.

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.

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.

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.

Causes of pulmonary hypertension

Pulmonary hypertension is usually inherited, and sometimes its cause is unknown. With pulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries, causing 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, 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

Sign and 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:

  • Fatigue
  • Unexplained dyspnea
  • Difficulty breathing
  • Dizziness and fainting
  • Swelling in the legs and ankles
  • Bluish lips and skin
  • Chest pain
  • Racing pulse
  • Trouble getting enough air
  • Palpitations

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

Risk Factors

Your risk of developing pulmonary hypertension may be greater if:

  • Overweight
  • Family history of the disease
  • Use of illegal drugs, such as cocaine
  • Use of certain weight loss mediciations
  • Connective tissue disease
  • HIV
  • Cirrhosis
  • Thromboembolic disease
  • Congenital heart disease
  • Sickle cell disease

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

How Ohio State diagnoses pulmonary hypertension

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.

How Ohio State treats pulmonary hypertension

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:

Medication

There are various medications used to treat pulmonary hypertension, and new studies are underway to test the efficacy of new medicine.

Surgery

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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