Pulmonary Hypertension

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What Is Pulmonary Hypertension?

Pulmonary hypertension (PH) is high blood pressure in the blood vessels in the lungs. PH can affect both men and women of all ages and races.

When the pressures in the lungs are high, it causes the right side of the heart to have to work harder.

Although it is a relatively uncommon disease, it's important to make an early and accurate diagnosis. If untreated, this condition can lead to right heart failure and increased risk of death.

Pulmonary Hypertension Symptoms

PH symptoms can include:

  • Shortness of breath with exertion
  • Lightheadedness
  • Fatigue
  • Cough
  • Chest pain
  • Passing out
  • Inability to lie flat without being short of breath
  • Swelling of the ankles or abdomen

Contact the UPMC Comprehensive Pulmonary Hypertension Program if you think you might have pulmonary hypertension.

Types of Pulmonary Hypertension

There are many different types of PH. Treatment options vary depending on the type:

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Pulmonary Hypertension Causes

Pulmonary hypertension (PH) can be caused by many different factors. Some causes of pulmonary hypertension include:

  • Liver disease
  • Certain heart diseases
  • Thromboembolic disease
  • Genetics
  • Rheumatic disorders
  • Lung conditions
  • Low-oxygen conditions

Pulmonary Hypertension Diagnosis

Making the diagnosis of PH begins with reviewing symptoms and doing tests.

Common tests for PH

Transthoracic echocardiogram is an ultrasound of the heart.

It is a good test for:

  • Evaluating the overall structure and pump-function of the heart.
  • Identifying increased pressures on the right side (pulmonary side) of the heart.

If this test is positive or if there is a high suspicion of PH, often the PH specialist will perform a right heart catheterization.

Right heart catheterization is the gold standard for diagnosing pulmonary hypertension. Doctors use a catheter to measure the pressure in the heart and lungs.

In this procedure, the patient has local numbing medicine.

Then, the PH specialist will:

  • Insert the catheter through the large vein in the neck or groin area.
  • Pass the catheter through the right side of the heart and into the pulmonary artery.
  • Measure the pressures and withdraw the catheter.

If the doctor suspects left heart disease, sometimes a patient may have a left heart catheterization at the same time. This examines the arteries of the heart for blockages and measures pressures on the left side of the heart.

Other tests for diagnosing PH include:

  • Blood tests
  • Six-minute walk test
  • Pulmonary function testing
  • Chest x-ray
  • CT scan of chest
  • Ventilation/perfusion scan
  • Cardiac MRI

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Pulmonary Hypertension Treatments

Although there is no cure to date, there are several medical and surgical treatments for PH.

Pulmonary hypertension treatment depends on the type and severity of disease.

For pulmonary arterial hypertension, there are currently 12 FDA-approved medicines on the market.

And, for those who do not respond to medical therapy, surgical treatments for pulmonary arterial hypertension include:

Follow up care for PH

While it can take weeks to months to notice a significant improvement with medical therapy, it is important to follow up closely with your PH specialist to make sure that there is improvement.

Patients will follow up within three months of their diagnosis.

If they are doing well, they will follow up every six to 12 months for continued monitoring of their disease.

Follow up can involve repeat testing including:

  • The six-minute walk test
  • Echocardiogram
  • Heart catheterization

Make an appointment today

Starting pulmonary hypertension treatment early is important in improving overall quality of life.

To schedule an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

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Learn More About Pulmonary Hypertension

UPMC Patient Education Materials:

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From our Health Library:


Schedule an Appointment

To schedule an appointment with the UPMC Comprehensive Pulmonary Hypertension Program team, call toll-free at
1-877-PH4-UPMC, or email PHprogram@upmc.edu.​​

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