Pulmonary Hypertension

Pulmonary hypertension occurs when the blood pressure in the lungs rises far above normal levels.

This often occurs because of narrowing of the lung blood vessels, reducing the flow of blood through the lungs. As a result, the heart has to pump harder as it tries to maintain a normal amount of blood flow.

Symptoms of Pulmonary Hypertension

Pulmonary hypertension causes shortness of breath, particularly with exertion. In advanced cases, right-sided heart failure can occur, causing more severe shortness of breath and, in some cases, swelling in the legs or abdomen.

 The most common symptoms of pulmonary hypertension include:

  • Shortness of breath, particularly with exertion (dyspnea)
  • Chest pain or angina
  • Fatigue
  • Dizziness
  • Fainting spells (syncope)
  • Racing pulse or irregular heart beat
  • Swelling in the ankles or legs (edema)
  • Bluish lips and skin (cyanosis)

Causes of Pulmonary Hypertension

There are many different conditions that can cause pulmonary hypertension. Two of the most common are:

  • Left heart conditions, such as heart failure or valvular heart disease
  • Lung diseases like emphysema

Other less common but serious causes of pulmonary hypertension include scleroderma and other connective tissue diseases, congenital heart disease, liver disease, and chronic pulmonary emboli (blood clots).

In some cases, pulmonary hypertension occurs without any underlying condition. This is referred to as idiopathic pulmonary arterial hypertension (also known as primary pulmonary hypertension).

Evaluation of Pulmonary Hypertension

Because there are so many different types of pulmonary hypertension, most patients will need to undergo a series of tests.

Once pulmonary hypertension is suspected, echocardiogram is usually the first test performed to estimate the pulmonary arterial pressures. An echocardiogram uses sound waves to take pictures of your heart to see how well it is beating and pumping blood. It also estimates how high the pressures are in the lungs.

Other tests that are useful in the evaluation of pulmonary hypertension include:

  • Overnight oximetry to measure oxygen levels while you sleep
  • Chest X-ray to evaluate for lung disease
  • Lung function test to evaluate for lung disease
  • VQ scan to evaluate for chronic blood clots (preferred over CT angiogram)

If your tests indicate significant pulmonary hypertension, we will recommend you undergo a right heart catheterization to confirm the diagnosis. During this test, a thin tube (catheter) is inserted in the neck or groin. The catheter is then used to measure the blood pressure in the lungs and heart and to measure how much blood the heart is pumping. Based on these results, a treatment plan can usually be identified.

Some patients will need to undergo other tests, based on their history or the results of the initial evaluation. These may include:

  • A sleep study to look for sleep apnea
  • High resolution CT scan to look for interstitial lung disease
  • Pulmonary angiography to look for chronic blood clots

Treatment Options for Pulmonary Hypertension

The right treatment depends on which type of pulmonary hypertension you have. Some conditions are best treated by focusing on the underlying condition, while others require pulmonary hypertension-specific treatment.

There are nine different approved medical therapies for pulmonary hypertension. Four are available as pills; the remaining five are either inhaled or given through a catheter that is inserted under the skin or into a blood vessel.

Close monitoring is required even with the pill medications because of the seriousness of the condition and because several medications require monthly laboratory monitoring. 

Treatments include:

  • Endothelin-1 antagonists: bosentan (Tracleer®), ambrisentan (Letairis)
  • Phosphodiesterase-5 inhibitors: sildenafil (Revatio®), tadalafil (Adcirca®)
  • Prostacyclins for inhalation: treprostinil (Tyvaso®), iloprost (Ventavis®)
  • Prostacyclins for injection: epoprostenol (Flolan®, Veletri®), treprostinil (Remodulin®)

Since there is no medical cure for pulmonary hypertension, the search for the cure is important and ongoing. UT Southwestern has been in the forefront of research participating in dozens of multicenter clinical trials trying to find a cure to this disease. This is the largest effort in North Texas to help patients suffering of this disease.

Patients who want to participate in clinical trials are offered the opportunity to be part of this quest for the cure. 

Our Team

Our pulmonary hypertension specialists have extensive experience in treating pulmonary hypertension, including the full range of oral, inhaled, and injection therapies.

Our pulmonologists – Fernando Torres, M.D.; Kelly Chin, M.D.; Sonja Bartolome, M.D.; and Megan Devine, M.D. – lead a multidisciplinary team dedicated to accurately diagnosing and treating patients with pulmonary hypertension.

Your team – which may include specialists in cardiology, rheumatology, sleep medicine, cardiovascular and thoracic surgery, and solid-organ transplant – will develop a personalized plan to treat your pulmonary hypertension and improve your quality of life.

The pulmonary hypertension team at UT Southwestern is well known nationally for the medical care and clinical research in this field. They are members of the leadership in the most prestigious societies like the Pulmonary Hypertension Association.

If surgery is needed, our surgeons are widely recognized as leaders in lung transplantation to treat pulmonary hypertension. 

Request an Appointment

For a pulmonary hypertension evaluation at UT Southwestern's facility in Dallas, or for more information about services we offer, request an appointment or call 214-645-8300.