Pulmonary Hypertension

New Patient Appointment or 214-645-6616

At UT Southwestern Medical Center, our specialists in pulmonology, cardiology, rheumatology, sleep medicine, cardiovascular and thoracic surgery, and solid-organ transplant have years of experience diagnosing and treating people with pulmonary hypertension. As a team, they develop a personalized plan for patients, using the latest advancements in heart and lung care to manage symptoms for improved health.

Advanced Treatment for Pulmonary Hypertension

Pulmonary hypertension is high blood pressure in the lung (pulmonary) arteries, which carry blood from the heart to the lungs to pick up oxygen. The condition develops when lung arteries tighten or narrow and cannot carry enough blood. The heart must pump harder to maintain normal blood flow, which increases pressure in the lung arteries and weakens the right ventricle (lower heart chamber).

If undetected and untreated, pulmonary hypertension can lead to shortness of breath, arrhythmia (irregular heartbeat), right-sided heart failure, and death.

Our physicians specialize in managing the most complicated cases of pulmonary hypertension, and have extensive experience in treating pulmonary hypertension with the full range of oral, inhaled, and injection therapies. Our pulmonologists lead a multidisciplinary team dedicated to accurately diagnosing and treating patients with pulmonary hypertension.

The pulmonary hypertension team at UT Southwestern is known nationally for its medical care and clinical research. Our specialists are leaders in the most prestigious organizations, including the Pulmonary Hypertension Association. Our surgeons are also widely recognized as leaders in lung transplantation, which is sometimes needed to treat severe cases of pulmonary hypertension.

UT Southwestern is the only medical center in North Texas that offers National Institutes of Health- and industry-sponsored pulmonary hypertension clinical trials. Our internationally recognized Pulmonary Hypertension Program is the largest referral center in the area by patient volume and ranks among the 10 largest pulmonary hypertension centers in the country.

Types of Pulmonary Hypertension

Pulmonary hypertension is categorized into five groups based on the condition’s cause:

Group 1 includes types that:

Have no known cause
Are inherited
Result from the use of certain drugs or toxins
Result from certain other health conditions

Group 2 includes pulmonary hypertension with left-sided heart disease, such as heart failure or mitral valve disease.

Group 3 includes types associated with lung diseases such as chronic obstructive pulmonary disease and pulmonary fibrosis.

Group 4 is pulmonary hypertension caused by blood clots in the lungs, known as chronic thromboembolic pulmonary hypertension (CTEPH).

Group 5 includes types caused by other diseases such as:

Blood disorders
Metabolic disorders, such as thyroid disease
Systemic disorders, such as sarcoidosis

Causes of Pulmonary Hypertension

Known risk factors and conditions associated with pulmonary hypertension include:

Autoimmune diseases that cause lung damage, such as scleroderma and lupus
Blood clots in the lungs (pulmonary embolism)
Certain medications
Chronic, low blood oxygen levels
Congenital birth defects in the heart
Heart conditions, such as heart failure or heart valve disorders
HIV infection
Lung diseases, such as emphysema or pulmonary fibrosis
Obstructive sleep apnea

In rare cases, pulmonary hypertension can develop for unknown causes. This condition is called idiopathic pulmonary arterial hypertension.

Symptoms of Pulmonary Hypertension

Often, the first symptom of pulmonary hypertension is shortness of breath or lightheadedness, particularly with exertion. Other common symptoms include:

Bluish lips and skin (cyanosis)
Chest pressure or pain (angina)
Dizziness
Enlarged abdomen
Fainting (syncope)
Fatigue and weakness
Rapid pulse or irregular heartbeat
Reduced ability to exercise
Swelling in the ankles or legs (edema)

Diagnosis of Pulmonary Hypertension

Our pulmonologists are experts at evaluating symptoms to confirm a diagnosis. We begin with a thorough evaluation that includes a:

Physical exam
Review of personal and family medical history
Discussion of symptoms

If they suspect pulmonary hypertension, they usually order an echocardiogram, or echo. An echocardiogram uses sound waves to create images of the heart to see how well it is beating and pumping blood. It also helps evaluate blood pressure in the lung arteries.

Other tests that are useful in confirming a diagnosis of pulmonary hypertension include:

Blood tests to check for signs of pulmonary hypertension or other, underlying conditions
Chest X-ray to rule out other causes for symptoms
Echocardiogram, or echo, to see how well the heart is beating and pumping blood and to evaluate blood pressure in the lung arteries
Electrocardiogram to check heart rhythm
Magnetic resonance imaging (MRI) scan to create detailed images of the heart, using radio waves and magnets, to evaluate right ventricle function and blood flow to the lungs
Pulmonary function tests to evaluate lung function, such as the rate of air flow in and out of the lungs, how well the lungs move oxygen into the bloodstream, and lung size
Ventilation/perfusion (VQ) scan to check for chronic blood clots

If tests indicate pulmonary hypertension, our specialists recommend patients undergo a right heart catheterization to confirm the diagnosis. During this test, a thin tube (catheter) is inserted into a small incision in the neck or groin. The doctor uses the catheter to measure blood pressure in the lungs and heart, and measure how much blood the heart is pumping.

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

Pulmonary angiography to look for chronic blood clots
Sleep study to check for sleep apnea

Treatment for Pulmonary Hypertension

Based on each patient’s test results, our team of specialists works together to develop a personalized treatment plan. Some conditions are best treated by focusing on the underlying condition, while others require pulmonary hypertension-specific treatment.

Medications for pulmonary hypertension can include pills taken by mouth, others that are inhaled, and still others given intravenously (IV).

Several medications require monthly laboratory monitoring.

Treatments include:

Endothelin-1 antagonists: Bosentan (Tracleer®), ambrisentan (Letairis®), macitentan (Opsumit®)
Phosphodiesterase-5 inhibitors: Sildenafil (Revatio®), tadalafil (Adcirca®)
Guanylyl cyclase stimulator: Riociguat (Adempas®)
Oral IP receptor agonists and oral prostacyclins: Treprostinil (Orenitram®), selexipag (Uptravi®)
Prostacyclins for inhalation: Treprostinil (Tyvaso®), iloprost (Ventavis®)
Prostacyclins for injection: Epoprostenol (Flolan®, Veletri®), treprostinil (Remodulin®)

Center of Comprehensive Care

UT Southwestern Medical Center’s Pulmonary Hypertension Program is accredited as a Center of Comprehensive Care through the Pulmonary Hypertension Care Centers (PHCC) program.

This effort, initiated and managed by the Pulmonary Hypertension Association and its Scientific Leadership Council, seeks to improve the quality of care and the health outcomes of patients with pulmonary hypertension, specifically pulmonary arterial hypertension.

At the PHCC, each patient’s health is managed by highly trained, specialized physicians, and allied health professionals dedicated to the proper clinical management and the larger pulmonary hypertension scientific community. The accreditation also signifies that UT Southwestern has met the rigorous standards of PHCC criteria.

A PHCC designation means that:

Patients receive comprehensive pulmonary hypertension care at centers with expertise in this area.
Therapies are appropriately used to improve long-term outcomes.
Increased opportunities are created for collaboration between centers for clinical care and pulmonary hypertension research.

Patient Transfers

The patient transport service provides 24-hour direct hospital-to-hospital patient transfers for emergency and complex cases to UT Southwestern facilities. Call 877-645-0911 to arrange a patient transfer – 24 hours a day, 7 days a week.

Clinical Trials

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

Patients who want to participate in clinical trials have the opportunity to be part of this quest for the cure. Find out more about clinical trials for pulmonary hypertension and other lung diseases.