Pulmonary hypertension (PH) is elevated blood pressure in the blood vessels of the lungs. PH can affect men and women of all ages and races. When the pressure in the lungs is high, it causes the right side of the heart to work harder. If left untreated, this condition can lead to right heart failure and a higher risk of death.
UPMC offers a full spectrum of options, including surgical care and new and improved medical treatments. These include oral, inhaled, subcutaneous (under the skin), and intravenous (vein) therapies.
Our level of expertise in managing the most complex cases means we can:
- Offer accurate diagnostic testing.
- Provide long-term management and co-management of patients with doctors in their local area.
- Participate in disease treatment.
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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 pressure in the lungs is high, it causes the right side of the heart to work harder. Left untreated, this condition can lead to heart failure and an increased risk of death.
What are the types of PH?
There are several types of PH, including:
Pulmonary arterial hypertension (PAH)
Pulmonary arterial hypertension (PAH) is a type of pulmonary hypertension, which means high blood pressure in the lungs. In PAH, the pulmonary arteries become stiff and dysfunctional. They can thicken or narrow as the disease progresses.
The heart must work harder to pump blood through the stiff, narrow arteries, which causes blood pressure in the lungs to increase.
The major types of pulmonary arterial hypertension (PAH) include:
- Idiopathic — Develops without an apparent or known cause.
- Heritable — Common among family members due to a genetic mutation.
- Drug and toxin-induced — Can be caused by medications or illicit substances.
Medical treatments for PAH may include:
- Inhaled medications.
- Oral medications.
- Subcutaneous and intravenous medications.
In addition to medicine, doctors may:
- Prescribe a low-salt diet to minimize fluid retention.
- Recommend monitored pulmonary rehabilitation.
- Refer people for a lung transplant evaluation in the event their disease is severe or progresses despite medicines.
COPD or pulmonary fibrosis-associated PH
Treating PH associated with chronic lung disease such as COPD or pulmonary fibrosis is challenging. People with lung disease do not necessarily respond the same way as those without lung disease.
Treatment for mild PH with COPD or pulmonary fibrosis includes lifestyle changes, such as quitting smoking and exercising. Treatment for moderate or severe PH with COPD or pulmonary fibrosis includes:
- Medications.
- Oxygen therapy.
- Pulmonary rehab.
- Surgery.
HIV-associated PAH
Approximately 1 in 200 people living with HIV will have HIV-associated pulmonary arterial hypertension (HIV-PAH), although recent studies have reported this may be higher.
If untreated, HIV-PAH can get worse and shorten one’s life.
A team of pulmonologists and cardiologists at UPMC works closely with the Pittsburgh AIDS Center for Treatment (PACT) to diagnose and treat HIV-PAH.
PH caused by chronic blood clots in the lungs
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare type of pulmonary hypertension that occurs after you have a condition called pulmonary embolism, or a blood clot in your lungs.
When a pulmonary embolism doesn’t completely go away after treatment, it is known as a chronic pulmonary embolism. A chronic pulmonary embolism can block blood flow in your pulmonary arteries, causing CTEPH.
UPMC is one of only five programs nationwide specializing in the treatment of CTEPH. UPMC's experts are skilled in diagnosing and treating CTEPH, offering personalized treatment options including surgical removal of blood clots, medications, and lung transplantation.
PH caused by systolic and diastolic heart failure
Every time a healthy heart pumps oxygen-rich blood from the lungs into the rest of the body, it goes through two phases — a contracting or pumping phase (called systolic function) and a relaxing phase (called diastolic function).
When a weakened heart can't pump efficiently, that's known as systolic dysfunction. Just like a kink in a garden hose, blood begins to back up into the lungs and causes pulmonary hypertension (PH).
When the muscles of the heart become stiff, they can't relax properly, creating a condition known as diastolic dysfunction. This inflexibility prevents the heart's ventricles from filling completely, causing blood to back up in the organs.
Pulmonary hypertension caused by diastolic or systolic heart dysfunction can't be cured, but the symptoms can be managed. Our treatment focuses on improving a person's quality of life and helping them maintain an independent lifestyle.
- Heart transplant — UPMC, a nationally known center of excellence in transplantation, has leading experts in heart transplantation.
- Implantable devices — An implantable cardioverter-defibrillator (ICD) or pacemaker can be combined with medical therapies to synchronize the heart's pumping motions and even improve the condition of the heart muscle itself.
- Left ventricular assist devices (LVAD) — UPMC cardiologists have been at the forefront of LVAD treatment since the technology was introduced in the 1980s and have led efforts to improve it ever since. These implantable devices pump blood into the body just as a healthy heart would. LVADs can be used:
- When a person is waiting for a heart transplant — LVADs are used as a "bridge" to heart transplantation, meaning that they do the work of the heart until a human heart becomes available.
- As a permanent treatment for heart failure — Today's LVADs are now fully implantable, small enough to fit in most adult chests and durable enough for long-term use.
- Medications — Help to lower blood pressure and make the heart's pumping function easier.
- New stem cell therapies — UPMC researchers are working on new therapies for systolic heart dysfunction that involve injecting healthy stem cells into failing hearts to improve their function.
Scleroderma-associated PH
Scleroderma is an umbrella term to describe thickening (sclero) of the skin (derma). People with scleroderma are at increased risk for developing several types of pulmonary hypertension (PH), including:
- Interstitial lung disease or pulmonary fibrosis.
- PH related to heart disease due to stiffening of the heart muscle.
- Pulmonary arterial hypertension.
At UPMC, we provide a comprehensive evaluation and care for scleroderma-associated pulmonary hypertension.
Our experts meet regularly with pulmonary hypertension and pulmonary fibrosis specialists to coordinate patient care. We also offer a full range of medical and surgical therapies, in partnership with one of the largest and most distinguished heart and lung transplantation centers in the world.
Sickle cell disease-associated PH
Pulmonary hypertension (PH) is known to affect between 6 and 10 percent of people with sickle cell disease. Our experts treat sickle cell disease-associated PH by:
- Effectively managing sickle cell disease to reduce symptoms and minimize the risk of complications.
- Treating other lung and airway conditions such as asthma, pulmonary embolism, and obstructive sleep apnea.
- Using pulmonary vasodilator therapy to widen blood vessels in the lungs and improve blood flow.
Lung transplant surgery may be a treatment option for some patients. UPMC performed the first lung transplant in a patient with sickle cell disease-associated pulmonary hypertension, with an excellent surgical outcome.
How common is PH?
PH is a rare condition. According to the National Institutes of Health, PH affects about 1 percent of the population worldwide.
What causes PH?
PH occurs when the blood pressure in the blood vessels of your lungs becomes high. Many different factors can cause PH, including:
- Certain heart diseases.
- Genetics.
- Liver disease.
- Low-oxygen conditions.
- Lung conditions.
- Rheumatic disorders.
- Thromboembolic disease.
Pulmonary hypertension risk factors
You may be at risk of PH if you have:
- A family history of PH.
- A personal or family history of blood clots in the legs or lungs.
- Heart disease, including heart failure or congenital heart disorders.
- HIV.
- Lung disease, such as emphysema, COPD, or pulmonary fibrosis.
- Sarcoidosis.
- Scleroderma.
- Sickle cell disease.
Complications of pulmonary hypertension
Left untreated, PH can lead to right heart failure and increased risk of death. Other complications may include:
- Anemia (low red blood cell count).
- Arrhythmias (abnormal heart rhythms).
- Blood clots or bleeding in your lungs.
- Liver damage.
- Pericardial effusion (fluid buildup around your heart).
- Pregnancy complications.
What Are the Signs and Symptoms of Pulmonary Hypertension?
Symptoms of PH may resemble those of other heart or lung disorders, making diagnosis challenging. PH symptoms may include:
- Chest pain.
- Cough.
- Fatigue.
- Inability to lie flat without being short of breath.
- Lightheadedness.
- Passing out.
- Reduced exercise capacity.
- Shortness of breath with exertion.
- Swelling of the ankles or abdomen.
- Weight gain.
When should I see a doctor about my PH symptoms?
Because PH is a rare condition that causes symptoms like other heart or lung disorders, it can be challenging to diagnose in its early stages. However, an early, accurate diagnosis can improve treatment outcomes.
If you are having symptoms of PH, you should schedule an appointment with your primary care provider or cardiologist right away.
How Do You Diagnose Pulmonary Hypertension?
At your evaluation, your UPMC doctor will ask about your symptoms and medical history. Your doctor will then perform a careful diagnostic work-up to determine the diagnosis, clarify the severity, and identify the potential cause.
What to expect during your visit
If your doctor suspects PH, they will:
- Ask about your symptoms.
- Order tests.
- Perform a physical exam.
- Review your health history and medications.
Since evaluating PH can be complex, you might see a heart or lung specialist first, along with another specialist, on the same day. We use this approach to review these tests together and ensure an accurate diagnosis so we can create an effective treatment plan.
Tests to diagnose PH
Doctors diagnose PH using tests including:
- Cardiac catheterization — This test is a diagnostic procedure that allows your doctor to see blockages, blood flow, and other problems within your arteries.
- Cardiac MRI — Uses radio waves, magnets, and a computer to create pictures of your heart without using ionizing radiation.
- Coronary CT angiography (CCTA) imaging — Also known as a CT angiogram, this test uses computed tomography (CT) to look at the blood flow in your coronary arteries.
- CT scans — CT scans create images of your lungs and heart, as well as other bones, organs, and tissues inside your body.
- Echocardiogram — Also known as an ECHO, this noninvasive heart ultrasound procedure assesses the function and structures of your heart.
- Electrocardiogram — Checks for signs of abnormal heart rhythms.
- Lung function tests — Check to see how well your lungs are working.
- Pulmonary angiography — Shows images of your lungs using a contrast agent to highlight your blood vessels.
- Six-minute walk test — Shows how far you can walk and measures your oxygen levels during exercise.
- Ventilation/perfusion scan (V/Q scan) — Measures air and blood flow in your lungs.
PH prognosis
Your prognosis after a PH diagnosis depends on the cause of your condition and whether it can be treated, as well as how you respond to medications and other treatments. Increased awareness of the condition has improved diagnostic tools and treatment options. As a result, many people with PH are living longer, healthier lives.
How Do You Treat Pulmonary Hypertension?
PH treatment depends on the type and severity of your disease. The goals of treatment are to manage symptoms, improve quality of life, and reduce heart and lung damage.
Although there is no cure, there are several medical and surgical treatments for PH, including:
Medication
Medication options for PH include:
- Inhaled medications.
- Intravenous (IV) medications.
- Medications injected under the skin (subcutaneous).
- Oral medications.
Surgical procedures
For those who do not respond to medical therapy, surgical treatments for PH include:
- Atrial septostomy — Uses a balloon-tipped catheter to create a hole in the wall that separates the two upper chambers of your heart. This allows blood to bypass your lungs and reduces symptoms of heart failure.
- Balloon pulmonary artery angioplasty (BPA) — Treats chronic blood clots causing a blockage in the pulmonary arteries. The procedure involves using a balloon-tipped catheter to push the clots against the wall of the artery and restore blood flow.
- Lung transplantation — Involves removing your diseased lung(s) and replacing them with healthy lung(s) from a deceased donor.
- Pulmonary thromboendarterectomy (PTE) — Also called pulmonary endarterectomy (PEA), this complex surgical procedure can provide a cure for CTEPH by removing blood clots, restoring normal blood flow to the lungs, and preventing heart failure and death.
Why Choose UPMC for Pulmonary Hypertension Care?
When you choose UPMC for PH care, you will receive:
- Access to innovative treatments — UPMC specialists are constantly investigating new ways to enhance our program with promising therapies that may help individualize future treatments for people with PH.
- Advanced diagnostic capabilities — Our experts have the tools and expertise required to diagnose PH in its earliest stages, which could improve treatment outcomes and quality of life.
- Experience in advanced procedures — UPMC is a national leader in PH surgery. People who require a lung transplant have access to advanced transplantation care from one of the country’s highest-volume lung transplant programs.
