Pulmonary hypertension is high blood pressure in the pulmonary (lung) artery between the lungs and heart. It’s caused by narrowed, blocked, or damaged blood vessels due to inflammation or scarring. When this happens, it affects the blood flow from the heart to the lungs.

Symptoms of pulmonary hypertension can include chest pain, shortness of breath, rapid heartbeat, swelling in ankles or legs, and fainting. Untreated, pulmonary hypertension can lead to right-sided heart failure (caused by the heart working too hard to pump blood), as well as blood clots (pulmonary embolism), irregular heartbeat (arrhythmias), or bleeding in the lungs.

There are different types of pulmonary hypertension based on the cause, which can be unknown (called idiopathic pulmonary hypertension). Pulmonary hypertension can be inherited or caused by other conditions, such as:

• Autoimmune diseases: including scleroderma and rheumatoid arthritis
• Blood disorders: including bleeding and clotting disorders and sickle cell disease
• Genetic disorders: such as lysosomal storage diseases
• Heart problems present at birth: also called congenital heart defects
• Heart disease: such as heart failure and aortic valve disease
• Infections: such as HIV
• Liver disease: including cirrhosis of the liver
• Lung diseases: including COPD and pulmonary fibrosis
• Medicines: such as diet drugs or toxins
• Sleep-disordered breathing: also called sleep apnea

If you or your doctor suspects pulmonary hypertension, visit with a pulmonologist (lung specialist) at the University of Miami Health System. The earlier we can start treatment, the better.

Test

Cardiology diagnostic tests
Your doctor may request diagnostic heart tests to look for heart problems related to pulmonary hypertension. These include an electrocardiogram (EKG or ECG), echocardiogram (ultrasound of the heart), or cardiac catheterization (a procedure that involves a flexible tube and a tiny camera passed through a blood vessel in the groin or arm to access the heart).

Exercise stress test
Your doctor may order an exercise test to measure lung function before, during, and after physical activity, using a treadmill or stationary bike.

Medical imaging
Chest X-rays and advanced diagnostic imaging, such as CT (computed tomography) or MRI (magnetic resonance imaging) scans, detect problems in the structure, function, and blood flow of the heart, lungs, and blood vessels.

Pulmonary function tests
Pulmonary function tests measure lung functioning. They show how much air your lungs can hold, how quickly air moves in and out, and how well the carbon dioxide/oxygen gas exchange works. Methods include spirometry, or a mouthpiece attached to a machine, and plethysmography, performed in a box that looks like a phone booth.

Ventilation/perfusion scan (V/Q)
A lung ventilation/perfusion scan (V/Q scan) is a nuclear imaging test that measures breathing (ventilation) and circulation (perfusion). The procedure involves inhaled and injected radioactive tracers to show airflow and blood flow in the lungs. The scan can detect blood clots and damage to blood vessels.

Treatments

Medicine therapy
Oral medications can improve blood flow, lower blood pressure, relax the heart muscles, or improve heart functioning. These include blood thinners, diuretics (get rid of water and salt in the body), calcium channel blockers (prevent calcium from entering the heart and blood vessel walls), and vasodilators (widen blood vessels).

Oxygen therapy
If blood oxygen levels are too low, your doctor may prescribe inhaled oxygen as needed, such as before exercise or long-term for most of the day. There are different devices available. Many are portable, meaning they’re easy to move or take with you on the go.

Pulmonary rehabilitation
Pulmonary rehabilitation programs involve support, education, exercise, and nutrition counseling and training to help you live better with COPD. Our programs are personalized to your needs, and focus on keeping you active and enjoying life.

Surgery
Atrial septostomy is a form of cardiothoracic surgery that relieves the pressure in the right side of the heart, improving blood flow to the lungs. It’s an open-heart surgery that involves making a small hole in the wall between the upper left and right chambers of the heart, called the atria.

Transplant
If your condition is severe and doesn’t respond well to other treatments, a lung transplant may be an option.

Why Choose UHealth?

Advanced diagnostics and care from specialists who know your condition. Pulmonary hypertension can go unnoticed in the early stages, or be confused with other heart or lung conditions. It’s good to know we use the most advanced heart and lung tests and technology to diagnose your condition as soon as possible. Early diagnosis means you’ll begin the right treatment sooner.

Multispecialty care with heart doctors, surgeons, and if needed, transplant teams. Our pulmonologists work closely with heart experts (cardiologists, cardiothoracic surgeons) and Miami Transplant Institute’s lung (and heart-lung) transplant team. You get comprehensive care from specialists with expertise in all stages of pulmonary hypertension, within the same system in South Florida