Pulmonary Hypertension

Pulmonary hypertension (PH) is a condition caused by high blood pressure in the arteries that carry blood from your heart to your lungs. In patients with PH, the heart has to work harder to pump blood to the lungs. Over time, PH can weaken the heart and, ultimately, cause heart failure. Untreated, PH is almost always fatal.

Types of Pulmonary Hypertension

There are two main types of PH—primary and secondary.

Primary PH includes these subtypes:

• PH with no known cause
• PH that is inherited
• PH caused by drugs and toxins, including street drugs and some diet pills
• PH caused by certain conditions, including connective tissue diseases (s)uch as lupus and scleroderma, liver disease, congenital heart disease, sickle cell disease, schistosomiasis, and HIV
• PH caused by conditions affecting veins and small blood vessels in the lungs.

Secondary PH is a side effect that results from another condition, such as:

• Some types of heart disease
• Lung diseases, such as COPD and pulmonary fibrosis
• Sleep-related disorders, such as sleep apnea
• Blood clots in the lungs and blood-clotting disorders
• Blood, systemic, and metabolic diseases.

Pulmonary hypertension most commonly affects individuals between the ages of 20 and 60. It is unknown how many people have the disorder.

There is no cure for PH, but treatments and lifestyle changes can help control the symptoms and slow the progress of the disease.

Symptoms 

Pulmonary hypertension develops slowly, and people sometimes have the disease for years without knowing it. Symptoms may be similar to those of other heart or lung conditions, including:

• Shortness of breath during everyday activities
• Chest pain or rapid heartbeat
• Fatigue
• Pain on the upper right side of the abdomen
• Decreased appetite.

Some people experience swelling in the legs and ankles, light-headedness, bluish-colored lips and skin, and difficulty doing physical activity as the condition worsens.

Diagnosis

If PH is suspected when you visit Norton Thoracic Institute (NTI), you will receive a full examination to evaluate your physical health and family medical history, plus any of the following:

• Specialized exercise studies, such as a six-minute walk test, to determine the severity of your PH
• Imaging studies such as echocardiography, chest x-ray, electrocardiogram, CT scan, and MRI, as indicated
• Lung function testing to measure how well air moves into and out of your lungs, and how efficiently the lungs deliver oxygen to your body
• Blood tests to identify any autoimmune, liver, or infectious diseases that may contribute to your condition.

Treatment

Untreated, PH is nearly always a fatal disease. While there is no cure for PH, significant advancements in treatment options are offering hope to those with this condition.  NTI offers advanced treatment for PH designed to:

• Increase your quality of life and ability to exercise
• Improve and increase your breathing
• Slow the progression of the disease.

Your individualized treatment plan may include such therapies as:

• Medications to relax the blood vessels in your lungs, reduce fluid build-up in your body, prevent blood clots, or help your heart beat stronger
• Oxygen therapy to increase your oxygen levels
• Treatment for any underlying conditions causing your PH
• Lung transplantation, if your disease is severe. NTI offers Arizona’s premier lung transplant center—the largest and most experienced in Arizona and one of the largest lung transplant programs in the world. The Norton Lung Transplant Center is known for streamlined processes that speed up treatment and result in exceptional outcomes. 

Living with Pulmonary Hypertension

There is currently no cure for PH, but you can take steps to manage your disease:

• Keep up with regular doctor’s appointments and follow your plan of care, taking medications as directed.
• Call your doctor immediately if your symptoms worsen or change.
• Talk to your doctor before taking any over-the-counter medicines, as some may worsen your condition.
• Eat a healthy diet, and maintain a healthy weight. You may need to limit salt and fluids. If your weight increases suddenly, contact your doctor as this may indicate your condition is worsening.
• Talk to your doctor about getting a flu shot and pneumonia vaccine.
• Get plenty of rest.
• If you smoke, quit now. If you don’t smoke, don’t start. Avoid second-hand smoke.
• Talk to your doctor about how to exercise safely with your condition.
• If you are a woman of child-bearing age, please consult your physician before getting pregnant, since PH can make pregnancy risky.
• Talk to others about your condition, both in person and online. Let family and friends know how they can help, and connect with others with the disease to learn how they cope.

Clinical Trials/Research

Norton Thoracic Institute offers all FDA-approved agents, including subcutaneous and intravenous prostacyclin infusions. Patients also have the option of enrolling into investigational protocols and participating in cutting-edge research.

Learn About Our Pulmonary Hypertension Services 

To learn more about Norton Thoracic Institute's services, call (602) 406-4000.