Pulmonary Hypertension Overview
Pulmonary hypertension describes a type of high blood pressure that affects only the arteries of your lungs and the right side of your heart. A rare lung disease, pulmonary hypertension develops when tiny blood vessels in your lungs become narrowed, blocked or damaged, making it hard for blood to flow through your lungs. The resulting pressure causes the heart’s lower right chamber (right ventricle) to work harder and eventually weaken.
Pulmonary hypertension is a serious and sometimes fatal illness without a cure. Your Wellstar physician can prescribe treatments designed to lessen symptoms and improve your quality of life.
In most cases, the symptoms of pulmonary hypertension develop gradually. They may include:
- Shortness of breath
- Chest pains
- Racing pulse
- Heart palpitations
- Fainting spells
In the early stages, the symptoms may not become noticeable for months or even years. As the disease progresses, you may experience a bluish color to your lips and skin (cyanosis) or swelling (edema) in your ankles, legs and abdomen.
Pulmonary hypertension may affect anyone, although variations of the disease with known causes typically affect older adults. Pulmonary hypertension of unknown origins (idiopathic pulmonary hypertension) is more common in younger people. It is as much as three times more likely in women than in men. Several other factors make you more likely to develop pulmonary hypertension as well. These include:
- Family history of the disease
- Personal history of heart, lung, liver diseases, blood clots or HIV infection
- Excessive use of certain diet medications or drugs, such as cocaine
Pulmonary Hypertension Prevention
In general, making healthy lifestyle changes, such as quitting smoking, cutting down on salt, eating healthy foods and regular exercise stand as the best preventive measures to avoid forms of this disorder.
Reduce Your Risk for Pulmonary Hypertension
Maintaining general good health with proper diet and exercise may lower your chances of developing pulmonary hypertension.
Tests and Screenings
Pulmonary hypertension can be difficult to diagnose early. Many signs and symptoms do not appear until months and, sometimes, years after the disorder develops. Even in more advanced stages of the disease, the signs and symptoms of a number of other pulmonary and heart diseases—such as chronic obstructive pulmonary disease (COPD), heart failure and asthma—mimic those of pulmonary hypertension, so your Wellstar physician needs to rule those out before making a diagnosis.
To diagnose pulmonary hypertension, your Wellstar physician will take a careful medical history and perform a physical examination. When pulmonary hypertension is suspected, your physician may order a variety of tests or procedures:
- Imaging, such as a chest x-ray, computerized tomography (CT) scan or magnetic resonance imaging (MRI). These imaging procedures help to eliminate other possible conditions causing signs and symptoms similar to those of pulmonary hypertension and to reveal details of your pulmonary arteries and the right ventricle of your heart.
- Genetic testing. If you have a family history of this condition, your physician might screen you for genes linked with pulmonary hypertension.
- Blood tests. These tests help rule out other possible diseases such as rheumatoid arthritis.
- Echocardiogram. This ultrasound test, which involves placing a transducer on your chest, shows the pumping performance of your heart and measures the size and size and thickness of your heart muscles as well as estimating the pressure in the vessels taking blood to the lung.
- Transesophageal echocardiogram. If it is difficult to get a clear picture of your heart and lungs, your physician may recommend this procedure in which a flexible tube outfitted with a transducer is guided down your throat to get detailed images of the heart and blood vessels.
- Right heart catheterization. If your doctor suspects pulmonary hypertension after examining the results of an echocardiogram, you might undergo this procedure—the most reliable way to diagnose the condition. During the procedure, a cardiologist places a catheter into a vein in your neck or groin and threads it into your right ventricle and pulmonary artery. This allows your doctor to directly measure the pressure in that part of your heart and to gauge how different medications may affect your condition.
- Lung function tests. These tests measure the volume of your lungs, the amount of air you breathe in and out, the rate of your breathing and the ability of your lungs to deliver oxygen to your blood.
- Exercise tests. These tests are another procedure to test lung function.
- Perfusion lung scan. This test uses small amounts of radioactive substances to study blood flow (perfusion) in your lungs. Immediately after injecting radioisotopes into a vein in your arm, the doctor uses a gamma camera to take pictures of the blood flow into your lungs. This test is often conducted along with a ventilation scan in which you inhale a small amount of radioisotopes while a gamma camera records the movement of air into your lungs.
- VATS lung biopsy. In rare situations, your physician may recommend this procedure to extract a small tissue sample of your lungs. VATS surgery uses a small camera to see inside the chest, while the surgery is done inside the chest through two small “band-aid” incisions. Advantages of this minimally invasive technique include:
- Less pain.
- Shorter hospital stay.
- Faster recovery and return to normal function.
- Lower infection rate.
- Lower complication rate including irregular heartbeats and pneumonia.
Pulmonary Hypertension Diagnosis
If screening tests or check-ups reveal abnormal results, your Wellstar physician will perform further evaluations regarding pulmonary hypertension. Your condition might receive a classification according to these guidelines based on an exercise test:
- Class Ⅰ—No limits. Condition comes with no symptoms.
- Class Ⅱ—Slight or mild limits. Condition exhibits no symptoms at rest, but fatigue, shortness of breath or chest pain develop with normal physical activity.
- Class Ⅲ—Marked or noticeable limits. Condition comes with comfortable feeling at rest, but symptoms occur with minimal physical activity.
- Class Ⅳ—Severe limits. Condition exhibits symptoms even at rest.
- Chest X-rays
- Computerized Tomography (CT) Scan
- Magnetic Resonance Imaging (MRI)
- Genetic testing
- Transesophageal echocardiogram
- Right heart catheterization
- Pulmonary function test
- Perfusion lung scan
- VATS lung biopsy
Pulmonary Hypertension Treatment
There is no known cure for pulmonary hypertension, so treatments—medications, surgery and lifestyle changes—focus on lessening the symptoms and keeping the condition from getting worse. Determining the best treatment for pulmonary hypertension may take time as methods are often complex or require change if a previous treatment becomes ineffective. If your condition is due to another underlying diagnosis, your doctor may treat the underlying cause whenever possible.
Medications currently available for pulmonary hypertension cannot cure the illness, but may relieve symptoms. As you use the medications, your Wellstar physician will monitor you closely to see how you respond to a particular drug and to ensure the medication and dosage treats your condition effectively. Medications may include:
- Blood-thinning medicines or anticoagulants, which help prevent blood clots from forming or getting larger. Warfarin (Coumadin®) is the most common anticoagulant used for pulmonary hypertension, but it must be used exactly as prescribed because of the risk of bleeding complications.
- Blood vessel medications. Drugs such as blood vessel dilators (vasodilators), endothelin receptor antagonists, sildenafil (Revatio®), ambrisentan (Letairis®) and calcium channel blockers are designed to open narrowed blood vessels or block substances in your body that causes them to narrow.
- Diuretics or “water pills.” These medications help eliminate excess fluid from your body, thus reducing the amount of work required by your heart and lungs.
- Oxygen treatment. Breathing pure oxygen may help patients particularly those who live in high altitudes or have sleep apnea.
If medications are not effective in controlling your pulmonary hypertension, surgery or transplantation of the heart, lungs or both may become options. With atrial septostomy, a Wellstar surgeon creates an opening between the left and right chambers of your heart to relieve pressure on the right side and allow the blood to circulate through your body without having to pass through the lungs first. This procedure can have complications because it reduces the amount of oxygen delivered to your body or can cause heart rhythm abnormalities called arrhythmias.
Ongoing Care for Pulmonary Hypertension
Wellstar’s world-class, community-based physicians utilize state-of-the-art medical centers and hospitals with the latest technologies and medical resources. Our clinicians are dedicated to:
- Helping you create and maintain healthy lifestyles.
- Offering advice to prevent illness and injuries.
- Providing early and appropriate care of acute illness to prevent its progression.
Pulmonary Medicine at Wellstar
Wellstar Pulmonary Network provides top-flight pulmonary care throughout the Wellstar Health System. Each of Wellstar’s physicians, certified in their field, have the knowledge and skill needed to diagnose and treat all pulmonary conditions and diseases, ranging from asthma, bronchitis, emphysema, pneumonia, respiratory failure, pulmonary hypertension, lung cancer and sleep apnea. In addition, many of our providers have special training in internal medicine, pulmonary diseases and critical care medicine. As a result, you can feel confident—at every level—about your pulmonary care at Wellstar.
Care at Home
Proper care and treatment of pulmonary hypertension can often improve symptoms and help you live longer. You and your Wellstar physician can work together to make your life more comfortable, so pay close attention to your body and your post-treatment regimen and keep your doctor updated. As part of your post-treatment care and in addition to maintaining a healthy diet and exercise, you should:
- Stop smoking.
- Seek prompt medical attention when symptoms increase in severity.
- Keep track of all medications you take.
- Keep track of your weight and blood pressure.
- Find ways to reduce stress.
- Avoid situations that can excessively lower blood pressure, such as taking long hot baths or sitting in hot tub or sauna.
- Avoid traveling or living at high altitudes as it can worsen the symptoms of pulmonary hypertension.
- Avoid using birth control pills or becoming pregnant, which can increase your risk of blood clots.
- Write down your questions, and never be afraid to ask your Wellstar physicians for clarification regarding your condition or your treatment.