What is pulmonary hypertension? Pulmonary hypertension is high blood pressure in the arteries going from the heart to the lungs. To understand the disease, it helps to review blood circulation. Blood returns, in veins, from the body’s organs and tissues carrying carbon dioxide and lacking oxygen. Venous blood enters the right side of the heart where the lower chamber—the right ventricle—pumps it to the lungs through the pulmonary arteries. In the lungs, blood gets rid of carbon dioxide and picks up fresh oxygen. Then, it returns to the left side of the heart where the lower chamber—the left ventricle—pumps it back out to the body through arteries. This sequence of events happens with each heartbeat. It takes a lot of pressure for the left side of the heart to pump blood out to the entire body. A normal blood pressure for this work is 120/80 mm Hg or lower. This is the pressure your doctor measures in your arm with a blood pressure cuff. But the distance from the right side of the heart to the lungs is much shorter. Because the pressure to do this work is much lower, a normal pressure in the pulmonary arteries is about 25/10 mm Hg. Pulmonary hypertension is a pressure of 40/20 mm Hg or higher. Pulmonary hypertension is rare. Directly measuring the pressure in your pulmonary arteries requires an invasive procedure, so doctors do not routinely measure this pressure. If you develop pulmonary hypertension symptoms, your doctor will run various diagnostic tests. Early in the disease, symptoms are usually vague. Many times, people do not notice them. As the disease progresses, symptoms become noticeable. They include shortness of breath, fatigue, and swelling in the ankles and legs. Pulmonary hypertension can develop without a known cause. This is idiopathic or primary pulmonary hypertension. The risk of pulmonary hypertension increases with age and if you have a family history of the disease. It is also more common in women, smokers, and people who use illegal drugs, such as cocaine. It can also occur as a result of another medical condition, such as lung disease. This is secondary pulmonary hypertension. Currently, there is no cure for pulmonary hypertension. Pulmonary hypertension treatment focuses on relieving symptoms and slowing the progress of the disease. Doctors use medications and lifestyle changes to accomplish these goals. In some cases, lung or heart-lung transplantation may be an option. Left untreated, pulmonary hypertension can be fatal. See your doctor promptly if you have new or unusual symptoms. Seek immediate medical care if you have potentially serious symptoms, such as chest pain or pressure, severe shortness of breath, or a bluish color to your skin, lips or nails. What are the symptoms of pulmonary hypertension? In the early stages, pulmonary hypertension causes few, if any, noticeable symptoms. As the disease progresses, it becomes harder for the heart to pump blood through the lungs against the increased pressure. Eventually, the heart muscle weakens, leading to heart failure. This heart failure causes many pulmonary hypertension symptoms. Common symptoms of pulmonary hypertension include: Cyanosis, which is a bluish discoloration of the skin, lips or nails Dizziness, lightheadedness or fainting Edema, which is swelling of the ankles and feet, and ascites, which is swelling in the abdomen Fatigue, weakness, and lack of energy Heart palpitations and chest discomfort Shortness of breath that eventually occurs even at rest Serious symptoms that might indicate a life-threatening condition The symptoms of pulmonary hypertension are similar to several other conditions, some of which can be life threatening. Seek immediate medical care (call 911) if you, or someone you are with, have any of these life-threatening symptoms including: Chest pain, pressure, squeezing, tightness or other discomfort Extreme sweating Nausea and vomiting Pain in the jaw, neck, shoulders, back or arms that radiates from the chest or occurs by itself Severe shortness of breath, wheezing, or labored breathing Sudden anxiety Regular medical care can help identify early signs and symptoms of a disease. However, pulmonary hypertension can look a lot like other diseases, such as asthma or COPD (chronic obstructive pulmonary disease). This makes it very difficult to diagnose the disease. Cardiologists and pulmonologists specialize in diagnosing and treating this disease. What causes pulmonary hypertension? There are five different classes of pulmonary hypertension depending on the cause: Group 1: Pulmonary arterial hypertension (PAH). This group includes idiopathic, inherited, congenital, and drug-, HIV-, and connective tissue disease-related forms. Group 2: Pulmonary hypertension due to left-sided heart failure. Valve disease or heart failure on the left side of the heart can cause a backup of blood in the lungs. This leads to increased pressure in the lungs. Group 3: Pulmonary hypertension due to lung disease. This includes such causes as COPD, pulmonary fibrosis, sleep apnea, and chronic high altitude exposure. Group 4: CTEPH (chronic thromboembolic pulmonary hypertension). Chronic blood clots in the lungs block blood flow and increase pressure in the lungs. Group 5: Pulmonary hypertension due to other causes. In this form, doctors can link pulmonary hypertension to another disease. Causes include metabolic disorders, sarcoidosis, and blood disorders, such as sickle cell disease. It’s important to know the specific type of pulmonary hypertension. It can guide treatment and other aspects of care. This is another reason it is necessary to see a specialist with experience diagnosing and treating the disease. What are the risk factors for pulmonary hypertension? Several factors increase the risk of developing pulmonary hypertension. However, not all people with risk factors will get the disease. Risk factors for pulmonary hypertension include: Aging Being female Being overweight Having a disease that can lead to pulmonary hypertension Having a family history of pulmonary hypertension Smoking Using certain appetite-suppressant medicines, cancer chemotherapies, or illegal drugs, such as cocaine Reducing your risk of pulmonary hypertension It is not always possible to prevent pulmonary hypertension. Risk reduction and prevention focuses on protecting yourself from risk factors you can control. You may be able to lower your risk of pulmonary hypertension by: Avoiding medications known to cause it Effectively treating and controlling medical conditions that can cause it, such as lung disease and heart disease Stopping smoking If you are at risk for pulmonary hypertension, talk with your doctor about screening. Depending on your medical history, your doctor may recommend tests to look for early changes in your heart and lungs. Spotting these changes may lead to early diagnosis. This offers the best chance of slowing progression and preventing complications. How is pulmonary hypertension treated? Treatment depends on the type of pulmonary hypertension and how advanced it is. In most cases, pulmonary hypertension is not curable. CTEPH is the exception. In certain people with CTEPH, surgery to remove the blood clots can reverse the disease. However, not all people are candidates for the procedure. In all other cases, doctors will treat any condition contributing to pulmonary hypertension. This can help slow the disease by controlling the underlying cause. Treatment can also help relieve symptoms and keep them from getting worse. Pulmonary hypertension treatment options include: Anticoagulants to prevent blood clots Digoxin to help the heart pump better Diuretics to relieve swelling and fluid retention Healthy lifestyle changes, including eating a heart-healthy diet, staying active, and stopping smoking Inhaled medicines to relieve shortness of breath Medicines to relax and widen your blood vessels, including oral, IV (intravenous), and under the skin (subcutaneous) routes Oxygen therapy Physical therapy to improve your ability to exercise Your doctor will likely ask you to monitor your weight closely. Rapid weight gain can be a sign of worsening pulmonary hypertension. In some cases, lung or heart-lung transplantation is an option. Doctors mainly recommend this for younger people with idiopathic pulmonary hypertension (no identifiable cause). What are the potential complications of pulmonary hypertension? Pulmonary hypertension can be fatal or lead to serious complications, including heart failure, blood clots in the lungs, arrhythmias, bleeding into the lungs, and liver damage. Pulmonary hypertensive crisis is another potential complication. It happens when lung pressures increase so drastically that the heart can’t effectively pump blood at all. It typically occurs in the setting of hospitalization for heart surgery.