Pulmonary hypertension makes your heart work harder to pump blood through your lungs. This can trigger a number of disruptive symptoms and, eventually, can cause the right side of your heart to become enlarged and unable to adequately pump blood. Damage to your heart from pulmonary hypertension can lead to serious and even life-threatening complications. (1,2) Pulmonary hypertension may cause these symptoms:
Shortness of breath (at first with physical activity but then at other times as the disease progresses)Fatigue or weaknessReduced appetiteCough (may be dry or produce blood)Light-headedness, dizziness, or faintingChest pain or pressureSwelling in the ankles, legs, or abdomenRacing heartbeat or heart palpitationsNausea, abdominal pain, or vomitingHoarseness or wheezingBluish color to the skin or lips (3)
In some people with a family history of pulmonary hypertension, a gene mutation has been identified that researchers believe contributes to the disorder. But some people with no known genetic factors also develop pulmonary hypertension. (1) Health conditions that may cause or contribute to pulmonary hypertension include these issues:
Blood clots in the lungs (pulmonary embolism)Tumors in the lungsBeing overweight or obeseChronic kidney diseaseLiver disease (especially with cirrhosis)Left-sided heart disease (which may be caused by hypertension, or regular high blood pressure)Congenital heart defectsHIVHepatitis B or hepatitis CParasitic infectionScleroderma and other connective tissue disordersSarcoidosisMetabolic disorders, including thyroid diseaseSickle cell diseaseSurgical removal of the spleenScoliosis (1,2)
Certain other factors may contribute to pulmonary hypertension, as well:
Family history of the conditionLiving at a high altitudeOlder age (age at diagnosis is typically 30 to 60)Being a woman (higher risk for the condition)Exposure to asbestos or silicaTaking certain weight loss drugs (especially fenfluramine and dexfenfluramine)Taking a selective serotonin reuptake inhibitor for depression or anxietyTaking certain chemotherapy drugs for cancerSmokingUsing certain illegal drugs, including cocaine (1,2)
The following tests may be used for pulmonary hypertension diagnosis: Blood Tests These tests can help identify certain causes of pulmonary hypertension, such as an increased risk of blood clots. Chest X-Ray This test can show enlargement of the heart or pulmonary arteries, which are signs that pulmonary hypertension may be present. Electrocardiogram This test can identify abnormal heart rhythms that may be caused by pulmonary hypertension. Echocardiogram This test uses sound waves to create moving images of your heart. It can be used to estimate your pulmonary blood pressure and make an initial diagnosis of pulmonary hypertension. Cardiac Catheterization (Right Heart Catheterization) This invasive test involves inserting a thin tube into your neck or groin and threading it into your pulmonary artery, allowing your doctor to directly measure your blood pressure in the area. Other tests may be used to assess your lung function or determine the causes of pulmonary hypertension. (1,2,3)
Types of Pulmonary Hypertension
As part of diagnosing pulmonary hypertension, your doctor will try to determine the cause of the condition. Based on these findings, your condition may be classified as being in one of five groups: Group 1: Pulmonary Arterial Hypertension (PAH) PAH is due to problems with the structure of the pulmonary arteries and may be related to other diseases or have no known cause. It’s sometimes called primary pulmonary hypertension. Group 2: Caused by Left-Sided Heart Disease This type of pulmonary hypertension can result from mitral valve or aortic valve disease, or long-term regular high blood pressure. Group 3: Caused by Lung Disease This type of pulmonary hypertension can be caused by chronic obstructive pulmonary disease, obstructive sleep apnea, or other lung disorders. Group 4: Caused by Blood Clots This type of pulmonary hypertension is typically caused by blood clots in your lungs or other clotting disorders. In chronic thromboembolic pulmonary hypertension, a rare form of the condition in this category, clots persist after three months of taking an anticoagulant. Group 5: Caused by Other Health Conditions A wide range of other health conditions may cause or contribute to pulmonary hypertension. (2,3,4) A severe type of pulmonary hypertension that affects newborn babies, called persistent pulmonary hypertension of the newborn (PPHN), is considered separate from each of the groups that are diagnosed in adults. PPHN is usually diagnosed within 72 hours following birth and typically requires medications and oxygen therapy to reach a stable condition. (5)
Prognosis of Pulmonary Hypertension
Whether your pulmonary hypertension gets better or worse, and how quickly, depends largely on what’s causing it and other individual factors. There’s no direct cure for pulmonary hypertension, but in some people, the condition may be improved or resolved if it’s caused by a treatable underlying health condition. But in other people, pulmonary hypertension gets worse over time and may lead to life-threatening complications. You may be able to improve your symptoms, and prevent or delay the worsening of pulmonary hypertension, by taking drugs or following other treatments as prescribed and making recommended lifestyle changes. (1,2,3) RELATED: Your Definitive Guide to Heart Disease Unless your pulmonary hypertension is due to an underlying health condition that can be effectively treated, such as blood clots in your lungs, there’s a good chance you’ll be living with it for the rest of your life. There are no defined stages of pulmonary hypertension, and your condition may get better or worse or remain somewhat stable over time. For most people, treatment for pulmonary hypertension is aimed at improving your symptoms and preventing your condition from getting worse. By following treatment and lifestyle recommendations, you may be able to improve your health and quality of life. (1,2,3)
Medication Options
The following drugs may be prescribed as pulmonary hypertension medications: Vasodilators These drugs help relax blood vessels to increase blood flow in the pulmonary arteries. Vasodilators may be given by mouth or intravenously, or may be inhaled using a machine called a nebulizer. Anticoagulants These anticlotting drugs — warfarin (Coumadin, Jantoven) is the most common — may be prescribed if your condition is caused by blood clots in your lungs or if you have risk factors for blood clots. Digoxin (Cardoxin, Lanoxin) This drug can help strengthen your heartbeat and improve blood flow throughout your body, and may prevent some types of abnormal heart rhythms. (6) Diuretics Also known as water pills, these drugs can help reduce fluid buildup in your body and reduce the strain this extra fluid puts on your heart. Oxygen Using supplemental oxygen may be recommended to help you breathe if you have sleep apnea, live at a high altitude, or have severe pulmonary hypertension. (7)
Surgeries and Procedures
The following procedures may be recommended in some cases of pulmonary hypertension: Atrial Septostomy This procedure involves creating a small hole between the right and left atria of your heart to reduce pressure on the right side and increase oxygen in your blood. Pulmonary Angioplasty This procedure uses a balloon to help restore blood flow through your pulmonary arteries. Pulmonary Endarterectomy This surgery removes blood clots from the blood vessels of your lungs. Heart or Lung Transplantation A transplant may be an option for some younger people with pulmonary hypertension from unknown causes, but it carries significant risks and drawbacks. (1,2,7)
Alternative and Complementary Therapies
A number of lifestyle changes and alternative treatment programs may help you manage your pulmonary hypertension: Have a heart-healthy diet. Reducing salt in your diet may help reduce your regular blood pressure and improve your overall heart function by reducing fluid in your body, potentially leading to some improvement of symptoms. Getting a variety of nutrients from fruits, vegetables, whole grains, and lean protein can also improve your heart health. Engage in physical activity. Getting enough exercise, as recommended by your healthcare team, can improve your heart function. But don’t exert yourself more than is safe for your condition, and take breaks or rest when needed. Quit smoking. If you smoke, ask your doctor about programs to help you stop. Do a pulmonary rehabilitation program. This type of exercise and education program can help you stay physically active while experiencing less shortness of breath. It may be recommended if you have trouble with daily activities. (8) Routine screening tests for pulmonary hypertension are generally recommended only for people with known risk factors, such as scleroderma, a family history of the condition, or certain forms of lung or heart disease. (1)
Blood clots in the small arteries of the lungsAnemia (inadequate red blood cells)Right-sided heart enlargement and heart failureLiver damagePericardial effusion (fluid buildup around the heart)Bleeding in the lungs (potentially life-threatening)Pregnancy complications (potentially life-threatening to both a woman and her developing baby) (1,2)
Pulmonary Hypertension and COVID-19
While there’s no evidence that people with pulmonary hypertension are at a greater risk for developing COVID-19 (the new coronavirus infection), you are at a greater risk for developing serious illness if you get infected, according to the Pulmonary Hypertension Association. For this reason, you should take precautions to reduce your risk of infection, such as staying home whenever possible, maintaining physical distance from other people, washing your hands frequently, and not touching your face. Covering your face with a cloth mask or other covering while in public is also currently recommended by the Centers for Disease Control and Prevention, unless you have trouble breathing. You can talk to your doctor or use your own judgment to decide whether it’s safe for you to wear a cloth face covering. (9) While pulmonary hypertension is more common with older age, it affects people of all ages. It’s more common in women, non-Hispanic black people, and people over age 75 than in other groups in the United States. (11) Worldwide, the incidence of pulmonary hypertension appears to be growing, according to a study published in February 2018 in the journal Circulation: Cardiovascular Quality and Outcomes. From 1993 to 2012, the number of people living with condition each year went from 99.8 to 127.3 per 100,000 people. The most common type of pulmonary hypertension is group 2 (caused by left-sided heart disease), making up 34.2 percent of cases, with an overlap of group 3 (caused by lung disease) that also meets criteria for group 2 making up another 29.3 percent. (12)
Blood clots in the lungsTumors in the lungsBeing overweight or obeseChronic kidney diseaseLiver disease (especially with cirrhosis)Left-sided heart disease (which may be caused by regular high blood pressure)Congenital heart defectsHIVHepatitis B or CParasitic infectionScleroderma and other connective tissue disordersSarcoidosisMetabolic disorders, including thyroid diseaseSickle cell diseaseSurgical removal of the spleenScoliosis (1,2)
The Pulmonary Hypertension AssociationPulmonary Hypertension webpage from the National Heart, Lung, and Blood InstitutePulmonary Hypertension webpage from the American Heart AssociationPulmonary Hypertension webpage from the Mayo Clinic
