Description

Pulmonary hypertension is a type of high blood pressure that affects arteries in the lungs and in the right side of the heart.

In one of the forms of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs become narrowed, blocked or destroyed. The damage that makes it difficult for blood to move through the lungs. The blood pressure in the arteries of the lung increases. The heart must work harder to pump blood through the lungs. The additional effort that eventually causes the heart muscle to become weak and not.

In some people, the pulmonary hypertension worsens slowly. It can be deadly. There is No cure for pulmonary hypertension. But treatments are available to help you to feel better, live longer, and improve their quality of life.

Symptoms

The symptoms of pulmonary hypertension are slow to develop. You may not notice them for months or even years. The symptoms get worse as the disease continues.

Pulmonary hypertension symptoms, which include:

  • Shortness of breath. You can first start during the year and get to happen in the rest.
  • Blue or gray skin. Depending on the color of the skin, these changes may be more difficult or more easy to see.
  • Chest pressure or pain.
  • Dizziness or fainting.
  • Pulse, fast or pounding heartbeat.
  • Fatigue.
  • Swelling in the ankles, legs, and the belly of the area.

These symptoms can be caused by other health conditions. Consult a health care professional for an accurate diagnosis.

Causes

Pulmonary hypertension is caused by changes in the cells that line the lung arteries. The changes can cause the walls of the arteries to narrow, stiffness, swelling and thick. It makes it more difficult for blood to flow through the lungs.

Pulmonary hypertension is classified into five groups, depending on the cause.

Group 1: Pulmonary arterial hypertension (PAH)

The causes are:

  • Of unknown cause, called idiopathic pulmonary arterial hypertension.
  • Changes in a gene that is passed down from parents to children, called heritable pulmonary arterial hypertension.
  • The use of certain medications or illicit drugs, including methamphetamine.
  • Condition of the heart that are present at birth is called a congenital defect of the heart.
  • Other health conditions, including scleroderma, lupus, and cirrhosis.

Group 2: Pulmonary hypertension caused by left heart disease

This is the most common form of pulmonary hypertension. The causes are:

  • Left-sided heart failure.
  • On the left side of the heart valves, including the mitral valve or aortic valve disease.

Group 3: Pulmonary hypertension caused by pulmonary disease

The causes are:

  • Scarring of the lungs, called pulmonary fibrosis.
  • Chronic obstructive pulmonary disease, also called COPD.
  • A sleep disorder in which breathing repeatedly stops and starts, it is called sleep apnea.
  • Being at high altitudes for prolonged periods of time, if you are at high risk of pulmonary hypertension.

Group 4: Pulmonary hypertension caused by obstruction in the pulmonary artery

The causes are:

  • Blood clots in the lungs that does not go away.
  • Tumors that block the pulmonary artery.

Group 5: Pulmonary hypertension caused by other health conditions

The causes are:

  • Blood disorders, such as polycythemia vera and essential thrombocythemia.
  • Inflammatory disorders such as sarcoidosis.
  • Conditions that affect the body's ability to break down certain sugars, including glycogen storage disease.
  • Kidney disease.

The Eisenmenger syndrome and pulmonary hypertension

The Eisenmenger syndrome can lead to pulmonary hypertension.

The Eisenmenger syndrome is a long-term complication of an unrepaired condition of the heart present at birth. An example is a large hole in the heart between the two lower heart chambers is called a ventricular septal defect.

To repair the hole in the heart causes the oxygen-rich blood to mix with oxygen-poor blood. The blood then reaches the lungs instead of going for the rest of the body. This increases the pressure in the pulmonary arteries.

Risk factors

Pulmonary hypertension is usually seen in people between the ages of 30 to 60 years. Aging can increase the risk of developing the Group 1 pulmonary hypertension, called pulmonary arterial hypertension (PAH). PAH cause is unknown, it is more common in young adults.

Other things that may increase the risk of pulmonary hypertension are:

  • A family history of the disease.
  • The overweight.
  • The habit of smoking.
  • Blood-clotting disorders or a family history of blood clots in the lungs.
  • A history of around asbestos.
  • A heart condition present at birth is called a congenital defect of the heart.
  • Living at a height of 8,000 feet (2,438 meters) or more.
  • The use of some medications, such as those used for weight loss.
  • Illicit drugs such as cocaine or methamphetamine.

Complications

Possible complications of pulmonary hypertension are:

  • Right-sided heart enlargement and heart failure.Also called cor pulmonale, this condition causes the heart to the right lower chamber for more. The camera has to pump harder than usual to move the blood through a narrowing or blockage of the arteries of the lung. As a result, the heart of the walls to get thick. The right lower chamber of the heart extends to increase the amount of blood that can contain. These changes create more strain on the heart. Finally, the right lower chamber of the heart failure.
  • The blood clots. Pulmonary hypertension increases the risk of blood clots in the small arteries in the lungs.
  • Irregular heartbeat, also called arrhythmias. Pulmonary hypertension can cause changes in the heart rhythm, which can be deadly.
  • Bleeding in the lungs. Pulmonary hypertension can lead to life-threatening bleeding in the lungs and coughing up blood.
  • Complications in pregnancy. Pulmonary hypertension can be life threatening for the mother and the developing baby.

Right-sided heart enlargement and heart failure. Also called cor pulmonale, this condition causes the heart to the right lower chamber for more. The camera has to pump harder than usual to move the blood through a narrowing or blockage of the arteries of the lung.

As a result, the heart of the walls to get thick. The right lower chamber of the heart extends to increase the amount of blood that can contain. These changes create more strain on the heart. Finally, the right lower chamber of the heart failure.

Diagnosis

The pulmonary hypertension is hard to diagnose early. It is not often found during a routine physical examination. Even when pulmonary hypertension is the most advanced, their symptoms are similar to those of other heart and lung.

To diagnose pulmonary hypertension, a health care professional examines you and asks you about your symptoms. Normally you have questions about your medical and family history.

Tests

Tests to diagnose pulmonary hypertension may include:

  • Blood tests. Blood tests may help find the cause of the pulmonary hypertension. The test can also help to find the complications of the disease.
  • X-ray of the chest. An x-ray is an image of the heart, the lungs and the chest. Can be used to detect other diseases of the lungs that can cause pulmonary hypertension.
  • Electrocardiogram (ECG or EKG). This simple test records the electrical activity of the heart. It is shown how the heart is beating.
  • The echocardiogram.Sound waves create images of the beating heart. An echocardiogram shows how blood flows through the heart and the heart valves. This test can be done to help diagnose pulmonary hypertension or to learn how the treatments are working. Sometimes, an echocardiogram is done while exercising on a stationary bike or treadmill to learn how the activity affects the heart. If you have this test, you may be asked to wear a mask that checks how well the heart and lungs use oxygen and carbon dioxide.
  • Right heart catheterization.If an echocardiogram shows pulmonary hypertension, this test may be done to confirm the diagnosis. During this procedure, a doctor inserts a thin, flexible tube called a catheter into a blood vessel, usually in the neck. The tube is gently guided in the lower right chamber of the heart and the pulmonary artery. The doctor can measure the blood pressure in the main pulmonary artery and the right ventricle.

The echocardiogram. Sound waves create images of the beating heart. An echocardiogram shows how blood flows through the heart and the heart valves. This test can be done to help diagnose pulmonary hypertension or to learn how the treatments are working.

Sometimes, an echocardiogram is done while exercising on a stationary bike or treadmill to learn how the activity affects the heart. If you have this test, you may be asked to wear a mask that checks how well the heart and lungs use oxygen and carbon dioxide.

Right heart catheterization. If an echocardiogram shows pulmonary hypertension, this test may be done to confirm the diagnosis.

During this procedure, a doctor inserts a thin, flexible tube called a catheter into a blood vessel, usually in the neck. The tube is gently guided in the lower right chamber of the heart and the pulmonary artery. The doctor can measure the blood pressure in the main pulmonary artery and the right ventricle.

Other tests may be done to check the condition of the lungs and the pulmonary arteries. The following tests can give you more information about the cause of the pulmonary hypertension:

  • The stress tests. These tests often involve walking on a treadmill or riding a stationary bike while the beat of the heart is seen. It can show how the heart responds to exercise.
  • Computed tomography (CT scan).This test uses X-rays to create images of specific parts of the body. Dye called contrast material may be administered into a vein to help the blood vessels show up more clearly in the images. A ct scan of the heart, called cardiac ct scan, it can show the size of the heart and the locks in the pulmonary arteries. May help diagnose lung diseases that can lead to pulmonary hypertension. Examples are COPD or pulmonary fibrosis.
  • Magnetic resonance imaging (MRI). This test uses magnetic fields and radio waves to take detailed pictures of the heart. You can display the flow of blood in the pulmonary arteries. The test may be done to learn the right lower chamber of the heart is working.
  • Pulmonary function test. For this test, you blow into a special device. The device measures the amount of air that the lungs can hold. It is shown how the air enters and leaves the lungs.
  • Sleep study. A sleep study measures brain activity, heart rate, blood pressure, oxygen levels, and other things like sleep. The test can help diagnose sleep apnea, which can cause pulmonary hypertension.
  • Ventilation/perfusion (V/Q) scanning. In this test, a radioactive tracer is given through a vein (IV). The scoreboard shows how the blood flows. You also can breathe in a marker that shows the flow of air to the lungs. A V/Q scan can tell if the blood clots are the cause of the symptoms of pulmonary hypertension.
  • Lung biopsy. Rarely, a tissue sample can be taken from the lungs to check for a possible cause of pulmonary hypertension.

Computed tomography (CT scan). This test uses X-rays to create images of specific parts of the body. Dye called contrast material may be administered into a vein to help the blood vessels show up more clearly in the images.

A ct scan of the heart, called cardiac ct scan, it can show the size of the heart and the locks in the pulmonary arteries. May help diagnose lung diseases that can lead to pulmonary hypertension. Examples are COPD or pulmonary fibrosis.

Genetic testing

The detection of changes in the genes that cause pulmonary hypertension may be recommended. If you have these changes in the genes, other members of the family may need to be screened.

Pulmonary hypertension functional classification

Once the diagnosis of pulmonary hypertension is confirmed, the condition is classified according to how the symptoms affect you and your ability to perform everyday tasks.

Pulmonary hypertension can fall into one of the following groups:

  • Class I. the Pulmonary hypertension is diagnosed, but there are no symptoms during rest or exercise.
  • Class II. There are No symptoms at rest. The daily tasks or activities such as going to work or to the grocery store can cause some shortness of breath, or a slight pain in the chest. There is a slight limitation of physical activity.
  • Class III. It is comfortable at rest, but doing simple tasks, such as bathing, dressing, or preparing meals, cause fatigue, shortness of breath and chest pain. The ability to perform physical activity is very limited.
  • Class IV. The symptoms occur at rest and during physical activity. Any type of activity leads to increased unrest.

Your health care team may use a risk calculator that looks at the symptoms and the results of the test to understand what type of treatment is needed. This is called pulmonary hypertension risk stratification.

Treatment

There is No cure for pulmonary hypertension. But treatments can improve symptoms and help you live longer. The treatment can also help prevent the disease from getting worse.

Often it takes some time to find the best treatment of pulmonary hypertension. The treatments are often complex. You usually need a lot of health screenings.

Drugs

If you have pulmonary hypertension, you may receive medications to treat your symptoms and help you feel better. Medications can also be used to treat or prevent complications. Treatment may include:

  • Medications to relax the blood vessels, called vasodilators.These medications expand the blood vessels and improve blood flow. The medication can be inhaled, taken by mouth or intravenously. Sometimes, it is administered continuously through a small pump connected to the body. Examples of vasodilators to treat pulmonary hypertension, including epoprostenol (Flolan, Veletri), treprostinil (Remodulin, Tyvaso, others), iloprost, and selexipag (Uptravi).
  • Soluble guanylate cyclase (sGC) stimulators. This type of medication relaxes the pulmonary arteries and reduces the pressure in the lungs. An example is riociguat (Built). Do not take these medications if you are pregnant.
  • Drugs to widen the blood vessels. Medicines called antagonists of endothelin receptors to reverse the effect of a substance in the walls of the blood vessels that causes the narrowing. These medications include bosentan (Tracleer), macitentan (Opsumit) and ambrisentan (Letairis). It may improve your energy level and symptoms. Do not take these medications if you are pregnant.
  • Medicines to increase blood flow. Medicines called phosphodiesterase 5 (PDE5) inhibitors can be used to increase the flow of blood through the lungs. These drugs also are used to treat erectile dysfunction. They include sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Alyq, Cialis).
  • High-dose calcium channel blockers. These drugs help relax the muscles in the walls of the blood vessels. They include amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension to improve, while taking them.
  • A blood thinner. Also called blood thinners, these medicines help prevent blood clots. An example is warfarin (Jantoven). The drugs may increase the risk of bleeding. This is especially true if you are having surgery or a treatment that enters the body or creates an opening in the skin. Talk with your health care team about your risk.
  • Digoxin (Lanoxin). This medicine helps the heart to beat stronger and pump more blood. You can help control irregular heartbeats.
  • Water pills, also called diuretics. These medicines help the kidneys remove excess fluid from the body. This reduces the amount of work the heart has to do. Diuretics can also be used to reduce the accumulation of fluid in the lungs, legs, and belly area.
  • Oxygen therapy. The breathing of pure oxygen may be suggested if you live in a high altitude or have sleep apnea. Some people with pulmonary hypertension, need for oxygen therapy all the time.

Medications to relax the blood vessels, called vasodilators. These medications expand the blood vessels and improve blood flow. The medication can be inhaled, taken by mouth or intravenously. Sometimes, it is administered continuously through a small pump connected to the body.

Examples of vasodilators to treat pulmonary hypertension, including epoprostenol (Flolan, Veletri), treprostinil (Remodulin, Tyvaso, others), iloprost, and selexipag (Uptravi).

Surgery or other procedures

If medicines do not help to control the symptoms of pulmonary hypertension, surgery may be recommended. Surgeries and procedures for the treatment of pulmonary hypertension may include:

  • Septostomy headset. This treatment can be done if the medications don't control your pulmonary hypertension symptoms. In a septostomy atrial, a doctor creates an opening between the upper left and right chambers of the heart. The opening is reduced, the pressure in the right side of the heart. The potential complications include irregular heartbeats called arrhythmias.
  • Lung or heart-lung transplantation. Sometimes, a lung or heart-lung transplantation may be necessary, especially for younger people who have idiopathic pulmonary arterial hypertension. After a transplant, the medicine should be taken for life to prevent the body from rejecting the new organ.

Lifestyle and home remedies

Lifestyle changes can help improve symptoms of pulmonary hypertension. Try these tips:

  • Eat healthy. Eating a healthy diet rich in whole grains, fruits and vegetables, lean meats and low-fat dairy products. Try to stay away from saturated fats, trans fats, and cholesterol. Use less salt.
  • Stay as active as possible and control the weight. Even mild forms of activity might be too strenuous for some people who have pulmonary hypertension. For others, the moderate exercise, such as walking, can be helpful, especially when done during oxygen therapy. Your health care team can help you plan a proper exercise program.
  • Do not smoke. If you smoke, stop smoking. If you need help, ask your health care team to treatments that can help. Avoid second-hand smoke also, if it is possible.
  • Get enough rest. In rest, it can reduce the fatigue associated with pulmonary hypertension.
  • Avoid high altitudes. High altitudes can make pulmonary hypertension worse. If you live in a height of 8,000 feet (2,438 meters) or more, you might be told to consider moving to a lower altitude.
  • Avoid activities that may lower blood pressure too much. These include sitting in a hot tub or in the sauna, or taking long hot baths or showers. Such activities can lower blood pressure and can cause fainting. In addition, do not perform activities that cause a lot of effort, such as lifting heavy objects or weights.
  • Tell your health care team about all the medicines you take. Some medicines can cause pulmonary hypertension worse or affect your treatment.
  • Get health checkups. Tell your health care team about any new or worsening symptoms or medication side effects. If the pulmonary hypertension affects their quality of life, ask about the treatments that might help.
  • Get the recommended vaccines. Respiratory infections can cause serious health problems for people with pulmonary hypertension. Ask your health care team the shots that you need to prevent the common viral infections.
  • Talk with a health care professional before you get pregnant. Pulmonary hypertension can cause serious complications for the pregnant person and the fetus, also called a fetus. Birth control pills can increase the risk of blood clots. Talk with your health care team about other options of birth control.

Coping and support

You may find that talking with other people who have pulmonary hypertension brings comfort and encouragement. Ask your health care team if there are any support groups in your area.

Preparing for your appointment

If you think you are at risk of or that may have pulmonary hypertension, to make an appointment for a health checkup.

There's often A lot to discuss at your appointment, so it's a good idea to be prepared. Here's some information to help you prepare for your appointment.

What you can do

  • Be aware of any preappointment restrictions. When you make your appointment, ask if there is something that you need to do before your check-up. For example, you might be told not to eat or drink before certain medical tests.
  • Write down the symptoms you are experiencing, including any that may not seem to be related to pulmonary hypertension. Try to remember when they started. To be specific, such as days, weeks, and months.
  • Make a list of important personal information. Include a family history of pulmonary hypertension, lung disease, heart disease, stroke, high blood pressure or diabetes. Also list any major stresses or recent life changes.
  • Make a list of all the medicines that you take. Also include vitamins, herbal products, supplements and any medication that you have bought without a prescription.
  • Take someone with you, if possible. Someone who goes with you can help you remember the information they give you.
  • Be prepared to discuss your diet and exercise habits. If you do not already follow a diet or exercise routine, talk with your health care team about the challenges that you might face in the introduction.
  • Make a list of questions to ask your health care team. A list of questions from most important to least important in case time runs out.

For pulmonary hypertension, some questions to ask your health care team are:

  • What is the likely cause of my symptoms or condition?
  • What are other possible causes?
  • What tests do I need?
  • What treatment do you recommend it?
  • What are the other treatment options?
  • There is a generic form of the medication you are prescribing?
  • What is an appropriate level of physical activity?
  • Are there any restrictions that I need to follow?
  • How often do I have any health examinations?
  • I have other health conditions. How can I best manage them together?
  • You should see a specialist?
  • Is there any information that I can take home? What websites do you suggest?

Do not hesitate to ask other questions.

What to expect from your doctor

Your health care team may ask you many questions. Be prepared to respond to them can give more time to discuss any concerns. You may ask:

  • When did you first start having symptoms?
  • Do you always have symptoms, or come and go?
  • On a scale of 1 to 10, with 10 being the worst, how bad are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

What you can do in the meantime

It is never too late to make changes for healthy lifestyle. Do not smoke, eat less salt, and choose nutritious foods. These changes can help prevent pulmonary hypertension worse.

Symptoms and treatment of Pulmonary hypertension