Pulmonary hypertension
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension; Cor pulmonale - pulmonary hypertensionPulmonary hypertension is high blood pressure in the pulmonary arteries of the lungs. It makes the right side of the heart work harder than normal.
Causes
The right side of the heart pumps blood into the pulmonary artery and then through the lungs, where it picks up oxygen. Blood returns to the left side of the heart, where it is pumped to the rest of the body.
When the small arteries (blood vessels) of the lungs become narrowed, they cannot carry as much blood. When this happens, pressure builds up. This is called pulmonary hypertension.
The heart needs to work harder to force the blood through the vessels against this pressure. Over time, this causes the right side of the heart to become larger and malfunction. This condition is called right-sided heart failure, or cor pulmonale.
Cor pulmonale
Cor pulmonale is a condition that causes the right side of the heart to fail. Long-term high blood pressure in the pulmonary arteries of the lung an...
Read Article Now Book Mark ArticlePulmonary hypertension may be caused by:
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Autoimmune diseases that damage the lungs, such as scleroderma and rheumatoid arthritis
Autoimmune diseases
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...
Read Article Now Book Mark ArticleScleroderma
Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the cells that line the ...
Read Article Now Book Mark ArticleRheumatoid arthritis
Rheumatoid arthritis (RA) is a disease that leads to inflammation of the joints and surrounding tissues. It is a long-term disease. It can also aff...
Read Article Now Book Mark Article - Birth defects of the heart
- Blood clots in the lung (pulmonary embolism)
Pulmonary embolism
A pulmonary embolus is a blockage of an artery in the lungs. The most common cause of the blockage is a blood clot.
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Heart failure (of the left side of the heart)
Heart failure
Heart failure is a condition in which the heart is no longer able to pump oxygen-rich blood to the rest of the body efficiently. This causes symptom...
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- HIV infection
- Low oxygen levels in the blood for a long time (chronic)
Chronic
Chronic refers to something that continues over an extended period of time. A chronic condition is usually long-lasting and does not easily or quick...
Read Article Now Book Mark Article - Lung disease, such as COPD or pulmonary fibrosis or any other severe chronic lung condition
COPD
Chronic obstructive pulmonary disease (COPD) is a common lung disease. Having COPD makes it hard to breathe. There are two main forms of COPD:Chroni...
Read Article Now Book Mark ArticlePulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is scarring or thickening of the lungs without a known cause.
Read Article Now Book Mark Article - Medicines (for example, certain diet medicines)
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Obstructive sleep apnea
Obstructive sleep apnea
Obstructive sleep apnea (OSA) is a problem in which your breathing pauses during sleep. This occurs because of narrowed or blocked airways.
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In rare cases, the cause of pulmonary hypertension is unknown. In this case, the condition is called idiopathic pulmonary arterial hypertension (IPAH). Idiopathic means the cause of a disease is not known. IPAH affects more women than men.
If pulmonary hypertension is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.
Symptoms
Shortness of breath or lightheadedness during activity is often the first symptom. Fast heart rate (palpitations) may be present. Over time, symptoms occur with lighter activity or even while at rest.
Palpitations
Palpitations are feelings or sensations that your heart is pounding or racing. They can be felt in your chest, throat, or neck. You may:Have an unpl...
Read Article Now Book Mark ArticleOther symptoms include:
- Ankle and leg swelling
- Bluish color of the lips or skin (cyanosis)
Cyanosis
A bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.
Read Article Now Book Mark Article - Chest pain or pressure, most often in the front of the chest
- Dizziness or fainting spells
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Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
Read Article Now Book Mark Article - Increased abdominal size due to fluid buildup
- Weakness
People with pulmonary hypertension often have symptoms that come and go. They report good days and bad days.
Exams and Tests
Your health care provider will perform a physical exam and ask about your symptoms. The exam may find:
- Abnormal heart sounds
- Feeling of a pulse over the breastbone
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Heart murmur on the right side of the heart
Heart murmur
A heart murmur is a blowing, whooshing, or rasping sound heard during a heartbeat. The sound is caused by turbulent (rough) blood flow through the h...
Read Article Now Book Mark Article - Larger-than-normal veins in the neck
- Leg swelling
- Liver and spleen swelling
- Normal breath sounds if pulmonary hypertension is idiopathic or due to congenital heart disease
Congenital heart disease
Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth.
Read Article Now Book Mark Article - Abnormal breath sounds if pulmonary hypertension is from other lung disease
In the early stages of the disease, the exam may be normal or almost normal. The condition may take several months to diagnose. Asthma and other diseases may cause similar symptoms and must be ruled out.
Tests that may be ordered include:
- Blood tests
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Cardiac catheterization
Cardiac catheterization
Cardiac catheterization involves passing a thin flexible tube (catheter) into the right or left side of the heart. The catheter is most often insert...
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Chest x-ray
Chest x-ray
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
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CT scan of the chest
CT scan of the chest
A chest CT (computed tomography) scan is an imaging method that uses x-rays to create cross-sectional pictures of the chest and upper abdomen....
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Echocardiogram
Echocardiogram
An echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...
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Electrocardiogram (ECG)
Electrocardiogram
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
Read Article Now Book Mark Article - Lung function tests
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Nuclear lung scan
Nuclear lung scan
Lung gallium scan is a type of nuclear scan that uses radioactive gallium to identify inflammation in the lungs.
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Pulmonary arteriogram
Pulmonary arteriogram
Pulmonary angiography is a test to see how blood flows through the lung. Angiography is an imaging test that uses x-rays and a special dye to see th...
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- Sleep study
- Tests to check for autoimmune problems
Treatment
There is no cure for pulmonary hypertension. The goal of treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve problems.
Many treatment options for pulmonary arterial hypertension are available. If you are prescribed medicines, they may be taken by mouth (oral), received through the vein (intravenous, or IV), or breathed in (inhaled).
Intravenous
Intravenous means "within a vein. " Most often it refers to giving medicines or fluids through a needle or tube inserted into a vein. This allows th...
Read Article Now Book Mark ArticleYour provider will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you are responding to the medicine. Do not stop taking your medicines without talking to your provider.
Other treatments may include:
- Blood thinners to reduce the risk of blood clots, especially for some forms of pulmonary hypertension
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Oxygen therapy at home
Oxygen therapy at home
Because of your medical problem, you may need to use oxygen to help you breathe. You will need to know how to use and store your oxygen.
Read Article Now Book Mark Article - Lung transplant, or in some cases, heart-lung transplant, if medicines do not work
Other important tips to follow:
- Avoid pregnancy.
- Avoid heavy physical activities and lifting.
- Avoid traveling to high altitudes.
- Get a yearly flu vaccine, as well as other vaccines such as the pneumococcal (pneumonia vaccine), and the COVID-19 vaccine.
Flu vaccine
All content below is taken in its entirety from the CDC Inactivated Influenza Vaccine Information Statement (VIS) www. cdc. gov/vaccines/hcp/vis/vis-...
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Content below is taken in its entirety from the CDC Information Statement (VIS): www. cdc. gov/vaccines/hcp/vis/vis-statements/pcv. html
Read Article Now Book Mark ArticleCOVID-19 vaccine
COVID-19 vaccines are used to prepare the body's immune system to protect against COVID-19. Everyone ages 6 months and older should get a 2024-2025 C...
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Stop smoking.
Stop smoking
There are many ways to quit smoking. There are also resources to help you. Family members, friends, and co-workers may be supportive. But to be su...
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Outlook (Prognosis)
How well you do depends on what caused the condition. Medicines for IPAH may help slow the disease.
As the illness gets worse, you will need to make changes in your home to help you get around the house.
Make changes in your home
Pulmonary hypertension (PAH) is abnormally high blood pressure in the arteries of the lungs. With PAH, the right side of the heart has to work harde...
Read Article Now Book Mark ArticleFor people with severe disease, lung transplant, or in some cases, heart-lung transplant can be considered.
When to Contact a Medical Professional
Contact your provider if:
- You begin to develop shortness of breath when you are active
- Shortness of breath gets worse
- You develop chest pain
- You develop other symptoms
- You feel lightheaded or dizzy
- You have persistent palpitations
References
Lammi MR, Mathai SC. Pulmonary hypertension: general approach. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 83.
Maron BA. Pulmonary hypertension. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 88.
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Respiratory system - illustration
Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.
Respiratory system
illustration
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Primary pulmonary hypertension - illustration
Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. The goal of treatment is control of the symptoms, although the disease usually develops into congestive heart failure.
Primary pulmonary hypertension
illustration
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Heart-lung transplant - series - Normal anatomy
Presentation
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Respiratory system - illustration
Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.
Respiratory system
illustration
-
Primary pulmonary hypertension - illustration
Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. The goal of treatment is control of the symptoms, although the disease usually develops into congestive heart failure.
Primary pulmonary hypertension
illustration
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Heart-lung transplant - series - Normal anatomy
Presentation
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Pulmonary hypertension
(Alt. Medicine)
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Scleroderma - InDepth
(In-Depth)
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Chronic obstructive pulmonary disease
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Chronic obstructive pulmonary disease - InDepth
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Sickle cell disease - InDepth
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Stroke - InDepth
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Heart failure - InDepth
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Myocardial infarction
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Edema
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Alpha-linolenic acid
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Review Date: 5/3/2024
Reviewed By: Allen J. Blaivas, DO, Division of Pulmonary, Critical Care, and Sleep Medicine, VA New Jersey Health Care System, Clinical Assistant Professor, Rutgers New Jersey Medical School, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.