What Is Pulmonary Hypertension?
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Medically reviewed by Jenny Sweigard, MD
Pulmonary hypertension (PH) is a condition that occurs when your blood pressure in the blood vessels between the heart and lungs is abnormally high. Blood pressure measures the amount of force it takes to push your blood through your arteries, which routes blood to the rest of your body. With pulmonary hypertension, the heart has to work harder to carry blood from the heart to the lungs, and symptoms such as shortness of breath, fatigue, chest pain, and lightheadedness can develop.
Pulmonary hypertension affects about 1% of the global population. Some cases of pulmonary hypertension are hereditary, and others develop due to underlying medical conditions. Pulmonary hypertension is a serious, progressive condition that requires treatment, such as medication, lifestyle modifications, oxygen therapy, or surgery.
Types
The five types of pulmonary hypertension are classified based on the underlying cause. Treatment options can vary depending on the type of pulmonary hypertension you have.
Group 1: Pulmonary Arterial Hypertension (PAH)
Pulmonary arterial hypertension causes the small lung arteries to become narrow, stiff, or thickened. PAH is often idiopathic, meaning there is no identified cause. Drug use, congenital heart disease, HIV, connective tissue disorders, and autoimmune diseases can also cause this type.
Group 2: Pulmonary Hypertension Due to Left Heart Disease
The most common form of PH, this type is a complication of heart conditions that affect the left side of the heart or heart valves, causing your heart to struggle with pumping blood returning from the lungs.
Group 3: Pulmonary Hypertension Due to Lung Disease
This type causes the lung arteries to constrict (tighten) so that blood only reaches areas of the lungs that receive the most oxygen, leading to increased blood pressure in the lungs. This can occur due to a variety of factors, including living in high altitudes, having sleep apnea, or having a lung disorder like chronic obstructive pulmonary disease (COPD) or interstitial lung disease.
Group 4: Pulmonary Hypertension Due To Chronic Blood Clots in the Lungs
Also known as chronic thromboembolic pulmonary hypertension (CTEPH), this type is a rare, progressive form of pulmonary hypertension that develops when blood clots frequently form in the pulmonary arteries, causing narrowed or clogged arteries.
Group 5: Pulmonary Hypertension With Unknown Causes
Some cases of pulmonary hypertension don't fit neatly into the first four categories. Certain conditions, such as sickle cell anemia, certain metabolic disorders, and sarcoidosis, can cause this fifth type.
Pulmonary Hypertension Symptoms
In the initial stages, symptoms of pulmonary hypertension are often mild or similar to symptoms of other conditions. As the disease progresses, symptoms may worsen and include:
Shortness of breath, especially during physical activity
Weakness
Dry cough (sometimes with blood)
Dizziness or fainting
Vomiting
Hoarse voice
Swelling in the abdomen, ankles, legs, or feet
Chest pain or discomfort, especially during physical exertion
Wheezing (whistling sound when breathing)
Rapid heartbeat
Decreased appetite
Upper right abdominal pain
Stages
The World Health Organization classifies the four stages of pulmonary hypertension based on the severity of symptoms. They include:
Class I: You have no limitations in physical activity; ordinary activities do not cause symptoms.
Class 2: You have slight limitations in physical activity and are comfortable at rest, but ordinary physical activity causes symptoms.
Class 3: You have significant limitations in physical activity and may experience symptoms during ordinary physical activity but not at rest.
Class 4: You cannot perform any physical activity without symptoms and may also have symptoms when resting.
What Causes Pulmonary Hypertension?
Pulmonary hypertension develops when the arteries in your lungs narrow, stiffen, or become damaged, making your heart work harder to pump blood through your lungs, where it picks up oxygen. At rest, normal pulmonary blood pressure is between 11-20 mm Hg (millimeters of mercury/pressure measurement). If your pulmonary blood pressure is 25 mm Hg or higher, you have pulmonary hypertension.
Sometimes, there is no identifiable cause of pulmonary hypertension; other times, it may develop due to an inherited (genetic) condition or underlying heart condition.
Underlying health conditions that can affect or damage the blood vessels can cause pulmonary hypertension. These include:
Respiratory diseases: Conditions that cause breathing problems, including obstructive sleep apnea, COPD (e.g., emphysema, chronic bronchitis), and pulmonary fibrosis, can affect the heart and blood vessels.
Connective tissue diseases: Scleroderma, lupus, and mixed connective tissue disease can inflame and damage blood vessels, leading to pulmonary hypertension.
Heart disease: Heart problems that affect the left side of your heart, such as coronary artery disease, valvular heart disease, and congenital heart defects, can restrict blood flow to the lungs.
Blood clots: Pulmonary embolism, or a blood clot in the lungs, can block one of the pulmonary arteries and lead to pulmonary hypertension.
Other conditions: HIV, liver disease, infections, and certain drugs (e.g., methamphetamine and cocaine) can damage blood vessels and cause pulmonary hypertension.
Risk Factors
Factors that may increase your risk of pulmonary hypertension include:
Age: The risk of pulmonary hypertension increases with age. Most people receive a diagnosis between the ages of 30 to 60.
Sex: Pulmonary hypertension is more common in people assigned female at birth than people assigned male at birth.
Family history: A family history of blood clots or pulmonary hypertension increases your risk of developing the condition.
Medications: Some prescription medications, such as appetite suppressants, opioids, selective serotonin reuptake inhibitors (SSRIs), and some drugs used to treat cancer are associated with an increased risk of pulmonary hypertension.
Lifestyle habits: Certain lifestyle habits, such as illicit drug use or smoking, raise the risk of pulmonary hypertension.
Environment: Living at a high altitude for an extended period, parasitic infections, or exposure to asbestos all increase your risk.
Diagnosis
Diagnosis of pulmonary hypertension involves a medical history review, physical examination, and diagnostic testing. To diagnose pulmonary hypertension, your healthcare provider will ask about your symptoms, current medications, and your personal and family medical history. During the physical examination, they will listen to your heart and lungs, measure your blood pressure, and check your legs, abdomen, and feet for signs of fluid buildup (edema).
Depending on your symptoms and risk factors, your healthcare provider may refer you to a cardiologist (a doctor specializing in heart and blood vessel diseases) or pulmonologist (a doctor specializing in lung diseases) who will order diagnostic tests to determine if you have pulmonary hypertension.
These tests may include:
Blood tests: These can be used to check for anemia, blood clots, or cardiac stress.
Cardiac imaging scans: Cardiac magnetic resonance imaging (MRI) provides detailed images of the heart and surrounding blood vessels so your healthcare provider can view their structure and assess their level of function.
Lung imaging scans: Imaging tests, such as a chest X-ray, take detailed pictures of the lungs and surrounding structures to help visualize the shape of the heart and pulmonary arteries.
Electrocardiogram (ECG/EKG): This measures the electrical activity of your heart to detect damage to your heart or surrounding blood vessels. It can determine if your heart is working too hard to pump blood to the lungs.
Treatments for Pulmonary Hypertension
There is no cure for pulmonary hypertension, but treatments can help manage symptoms and slow disease progression. Treatment options include medications, oxygen therapy, various procedures, and lifestyle modifications.
Medications
Prescription drugs can help lower your blood pressure in the pulmonary arteries, improve blood flow to the lungs, and reduce inflammation. Oral medications (taken by mouth) commonly prescribed for pulmonary hypertension include:
Vasodilators: These medications help relax your blood vessels to reduce pressure in your pulmonary arteries. Examples include calcium channel blockers, such as Adizem (diltiazem) and Adalat (nifedipine).
Endothelin receptor antagonists: These block the effects of endothelin, a protein that constricts and narrows blood vessels. Examples include Letairis (ambrisentan), Tracleer (bosentan), and Opsumit (macitentan).
Phosphodiesterase 5 inhibitors: These help relax blood vessels to improve blood flow and reduce blood pressure. Examples include Revatio (sildenafil) and Adcirca (tadalafil).
Pulmonary hypertension medications are sometimes delivered subcutaneously (with a small needle under the skin), intravenously (IV), or breathed in through an inhaler. These medicines help open the blood vessels to manage symptoms such as shortness of breath and chest pain. These include:
Remodulin, Tyvaso (treprostinil)
Flolan, Veletri (epoprostenol)
Ventavis (iloprost)
Oxygen Therapy
Supplemental oxygen may be necessary to improve your oxygen levels and reduce symptoms of pulmonary hypertension, such as shortness of breath. Oxygen therapy is helpful for more advanced cases of PH where shortness of breath occurs during physical activity or at rest.
Specialist Procedures
In some cases, surgery or other procedures may be necessary to treat pulmonary hypertension. These include:
Balloon atrial septostomy: This procedures involves widening the hole (foramen ovale) between the left and right atriums of your heart to improve blood flow and increase blood oxygen levels.
Balloon pulmonary angioplasty: This procedure opens blood vessels in your lungs that are narrow or blocked. This reduces blood pressure in the blood vessels of your lungs and improves your heart function.
Pulmonary endarterectomy: This surgery removes old blood clots and scarring inside pulmonary blood vessels. It may cure some cases of group 4/pulmonary hypertension due to chronic blood clots.
Lifestyle Modifications
Along with medical treatments, healthy lifestyle can help manage symptoms of pulmonary hypertension. Heart-healthy lifestyle habits include:
Exercise: Regular physical activity, such as walking, swimming, or cycling, benefits cardiovascular health. Avoid heavy lifting or strenuous exercise that causes chest pain or worsens your symptoms.
Healthy diet: A nutritious, balanced diet with plenty of fruits, vegetables, whole grains, lean proteins, and low-fat dairy products may help slow disease progression. Limit your intake of fat, cholesterol, sodium (salt), and processed sugars.
Avoid smoking: If you smoke, talk to your healthcare provider about smoking cessation programs to help you quit.
Stress management: Living with pulmonary hypertension can take a toll on your emotional and psychological well-being. Finding healthy ways to manage stress can help you cope with the stressors of PH.
Prevention
It is not always possible to prevent pulmonary hypertension, but there are things you can do to reduce your risk. This includes seeing your healthcare provider regularly for checkups so they can help detect symptoms of PH early. This is especially true if you have a medical condition associated with an increased risk of PH, such as COPD or sleep apnea. Following your treatment plan for any chronic conditions you have can help prevent PH,
Regular physical activity, consuming a nutritious diet, and maintaining a healthy weight are important for your overall health, and especially your heart. If you smoke, quitting is one of the best ways to protect your heart, blood vessels, and lungs.
Related Conditions
Pulmonary hypertension is a progressive disease that can worsen over time. People with PH are at an increased risk of developing other conditions, including:
Anemia: Develops when the body does not have enough healthy red blood cells. PH can cause anemia if the bone marrow, where red blood cells are produced, does not get sufficient oxygen. PH can also increase the breakdown of red blood cells and lead to anemia.
Arrhythmia: High blood pressure in the pulmonary blood vessels can lead to heartbeat irregularities.
Blood clots: Narrowed blood vessels can slow blood flow, causing it to clump together and form clots within the blood vessels.
Heart failure: Right-sided heart failure occurs when the heart cannot pump enough blood to meet your body's needs.
Pericardial effusion: Increased blood pressure in the pulmonary vessels can cause fluid buildup around your heart.
Living With Pulmonary Hypertension
Pulmonary hypertension is a chronic condition that can impact every part of your life. With proper management, most people with PH can control their symptoms and live well.
Along with working closely with your healthcare team, being proactive about your health can help slow disease progression. This includes following your treatment plan (including taking medications as prescribed) and having regular check-ups with your healthcare provider.
It is safest to avoid activities that may worsen your symptoms, including intense workouts, lifting heavy objects, soaking in a hot tub, and spending prolonged periods at high altitudes.
Frequently Asked Questions
What is the life expectancy of pulmonary hypertension?
Your life expectancy with pulmonary hypertension depends on several factors, including the severity and type of PH you have. Research advancements have led to many effective treatments for PH, which help slow disease progression and control symptoms.
What foods should you avoid if you have pulmonary hypertension?
While there are no specific foods to avoid with pulmonary hypertension, you should generally aim for a heart-healthy diet that is low in sodium to help manage the condition. Reducing salt intake is important because excessive sodium can lead to fluid retention and worsen symptoms. A diet rich in fruits, vegetables, whole grains, lean proteins, and limited processed foods can support cardiovascular health and complement your treatments.
Is drinking water good for pulmonary hypertension?
While it is always important to stay hydrated, drinking too much water or other fluids (especially at one time) can make it difficult for your heart to move the fluid around your body. This can worsen your symptoms of pulmonary hypertension, such as fluid retention and bloating.
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