Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension (PH) means that you have high blood pressure in your pulmonary arteries. The blood vessels carry oxygen-poor blood from your heart and send blood to your lungs. There can be multiple causes of pulmonary hypertension.

Pulmonary hypertension is dangerous, because it causes a disruption of blood flow through your heart and lungs. High blood pressure in the pulmonary arteries causes them to narrow. Your heart will need to work hard to deliver oxygen-poor blood to the lungs.

Manage pulmonary hypertension by seeking appropriate medical treatment in a timely manner. Your healthcare provider will determine which treatment option is best for you to reduce the risk of lasting heart damage or other problems throughout your body.

What are the symptoms of pulmonary hypertension?

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There are early and later symptoms that may indicate the presence of pulmonary hypertension. Early symptoms of the condition can include shortness of breath while:

  • Doing daily activities (climbing stairs, grocery shopping)
  • Exercising

It is also possible to exhibit no symptoms during the early stages of pulmonary hypertension. Symptoms may also be mild enough that they do not raise concerns until they interrupt your ability to do certain regular activities.

Later symptoms of pulmonary hypertension will include experiencing shortness of breath even when you are stationary. You may also notice:

  • Chest pain or pressure
  • Dizziness or fainting
  • Fatigue
  • Bluish or grey coloring on lips or skin
  • Loss of appetite
  • Racing heartbeat
  • Swelling (ankles, legs, stomach)
  • Pain in the upper ride side of your stomach

If you notice these or worsening symptoms, it is important to immediately contact a healthcare provider or seek emergency medical assistance.

How can pulmonary hypertension affect my body?

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Pulmonary hypertension is treatable and manageable, but it can lead to serious problems if you ignore the symptoms. Pulmonary hypertension can result in:

  • Anemia
  • Arrhythmias
  • Blood clots in the pulmonary arteries
  • Pericardial effusion (fluid buildup around the heart)

Those who are pregnant should monitor for any signs of pulmonary hypertension. PH can cause complications for the mother and the fetus. Undiagnosed and untreated pulmonary hypertension will overtax your heart and can prove fatal. An overworked right ventricle can lead to right-sided heart failure that can disrupt healthy organs and systems throughout your body.

Your healthcare provider can prescribe treatment for you that is based specifically on the cause of your pulmonary hypertension before it becomes potentially life-threatening.

What causes pulmonary hypertension?

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There are different causes of pulmonary hypertension based on the type of PH that a doctor might diagnose. Causes can range from diseases and underlying conditions to environmental exposure to drugs and toxins.

Pulmonary hypertension is sorted into five groups:

Group 1: Pulmonary Arterial Hypertension (PAH)

You may be affected by pulmonary arterial hypertension if you are living with:

  • Congenital heart disease
  • Genetic mutations
  • Glycogen storage diseases
  • HIV
  • Liver disease
  • Lupus
  • Portal hypertension
  • Schistosomiasis
  • Scleroderma
  • Pulmonary capillary hemangiomatosis
  • Pulmonary veno-occlusive disease

Taking recreational drugs like methamphetamine or years of taking diet pills like “fen-phen” may cause pulmonary arterial hypertension. Some individuals develop PAH without any clear cause.

 

Group 2: PH Due to Left-Sided Heart Disease

Heart problems may cause pulmonary hypertension. What affects the left side of the heart can lead to a ripple effect throughout the organ, as well as your pulmonary arteries and lungs. The common left-sided heart problems that can cause PH are:

  • Aortic valve disease: Your valve becomes leaky or narrow, which prevents it from sending oxygen-rich blood out to the rest of your body.
  • Left-sided heart failure: The heart’s left ventricle may be too weak or stiff to allow the heart to pump blood as it should.
  • Left ventricular hypertrophy: Long-term hypertension or arrhythmias result in a thickening of the muscular walls of the left ventricle.
  • Mitral valve disease: Blood is unable to flow from your left atrium to your left ventricle due to increased stretchiness (prolapse) or narrowing.

 

Group 3: PH Due to Lung Disease or Hypoxia

Lung issues that can result in pulmonary hypertension are:

  • Chronic Obstructive Pulmonary Disease (COPD): Breathing becomes more difficult due to a group of diseases, including chronic bronchitis and emphysema.
  • Obstructive Sleep Apnea: When your airway is partly or completely blocked repeatedly as you sleep, which prevents sufficient oxygen from reaching your organs.
  • Interstitial Lung Disease: Fibrosis, or scarring, is present in your lung tissue and causes breathing problems and coughing.

 

Group 4: PH Due to Blockages in the Lungs

Chronic thromboembolic pulmonary hypertension (CTEPH) can cause pulmonary hypertension due to blood clots and scarring developing in the arteries of your lungs. Blood clots that travel from another part of your body may leave behind scar tissue. The scars sometimes prevent or make it difficult for blood to flow through the pulmonary arteries.

 

Group 5: PH Due to Other Disorders

There are other conditions that you should speak to your healthcare provider about in relation to possible pulmonary hypertension causes. Those who are at higher risk of developing pulmonary hypertension include people living with:

  • Kidney disease
  • Thyroid disease
  • Cancerous or benign tumors near the lungs
  • Gaucher disease (a metabolic disorder affecting bones and organs)
  • Sarcoidosis (an inflammatory disease affecting the lungs and lymph glands)
  • Langerhans cell histiocytosis (a disorder resulting in lung scarring and cysts)

It is important to provide your healthcare provider with as much of your complete medical history as possible for the most effective treatment.

What are the pulmonary hypertension risk factors?

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Aging brings an increased risk of developing Group 1 pulmonary hypertension. Younger adults with pulmonary arterial hypertension often develop it from an unknown cause. PH is commonly seen in adults aged 30 to 60. Other risk factors to consider are:

  • Smoking
  • Being overweight
  • Family history
  • Asbestos exposure
  • Blood-clotting disorders
  • Congenital heart defects
  • Consistent high altitude exposure (living at 8,000 ft. or higher)
  • Weight loss medications
  • Illicit drugs (methamphetamine, cocaine)

How common is pulmonary hypertension?

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Pulmonary arterial hypertension and PH caused by blood clots are less common conditions. Other types of pulmonary hypertension are more common, such as pulmonary hypertension brought on by heart or lung problems. Some estimates show that PH affects 1 in 100 people. Around the world, roughly 1 in 10 adults who are over the age of 65 live with PH.

How is pulmonary hypertension diagnosed?

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Your physician will likely perform a physical exam and run some tests to determine your PH diagnosis. A physical exam will check for any signs of pulmonary hypertension or any other heart or lung concerns. Your provider will:

  • Ask about your health and medical history
  • Ask you to provide a list of any symptoms
  • Examine your neck veins (bulging may indicate right-sided heart failure)
  • Feel the upper right area of your stomach (checking the size of your liver)
  • Listen to your heart and lungs
  • Check for swelling in stomach, ankles, and legs
  • Use a pulse oximeter to measure your blood oxygen level
  • Measure your blood pressure

 

Your medical provider may run additional tests since pulmonary hypertension symptoms can be similar to those that present themselves for other conditions. You or your provider may also find that your best path forward is meeting with a pulmonologist or cardiologist.

What tests diagnose pulmonary hypertension?

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There are different pulmonary hypertension tests for different purposes. If your doctor wants to measure the blood pressure in your pulmonary arteries, they might suggest doing a:

  • Right heart catheterization
  • Doppler echocardiogram

Tests that are used to reveal underlying causes of pulmonary hypertension are:

  • Blood tests (for organ function, hormone levels, infections)
  • Chest CT scan (for blood clots, lung conditions)
  • Chest X-ray (to check on the size of the right ventricle or pulmonary arteries)
  • Polysomnogram (a sleep test to diagnose sleep apnea)
  • Pulmonary ventilation/perfusion scan (to check for blood clots in the lungs)

Your doctor may ask you to do a six-minute walk test. The test measures your endurance and shows how much oxygen circulates in your blood during the exercise. A provider can use the test to determine if your pulmonary hypertension is mild or severe.

What is the treatment for pulmonary hypertension?

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How a doctor treats you for pulmonary hypertension greatly varies based on your type of PH and any other existing medical conditions. There is direct treatment for only two types of PH: pulmonary artery hypertension and chronic thromboembolic pulmonary hypertension.

Treating pulmonary artery hypertension may include:

  • Calcium channel blockers to lower the blood pressure in your pulmonary arteries. The blockers also regulate your body’s blood pressure.
  • Diuretics to rid your body of excess fluid
  • Oxygen therapy
  • Pulmonary vasodilators to relax and open up your pulmonary arteries. The medications can improve blood flow and reduce any strain on your heart.

Treating chronic thromboembolic pulmonary hypertension may involve:

  • Anticoagulants to prevent blood clots
  • Balloon atrial septostomy, a procedure that helps stabilize you while you await a lung transplant
  • Balloon pulmonary angioplasty, a catheter-based procedure for patients who cannot undergo open surgery
  • Medication, like a soluble guanylate cyclase stimulator, may slow down the progression of CTEPH
  • Pulmonary endarterectomy, a surgical procedure to remove blood clots of the lung. It is presently the only cure for pulmonary hypertension and exclusively for CTEPH patients.

 

There are dietary changes and lifestyle adjustments that people with pulmonary hypertension can make. Speak to your healthcare provider about what is the best option for you.

 

How can I prevent pulmonary hypertension?

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It may not be possible to prevent pulmonary hypertension, but there are certain risk factors that are more within your control. Steps that you can take include:

  • Creating an exercise plan
  • Embracing a heart-healthy diet (less salt and saturated fat)
  • Quitting smoking, eliminating tobacco use
  • Taking blood pressure medication as prescribed

What is the outlook for pulmonary hypertension?

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Many factors can determine the outlook for someone who is diagnosed with pulmonary hypertension. They are:

  • The cause of the condition
  • Stage of diagnosis
  • Severity of symptoms
  • Other related medical conditions

Learning about your prognosis sooner can provide a much more positive outlook for living with and treating pulmonary hypertension. If you have questions or concerns, speaking with one or more providers is completely acceptable and encouraged.

What follow-up care is needed for pulmonary hypertension?

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Your provider will be able to tell you when you should schedule checkups and any tests. Other topics that you may wish to discuss with a provider are:

  • Pregnancy concerns and/or birth control options
  • Necessary items for creating an emergency kit
  • Which exercises are safe and which ones to avoid
  • Seasonal vaccine recommendations
  • Finding support groups or counseling

 

  • Navigating future travel plans

When should I seek medical assistance for pulmonary hypertension?

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There are specific circumstances when you should call your healthcare provider or seek urgent medical care. Call your provider if you experience:

  • A fast heart rate (120 beats per minute)
  • A respiratory infection or worsening cough
  • Consistent dizziness or lightheadedness
  • Chest pain
  • Extreme fatigue
  • Nausea or lack of appetite
  • Restlessness or confusion
  • Worsening shortness of breath
  • Increased frequency of swelling of the ankles, legs, or stomach
  • Sudden weight gain

Seek emergency medical attention if you experience:

  • A persisting heart rate of 120-150 beats per minute
  • Fainting spells where you lose consciousness
  • Shortness of breath even when you rest
  • Sudden and severe chest pain or headache
  • Hickman catheter complications with intravenous prostacyclins (catheter displacement, infection, solution leak, bleeding and IV pump malfunction)
  • Sudden weakness/paralysis in your arms or legs

Processing a pulmonary hypertension diagnosis can be challenging. There are resources that are available to help you navigate the process. Speak with your provider to better understand the best path forward for you.

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