Pulmonary Hypertension

Table of Contents

Pulmonary hypertension (PH) is high blood pressure in the arteries leading to your lungs. PH is a serious medical condition. If you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow – which makes your heart work harder to pump blood. Over time, your heart weakens and cannot do its job and you may develop heart failure.

Symptoms of PH include:

  • Shortness of breath during routine activities, such as climbing stairs
  • Fatigue
  • Chest pain
  • Racing heartbeat
  • Pain in the upper right side of the stomach
  • Loss of appetite

As PH gets worse, you may find it difficult to engage in any physical activity.

There are two primary types of PH. One occurs in the family or appears for no known reason. The other type is linked to other conditions, usually heart or lung disease.

There is no cure for PH. Treatment can help control symptoms. Treatment includes treating heart or lung disease, medications, oxygen, and sometimes a lung transplant.

Overview

To understand PH, it is helpful to understand the way your heart and lungs work. Your heart has two sides which are separated by an inner wall called the septum. Each side of the heart has an upper and lower chamber. The lower right chamber of your heart, the right ventricle, pumps blood into your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen. The upper left chamber of your heart, the left atrium, receives oxygen-rich blood from your lungs. Blood is then pumped into the lower left chamber of your heart, the left ventricle. From the left ventricle, blood is pumped around the body through an artery called the aorta.

PH begins with inflammation and changes in the cells that line your pulmonary arteries. Other factors can also affect the pulmonary arteries and cause PH. For example, this condition can develop if:

  • The artery walls tighten.
  • The artery walls are stiff at birth or become stiff due to overgrowth of cells.
  • Blood clots form in the arteries.

These changes make it difficult for the heart to push blood through the pulmonary arteries and into the lungs. As a result, the pressure in the arteries increases. Since the heart is working harder than normal, the right ventricle becomes tense and weak. Your heart may become so weak that it cannot pump enough blood to your lungs – which could lead to heart failure. Heart failure is the most common cause of death in people with PH.

PH is divided into five groups based on the cause. In all groups, the mean pulmonary artery pressure is greater than 25 mmHg at rest or 30 mmHg during physical activity. The normal pulmonary artery pressure is 8-20 mmHg at rest. (MmHg is a millimeter of mercury – a unit used to measure blood pressure).

Other diseases or conditions, such as heart and lung disease or blood clots – are common causes of PH. Some people inherit the condition. In some cases, the exact cause is unknown.

PH has no cure. However, researchers are still conducting studies on new treatments. PH will be easier to manage if detected and treated early. Treatment includes medications, procedures, and therapies. These options of treatments can relieve PH symptoms and slow the disease’s progression. Lifestyle changes can also help control symptoms. 

Types of Pulmonary Hypertension

The World Health Organization divides pulmonary hypertension (PH) into five groups. These groups are classified based on the causes of the condition. In all groups, the mean pulmonary arteries pressure is greater than 25 mmHg at rest or 30 mmHg during physical activity. The normal pulmonary artery pressure is 8–20 mmHg at rest.

(Note that group 1 is called pulmonary arterial hypertension (PAH) and group 2 – 5 are called pulmonary hypertension (PH). However, all groups are called pulmonary hypertension.)

Group 1 Pulmonary Arterial Hypertension

Group 1 PAH includes:

  • PAH with unknown cause.
  • PAH is inherited (passed from parents to children through genes).
  • PAH due to drugs or toxins, such as street drugs and certain diet drugs.
  • PAH caused by certain conditions such as:
    • Connective tissue disorders. (Connective tissue helps support all your body parts, including your skin, eyes, and heart.)
    • HIV infection.
    • Liver disease.
    • Congenital heart disease. This is a heart disease that is present at birth.
    • Sickle cell disease.
    • Schistosomiasis. This is an infection caused by parasites. Schistosomiasis is one of the most common causes of PAH in many countries. 
  •  PAH due to conditions that affect the veins and small blood vessels in the lungs.

Group 2 Pulmonary Hypertension

Group 2 refers to PH with left heart disease. Conditions that affect the left side of the heart, such as mitral valve disease or long-term high blood pressure – can cause left heart disease and PH. Left heart disease is probably the most common cause of PH.

Group 3 Pulmonary Hypertension

Group 3 includes PH associated with lung diseases, such as COPD (chronic obstructive pulmonary disease) and interstitial lung disease. Interstitial lung disease causes scarring of the lungs.

Group 3 also includes PH associated with sleep-related respiratory disorders, such as sleep apnea.

Group 4 Pulmonary Hypertension

Group 4 refers to PH caused by blood clots in the lungs or blood clotting disorders.

Group 5 Pulmonary Hypertension

Group 5 refers to PH caused by various diseases or other conditions. Examples include:

  • Systemic disorders, such as sarcoidosis and vasculitis. Systemic disorders involve numerous internal organs 
  • Metabolic disorders, such as thyroid disease and glycogen storage disease. (In glycogen storage disease, the body’s cells do not use the form of glucose (sugar) properly)
  • Other conditions, such as tumors pressing on the pulmonary arteries and kidney disease.

Causes of Pulmonary Hypertension

Pulmonary hypertension (PH) starts with inflammation and cell changes that line your pulmonary arteries. Other factors can also affect the pulmonary arteries and cause PH. For example, this condition can develop if:

  • The artery walls tighten.
  • The artery walls are stiff at birth or become rigid due to overgrowth of cells.
  • Blood clots form in the arteries.

These changes make it difficult for your heart to push blood through your pulmonary arteries and into your lungs. Thus, the pressure in the arteries increases – leading to PH. Many factors can contribute to the processes that cause different types of PH.

Group 1 pulmonary arterial hypertension (PAH) may have unknown causes, or the condition may be inherited. Several diseases and conditions can also cause group 1 PAH. Examples include HIV infection, congenital heart disease, and sickle cell disease. Moreover, the use of street drugs (such as cocaine) and certain diet drugs can also cause PAH.

Many diseases and conditions can cause groups 2 to 5 PH (often called secondary PH), including:

  • Mitral valve disease
  • Lung diseases, such as COPD (chronic obstructive pulmonary disease)
  • Sleep apnea
  • Sarcoidosis

Risk Factors for Pulmonary Hypertension

Group 1 pulmonary artery hypertension (PAH) without a known cause is rare. Women are affected more often than men. People who have group 1 PAH tend to be overweight. PH that occurs with other diseases or conditions is relatively more common.

There is no cure for PH. However, treatment can help control symptoms. Treatment involves treating heart or lung disease, medications, oxygen, and sometimes a lung transplant. PH usually develops between the age of 20 and 60, but it can affect people of any age. People who are at high risk for PH include:

  • People with a family history of the condition.
  • People with certain diseases or conditions, such as heart and lung disease, liver disease, HIV infection, or blood clots in the pulmonary arteries.
  • People who use street drugs (such as cocaine) or certain diet drugs.
  • People who live in high altitudes

Signs and Symptoms of Pulmonary Hypertension

Signs and symptoms of pulmonary hypertension (PH) can include:

  • Shortness of breath during routine activities, such as climbing stairs
  • Fatigue
  • Chest pain
  • Racing heartbeat
  • Pain in the upper right side of the stomach
  • Loss of appetite

As PH worsens, you may find it difficult to engage in any physical activity. At this point, other signs and symptoms may include:

  • Feeling dizzy, especially when doing physical activities
  • Fainting
  • Swelling in your feet and ankles
  • Bluish lips and skin

Diagnosis of Pulmonary Hypertension

Your doctor will diagnose pulmonary hypertension (PH) based on your personal and your family’s medical history, a physical test, and the test results. PH can develop slowly. In fact, you may have had it for years and did not realize it. This is because the condition has no early signs or symptoms. When it does occur, symptoms are similar to other heart and lung conditions, such as asthma. This makes PH difficult to diagnose.

Personal and Family’s Medical History

Your doctor may ask about your signs and symptoms – how and when they started. He or she may also ask if you have any other medical conditions that may cause PH. Your doctor will want to know if anyone in your family has or has had PH. People who have a family history of PH are at a higher risk for this condition.

Physical Tests

In a physical test, your doctor will listen to your heart and lungs with a stethoscope. Your doctor will also check your ankles and feet for swelling and your lips and skin for a bluish tinge –  these are signs of PH.

Diagnostic Tests and Procedures

Your doctor may recommend tests and procedures to confirm a diagnosis of PH and look for the underlying cause. The doctor will also use the test results to determine the severity of your PH.

Tests and Procedures To Confirm the Diagnosis

Echocardiography

Echocardiography or echo, uses sound waves to create moving pictures of your heart. This test can estimate the pressure in your pulmonary arteries. The Echo can also show the size and thickness of your right ventricle and how well it is working.

Chest x-ray

A chest x-ray takes pictures of the structure in your chest – such as your heart, lungs, and blood vessels. This test can show if your pulmonary artery and right ventricle are enlarged. The pulmonary artery and right ventricle can become enlarged if the right ventricle has to work harder to pump blood through the pulmonary artery. Chest x-rays can also show signs of underlying lung disease that may cause or contribute to PH.

ECG (electrocardiogram)

An ECG is a simple, painless test that records the electrical activity of the heart. This test also shows whether you have a regular or irregular heart rhythm. An ECG can show if your right ventricle is enlarged or strained.

Right heart catheterization

This procedure measures the pulmonary arteries pressure and shows your heart’s pumping ability. Right heart catheterization can find a leak between the left and right sides of the heart. In this procedure, a thin, flexible tube called a catheter is inserted into a blood vessel in the groin (upper thigh) or neck. This tube is passed down the right side of your heart and into the pulmonary artery. Through the tube, your doctor can perform tests and treatment on your heart.

Tests To Identify The Main Cause Of Pulmonary Hypertension

Since PH can be due to numerous causes, many tests are required to perform to identify the main cause.

Chest CT scan

A chest tomography scan, or chest CT scan, produces pictures of the structures inside your chest – such as your heart, lungs, and blood vessels. These images can show signs of PH or conditions that can cause PH.

Chest MRI

Chest magnetic resonance imaging or chest MRI, shows how your right ventricle is working. This test also shows the blood flow in your lungs. A chest MRI can also help detect signs of PH or the underlying condition causing PH.

Pulmonary function tests

Pulmonary function tests involve measuring how much air you can inhale and exhale, how fast you can blow the air out, and how well your lungs deliver oxygen to your blood. This test can help detect lung diseases that may be causing PH.

Polysomnogram (PSG)

This test records brain activity, eye movements, heart rate, and blood pressure while you sleep. PSG also measures the level of oxygen in your blood. Low oxygen levels during sleep are common with PH, and can worsen the condition. PSG is usually done when you stay at a sleep center.

Pulmonary ventilation / perfusion (VQ) scan

A lung VQ scan measures the flow of air and blood in your lungs. This test can help detect blood clots in the veins of your lungs.

Blood tests

Blood tests are used to rule out other diseases, such as HIV, liver disease, and autoimmune diseases (such as rheumatoid arthritis).

Identifying the Severity of Pulmonary Hypertension

Physical exercise tests are used to determine the severity of PH. This test consists of a 6-minute walk test or a cardiopulmonary exercise test. The 6-minute walking test measures the distance you covered by walking briskly for 6 minutes. The cardiopulmonary exercise test measures how well your lungs and heart are working when you exercise on a treadmill or bicycle.

During the test, your doctor will assess your activity level. Your level is linked to the severity of your PH. The level system ranges from grade 1 to grade 4.

  • Class 1 without limitations. You can do regular physical activity, such as walking or climbing stairs. These activities do not cause PH symptoms such as fatigue, shortness of breath, or chest pain.
  • Class 2 with small limitations. You feel comfortable resting, but doing regular physical activity causes PH symptoms.
  • Class 3 with clear or obvious boundaries. You feel comfortable while resting. However, walking a block or two or climbing one-flight of stairs can cause PH symptoms.
  • Class 4 with strict restrictions. You cannot do any physical activity comfortably. You may also experience PH symptoms at rest.

Over time, you may require more exercise tests to find out how well your treatment is working. Each time the test is performed, the doctor will compare your activity level with the previous.

Treatment for Pulmonary Hypertension

Pulmonary hypertension (PH) cannot be completely cured. However, medications can help relieve symptoms and slow the disease’s progression. PH is treatable with medications, medical procedures, and other therapies. Treatment will depend on the type and the severity of PH. 

Group 1 Pulmonary Arterial Hypertension

Group 1 pulmonary arterial hypertension (PAH) includes inherited PH that has unknown cause, or caused by certain medications or conditions. Treatment for group 1 PAH includes medications and medical procedures.

Medications

Your doctor may prescribe medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. When the blood vessels relax, it helps more blood flow through them. The doctor may prescribe oral, inhaled, or injected medicines.

Examples of medications to treat group 1 PAH include:

  • Phosphodiesterase-5 inhibitors — such as sildenafil
  • Prostanoids — such as epoprostenol
  • Endothelin receptor antagonists — such as bosentan and ambrisentan
  • Calcium channel blockers — such as diltiazem

Your doctor may prescribe one or multiple of these medicines. To find out which medicine works best, you may have to undergo an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medications. This test is usually done during right heart catheterization.

Medical and Surgical Procedures

If you have group 1 PAH, your doctor may recommend one or more of the following procedures.

Atrial septostomy

In this procedure, a thin, flexible tube called a catheter is inserted into a vein in your leg and into your heart. The tube is then inserted through the wall that separates your right and left atria (the upper chambers of your heart). This wall is called the septum. A small balloon at the end of the tube is inflated. This creates an opening between the atria. This procedure reduces pressure on the right atrium and increases blood flow. 

Lung transplant

Lung transplantation is a surgical procedure to replace diseased lungs with healthy lungs from a recently deceased donor. This procedure can be used to treat severe lung disease that causes PAH.

Heart-lung transplant

A heart-lung transplant is a surgery in which the heart and lungs are replaced with healthy organs from a deceased donor.

Group 2 Pulmonary Hypertension

Conditions that affect the left side of the heart, such as mitral valve disease, can cause group 2 PH. Treating the underlying condition will help treat PH. Treatment may include lifestyle changes, medications, and surgery.

Group 3 Pulmonary Hypertension

Lung diseases, such as chronic obstructive pulmonary disease (COPD) and interstitial lung disease – can cause group 3 PH. Certain sleep disorders like sleep apnea can also cause group 3 PH. If you have this type of PH, you may require oxygen therapy. This treatment increases the oxygen level in your blood. You will receive oxygen through a soft plastic tip that gets into your nose. Oxygen therapy can be done at home or in a hospital. Your doctor may also recommend other treatments if you have underlying lung disease.

Group 4 Pulmonary Hypertension

Blood clots in the lungs or blood clotting disorders can cause group 4 PH. If you have this type of PH, your doctor may prescribe blood thinners. This medicine prevents the formation or enlargement of clots. In some cases, doctors perform surgery to remove scar tissue in the pulmonary arteries due to old blood clots.

Group 5 Pulmonary Hypertension

Various diseases and conditions, such as thyroid disease and sarcoidosis, can cause group 5 PH. Tumors pressing on the pulmonary arteries can also cause group 5 PH. To treat Group 5 PH, it is required to treat the cause.

All Types of Pulmonary Hypertension

Several treatment types can help treat all types of PH. These treatments include:

  • Diuretics, also called water pills. These medications help reduce fluid buildup in the body, including swelling in the ankles and feet.
  • Blood thinners. These medicines help prevent the formation or enlargement of blood clots.
  • Digoxin. This medicine helps the heart beat stronger and pump more blood. Digoxin is sometimes used to control heart rate if an abnormal heart rhythm, such as atrial fibrillation or atrial flutter, occurs.
  • Oxygen therapy. This treatment increases the oxygen level in your blood.
  • Physical activity. Regular activity can help improve your ability to be more active. Talk to your doctor about the right physical activity plan for you.

Researchers are still trying to find better PH treatments.

Home Care For Patients Diagnosed with Pulmonary Hypertension

Pulmonary hypertension (PH) cannot be cured. However, you can work closely with your doctor to manage symptoms and slow the disease’s progression.

Continuous Care

Adhere to your treatment plan as recommended by your doctor. Call your doctor if your PH symptoms get worse or change. It will be easier for your doctor to treat the symptoms early.  Some symptoms, such as chest pain, may require emergency care. Ask your doctor when to call or seek emergency care. You should also consult your doctor before taking any over-the-counter medicines. Some medications can make your PH worse or interfere with the PH medicines you take. Furthermore, ask your doctor if you should get the pneumonia vaccine and the annual flu shot.

You may deal with a complicated schedule for taking your medications. Contact your doctor or nurse if you have problems with the schedule. It will be helpful for you to learn the names of your medicines and their functionality. Create a list of your medications and do not stop or change the medicines unless you talk to your doctor first.

In addition, you should also keep track of your body weight. You may need to keep a diary of your weight. You must weigh yourself at the same time every day. If you notice rapid weight gain (2 pounds or more in 1 day / 5 pounds or more in 1 week) – call your doctor. This may be a sign that your PH is getting worse.

Pregnancy runs a high risk for women who have PH. Consider using birth control if you do not plan to get pregnant. Consult your doctor about which birth control is safe for you.

Lifestyle Changes

Changing your lifestyle can help you manage your symptoms. This change will depend on the type of PH you have. Talk to your doctor about lifestyle changes that suit your condition. 

Quit smoking

If you are a smoker, you should quit. Smoking makes PH symptoms worse. Ask your doctor about programs and products that can help you quit. You should also avoid exposure to secondhand smoke.

Implement a Healthy Diet

Starting a healthy diet and maintaining an ideal body weight are part of a healthy lifestyle. A healthy diet includes a variety of fruits, vegetables and whole grains. It also includes lean meat, poultry, fish, and fat-free or low-fat milk or dairy products. A healthy diet also means you should consume food sources that are low in saturated fat, trans fat, cholesterol, sodium (salt), and added sugar.

Ask your doctor whether you need to limit the amount of salt and fluids in your diet. You should also ask whether you need to consume foods that contain vitamin K. These foods can affect how well blood-thinning medicines work. Vitamin K is found in green leafy vegetables and some oils, such as canola and soybean oil.

Be Physically Active

Physical activity is an important part of a healthy lifestyle. Try to engage in physical activity, such as walking regularly. This will keep your muscles strong and help you stay active. Ask your doctor about the right physical activity for you. Your doctor may ask you to limit or avoid certain activities, such as:

  • Performing heavy-lifting activities.
  • Sitting in a hot tub or sauna or taking a shower for a long time. These activities can lower your blood pressure
  • Flying in an airplane or traveling to high altitude areas. Your doctor may ask you to use extra oxygen during the flight

Avoid activities that cause breathing problems, dizziness, or chest pain. If you experience any of these symptoms, seek immediate treatment.

Emotional Issues and Support

Living with a PH condition can cause fear, anxiety, depression, and stress. You may be worried about your medical condition, treatment, finances, and other concerns. Share your feelings with your healthcare provider. Talking to a professional counselor may also help. If you are very depressed, your doctor may recommend medications or other treatments that can improve your quality of life.

Participating in a support group with fellow patients can help you adjust to PH. You can see how other people who have had the same symptoms cope with it. Ask your doctor about local support groups or check with your local health centre.

Support from family and friends can also help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

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