Pulmonary Hypertension: Symptoms, Causes, Diagnose, Risk factors, Treatment & Prevention

 

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

One type of pulmonary hypertension is pulmonary arterial hypertension (PAH), in which the blood arteries in the lungs become narrowed, blocked, or destroyed. 

 The injury makes it difficult for blood to get through the lungs. Blood pressure in the lung arteries rises. The heart has to work harder to push blood through the lungs. The increased effort eventually leads the heart muscle to weakening and failing.

In some cases, pulmonary hypertension gradually worsens. It has the potential to cause life- threatening conditions. There is no cure for pulmonary hypertension. However, there are treatments available that can help you feel better, live longer, and have a higher quality of life.

Pulmonary hypertension is defined as high blood pressure in the pulmonary arteries, which carry oxygen-poor blood from the heart to the lungs. The first symptom is shortness of breath during your normal activities. The most common causes are cardiovascular disease, pulmonary disease, and hypoxia. Early diagnosis and treatment can improve your quality of life.

Also Read: Chronic Kidney Disease: Symptoms, Causes, Treatment & Prevention

What is pulmonary hypertension?

Pulmonary hypertension (PH) is a broad term for having excessive blood pressure in pulmonary arteries. These are the blood arteries that transport oxygen-poor blood from the heart to the lungs.

Pulmonary hypertension has different causes. It is typically a complication of heart or lung disease. However, many other diseases and environmental factors can increase your risk of PH.

Pulmonary hypertension is dangerous because it affects blood flow through the heart and lungs. High blood pressure causes the pulmonary arteries to narrow. As a result, your heart has to work harder to deliver oxygen-poor blood to your lungs.

Over time, PH can harm your heart and trigger problems throughout your body. It could be fatal if left untreated.

What are the different types of pulmonary hypertension?

The World Health Organization (WHO) classifies pulmonary hypertension into five categories based on the cause.

• Group 1 PH occurs as a result of pulmonary arterial hypertension. PAH can be caused by a variety of factors, including underlying diseases and drugs. PAH causes your pulmonary arteries to constrict, thicken, or stiffen. Less blood can flow through, increasing the pressure in your pulmonary arteries.

• Group 2 PH results from left-sided heart disease. The left side of your heart pumps blood throughout your body. If there is a problem on this side of your heart, it will impact the right side of your heart as well as the complete pulmonary circuit. Blood builds up in your heart, increasing the pressure in your pulmonary arteries.

• Group 3 PH is the result of lung disease or hypoxia. Certain lung problems cause the arteries in your lungs to constrict. Less blood can travel through your lungs, raising the pressure in your pulmonary arteries.

• Group 4 PH occurs as a result of pulmonary obstructions. Blood clots or their scars restrict regular blood flow through your lungs. This increases stress on the right side of your heart and elevates pulmonary blood pressure.

• Group 5 PH is caused by various diseases. PH is associated with conditions such as blood abnormalities and metabolic disorders. The particular mechanisms by which the disease triggers PH are not always clear.

What are the first symptoms of pulmonary hypertension?

The first symptom of pulmonary hypertension is shortness of breath during your daily activities. These could involve climbing stairs or going food shopping. You may also have shortness of breath when exercising.

At the beginning of pulmonary hypertension, you may not experience any symptoms. When you first detect symptoms, they may be mild. However, PH symptoms worsen over time, making it difficult for you to carry out your normal activities.

What are the later symptoms of pulmonary hypertension?

As PH progresses, you will have shortness of breath even when you are not moving. Additional symptoms include:

• Bluish color on your skin or lips

• Pain in the chest or pressure

• Feeling dizzy or faint

• Fatigue

• I feel less hungry than usual

• Pain in the upper right side of your stomach

• A racing heartbeat

• Swelling (edema) in the ankles, legs, or tummy

PH symptoms make it difficult to exercise or go about your typical daily activities.

What are the different stages of pulmonary hypertension?

There are four major stages of pulmonary hypertension. The World Health Organization (WHO) refers to these as "functional classes." They are based on the symptoms you experience and refer to your ability to carry out daily activities. As PH worsens, the symptoms become more visible and disruptive to your daily life.

• Class 1: You have no symptoms.

• Class 2: You do not experience symptoms when you are resting. However, you experience some discomfort or shortness of breath during everyday tasks. These include completing domestic duties and ascending stairs.

• Class 3: You may still feel fine while resting. However, doing typical duties has become much more difficult due to fatigue or shortness of breath.

• Class 4: You experience symptoms even when you are resting. When you try to complete a typical task, the symptoms worsen.

What causes pulmonary hypertension?

The causes of pulmonary hypertension differ greatly depending on the type of PH you have. They encompass a variety of disorders and underlying conditions, as well as environmental exposures (toxins and medications).


Group 1 PH owing to pulmonary arterial hypertension (PAH).

There are numerous causes of pulmonary arterial hypertension. They include:

• Congenital heart disease

• Diet medicines such as "fen-phen" can develop PAH years after use

• Genetic mutations

• Glycogen storage disorders

• HIV

• Liver disease

• Lupus

• Portal hypertension

• Pulmonary capillary hemangiomatosis

• Pulmonary venous occlusive disease

• Schistosomiasis

• Scleroderma

• Recreational drugs such as methamphetamine

Some people get PAH without a recognized etiology. These situations are referred to as "idiopathic."

Group 2 PH resulting from left-sided heart disease.

Heart issues are a common cause of pulmonary hypertension. Because your heart's left and right halves work together, a problem with one affects the other. The right side of your heart pumps blood into your pulmonary arteries. So, left-sided heart problems set off a chain reaction that affects the remainder of your heart, pulmonary arteries, and lungs.

Pulmonary hypertension can be caused by left-sided heart problems such as:

Aortic valve disease. The aortic valve connects your heart's left ventricle (the primary pumping chamber) to the aorta. Your aorta is your body's main blood vessel, responsible for transporting oxygen-rich blood throughout the body. Aortic valve disease can cause your valve to be leaky (regurgitation) or narrowed (stenosis). Other issues, such as congenital heart disease and endocarditis, can impact your aortic valve.

Left-sided heart failure.  This signifies that the left side of your heart cannot pump blood properly.  Your left ventricle may be too weak or overly stiff.  In either case, your body's organs and tissues will receive insufficient oxygen-rich blood flow.  Over time, left-sided heart failure can progress to right-sided heart failure and associated problems.

 Left ventricular hypertrophy.  This is a thickening of the left ventricle's muscular walls that reduces the heart's efficiency in pumping blood.  It is typically caused by long-term hypertension or other heart conditions such as arrhythmias.

Mitral valve disease. The mitral valve allows blood to pass from the left atrium to the left ventricle. It may become overly stretched (prolapse), resulting in regurgitation. It may also get overly thin. Mitral valve dysfunction causes stress and damage to your heart over time.

Group 3 PH resulting from lung disease or hypoxia.

Lung problems are another common source of pulmonary hypertension. Some persons with left-sided cardiac disease suffer from lung disease or a shortage of oxygen (hypoxia).

The following lung problems can lead to pulmonary hypertension:

COPD (Chronic Obstructive Pulmonary Disease). This is a group of diseases that encompasses chronic bronchitis and emphysema. COPD makes it tougher to breathe.

Interstitial lung disease. This indicates that you have fibrosis, or scarring, in your lung tissue. The scarring causes breathing issues and coughing.

Obstructive Sleep Apnea. This disorder causes your airway to become partially or totally closed repeatedly while sleeping. As a result, your organs receive less oxygen.

Group 4 PH due to lung blockages

This form of PH is typically characterized by chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a disorder characterized by blood clots and scarring in the arteries of the lungs.

A pulmonary embolism is a blood clot that travels from elsewhere in your body and becomes lodged in a blood vessel in your lungs. These blood clots are typically curable, but they may leave scar tissue.

These scars can restrict blood flow through your pulmonary arteries. As a result, the blood pressure in your pulmonary arteries goes up.

Group 5 PH due to other disorders.

Many other diseases induce pulmonary hypertension in ways that scientists do not fully understand. Scientists do not understand the exact mechanisms that cause these circumstances to produce PH. They do know that there is an association between these disorders and pH. People with these conditions may be more likely to develop PH. These conditions include:

• Gaucher disease is a metabolic disorder that affects the organs and bones.

• Kidney disease refers to problems with the kidneys' function.

• Langerhans cell histiocytosis is a condition in adults that produces scarring and cysts in the lungs.

• Sarcoidosis is an inflammatory disease that typically affects the lungs and lymph nodes.

• Thyroid disease refers to disorders that impact the thyroid's hormone production.

• Tumors: Cancerous or benign tumors can impose pressure on blood vessels in the lungs.

How is pulmonary hypertension diagnosed?

To diagnose pulmonary hypertension, your provider will perform a physical examination and administer tests.

First, you'll undergo a physical exam to look for signs of pulmonary hypertension and other heart or lung problems. During the examination, your physician will:

• Ask you about your health and medical history.

• Ask about your symptoms.

• Check the size of your neck's veins. Bulging neck veins (jugular venous distention) may be indicative of right-sided heart failure.

• Feel the upper right part of your abdomen to determine the size of your liver.

• Listen to your heart and lungs with a stethoscope.

• Check your abdomen, ankles, and legs for edema.

• Check your blood pressure.

Pulmonary hypertension is difficult to diagnose because many of its symptoms are similar to those of other illnesses. So, after your physical exam, your provider may administer several tests to get further information.

Your physician may also refer you to a pulmonologist or cardiologist.

What tests diagnose pulmonary hypertension?

Your physician may administer multiple tests for various causes.

These examinations determine the blood pressure in your pulmonary arteries.

Right heart catheterization: This test is also known as pulmonary artery catheterization. It monitors the pressure inside your pulmonary arteries and determines how much blood your heart can pump in a minute.

A Doppler echocardiography employs sound waves to illustrate how your right ventricle functions. It also measures blood flow through your heart's valves. It enables your provider to calculate your systolic pulmonary artery pressure.

These tests seek to identify the underlying cause of pulmonary hypertension.

• Blood tests: Look for abnormalities with organ function, hormone levels, and infections. A full metabolic panel and a complete blood count are two specific blood tests.

• Chest CT scan: Looks for blood clots and other lung diseases that could be causing or exacerbating your pulmonary hypertension.

• Chest X-ray: Determines whether your right ventricle or pulmonary arteries are larger than they should be.

• Polysomnography (PSG): This overnight sleep test determines whether you have sleep apnea.

• Pulmonary ventilation/perfusion (VQ) scan: Checks for blood clots in the lungs.

• Your provider may also perform a six-minute walk test. This test shows how much exercise you can handle and how much oxygen circulates in your blood when you exercise. The results determine if your pulmonary hypertension is mild or severe.

Risk Factors:

Pulmonary hypertension is typically prevalent in adults aged 30 to 60. Growing older increases the likelihood of having Group 1 pulmonary hypertension, often known as pulmonary arterial hypertension (PAH). PAH due to an unknown origin is more common in young adults.

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

• A family history of the condition

• Being overweight

• Smoking

• Blood clotting disorders or a family history of lung clots

• A history of being around asbestos

• A congenital heart defect is a heart abnormality that exists from birth

• Living at altitudes of 8,000 feet (2,438 meters) or higher

• Use of some medications, especially those for weight loss

• Illicit drugs include cocaine and methamphetamine

Complications:

Potential complications of pulmonary hypertension include:

• Right-sided heart enlargement and failure. This disorder, also known as cor pulmonale, enlarges the right lower chamber of the heart. The chamber must pump harder than usual to transport blood through restricted or obstructed lung arteries.

• As a result, the heart walls get thicker. The right lower heart chamber extends to increase the amount of blood it can accommodate. These alterations place additional strain on the heart. Eventually, the right lower heart chamber fails.

• Blood clots. Pulmonary hypertension increases the risk of blood clots in the small arteries of the lungs.

• Irregular heartbeats, often known as arrhythmias. Pulmonary hypertension can alter the heartbeat, which can be life-threatening.

What are the treatments for pulmonary hypertension?

The type of pulmonary hypertension you have, as well as your other medical conditions, determines how you treat it. Your healthcare team will tailor treatment to your specific needs.

Currently, only two forms of pH can be handled directly:

• Pulmonary artery hypertension (PAH).

• Chronic thromboembolic pulmonary hypertension, or CTEPH.

• Other types of PH are treated by addressing underlying medical conditions.

Treatment for pulmonary arterial hypertension (PAH) involves:

•  Calcium channel blockers.  These medications can help reduce blood pressure in the pulmonary arteries and throughout the body.

•  Diuretics.  These "water pills" assist your body eliminating excess fluid.

•  Oxygen therapy.  If your blood is depleted of oxygen, you may require this treatment.

•  Pulmonary vasodilators.  These medicines help your pulmonary arteries relax and open up more.  This increases blood flow and lowers the strain on the heart.

Treatment for CTEPH involves:

• Anticoagulants. These medications help prevent blood clots.

• Balloon atrial septostomy (BAS). The procedure is frequently performed on babies with significant cardiac abnormalities. However, it is also used to treat individuals with pulmonary hypertension. It's a bridge that keeps you stable while you wait for a lung transplant.

• Balloon pulmonary angioplasty (BPA). This catheter-based surgery employs a balloon to expand your pulmonary artery. It is typically used when open surgery is not an option.

• Medication. A soluble guanylate cyclase stimulator (SGCS) may help slow the disease's progression.

Pulmonary endarterectomy (PEA). This type of surgery removes blood clots from the lungs. It is now the only viable cure for pulmonary hypertension, but only for those with CTEPH.
The treatment for PH caused by heart or lung disorders focuses on addressing the underlying issues. Because PH is caused by a wide range of heart and lung problems, treatment approaches vary greatly from person to person. Talk to your provider about what is best for you. Generally, your provider may recommend:

• Dietary changes.

• Lifestyles change.

• Medication to treat conditions such as hypertension and heart failure.

• Oxygen therapy.

• Surgery, such as heart valve repair.

Treatments for PH induced by other medical problems (WHO Group 5) are constantly changing. Your provider will collaborate with you to develop the optimum care plan.

A lung transplant is a last resort treatment for some patients with severe pulmonary hypertension.

Which medications can be used to treat pulmonary hypertension?

 Pulmonary vasodilators are medications used to treat PAH and CTEPH.  They cannot be used to treat other kinds of PH, such as those caused by underlying heart or lung conditions.

 Pulmonary vasodilators help your pulmonary arteries to relax.  This decreases blood pressure and reduces the strain on the right side of the heart.

 People with PH may take a range of additional medications depending on their underlying conditions.

How should I manage my pulmonary hypertension medication?

You may be taking multiple drugs to treat PH and other medical issues. There might be a lot to keep track of. Here is some guidance.

Know the names of all your medications and how they operate. Have a list with you at all times.
Take the medication at the same time every day. If you miss a dose, do not take two doses to make up for it. Call your provider and ask what to do.
Do not take any over-the-counter medications until you consult your doctor first. Some cold and flu medications and pain relievers can be harmful to people with heart failure.

Avoid over-the-counter medications with cautions for patients with high blood pressure.
Do not stop taking your medication or make any changes to your medications without first consulting your provider.
Avoid herbal supplements, especially herbal tea. Herbal products may interfere with medications.

Prevention:

What increases a person's risk of acquiring pulmonary hypertension?
The risk factors for developing pulmonary hypertension are:

• Asbestos exposure

• A family history of blood clots

• A family history of pulmonary hypertension

• Living at a high altitude

• Smoking and using tobacco products

• Use of diet pills, such as "fen-phen" (dexfenfluramine and phentermine)

• Use of certain prescription medications for cancer and depression

• Use of recreational drugs

Certain medical conditions raise your risk. This includes:

• Blood clots in the pulmonary arteries

• Connective tissue Disease

• Down Syndrome

• Gaucher Disease

• Heart disease

• HIV

• Liver disease

• Lung disease

Talk to your provider about your risk factors and what you can do to reduce them.

How can I prevent pulmonary hypertension?

It is not always possible to avoid pulmonary hypertension. Some risk factors are beyond your control. If you have risk factors, your doctor may suggest regular testing to monitor your heart and lung function.

Taking steps to avoid or treat other medical issues can help reduce your chance of pulmonary hypertension. You can take the following steps:

• Create an exercise plan. Ask your healthcare professional about which exercises are safe for you.

• Maintain a heart-healthy diet. Avoid processed foods, fast food, and other foods high in sodium and saturated fat.

• Quit smoking and stop using tobacco. Smoking and tobacco use are major risk factors for heart and lung disease. Quitting is difficult, particularly if you've been smoking or using tobacco for a long period. However, your provider may be able to help with resources. Support groups may also be helpful.

• Take your blood pressure and other medications exactly as prescribed.

References:

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• Asthma and Lung UK. Treatment for Pulmonary Hypertension (https://www.blf.org.uk/support-for-you/pulmonary-hypertension/treatment). Accessed 6/2/2022.

• American Academy of Family Physicians. Pulmonary Hypertension (https://familydoctor.org/condition/pulmonary-hypertension/?adfree=true). Accessed 6/2/2022.

• Bhogal S, Mukherjee D, Banerjee S, Islam AM, Daggubati R, Paul TK. Current trends and future perspectives in the treatment of pulmonary hypertension: WHO Group II-V (https://pubmed.ncbi.nlm.nih.gov/29153390/). Curr Probl Cardiol. 2018 May;43(5):217-231. Accessed 6/2/2022.

• Khan MS, Memon MM, Amin E, et al. Use of balloon atrial septostomy in patients with advanced pulmonary arterial hypertension: A systematic review and meta-analysis (https://pubmed.ncbi.nlm.nih.gov/30910639/). Chest. 2019 Jul;156(1):53-63. Accessed 6/2/2022.

• Pulmonary Hypertension Association. Multiple pages (https://phassociation.org/types-pulmonary-hypertension-groups/). Accessed 6/2/2022.

• European Pulmonary Hypertension Association. Classification and WHO Functional Class (https://www.phaeurope.org/about-ph/classification-and-who-functional-class/). Accessed 6/2/2022.

• Rosenkranz S, Howard LS, Gomberg-Maitland M, Hoeper MM. Systemic consequences of pulmonary hypertension and right-sided heart failure (https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.116.022362). Circulation. 2020 Feb;141:678-693. Accessed 6/2/2022.

• United Kingdom, National Health Service. Pulmonary Hypertension (https://www.nhs.uk/conditions/pulmonary-hypertension/). Accessed 6/2/2022.

• Asthma and Lung UK. Treatment for Pulmonary Hypertension (https://www.blf.org.uk/support-for-you/pulmonary-hypertension/treatment). Accessed 6/2/2022.

• Corris PA, Seeger W. Call it by the correct name – pulmonary hypertension not pulmonary arterial hypertension: Growing recognition of the global health impact for a well-recognized condition and the role of the Pulmonary Vascular Research Institute (https://pubmed.ncbi.nlm.nih.gov/32233785/). Am J Physiol Lung Cell Mol Physiol. 2020 May;318(5):L992-L994. Accessed 6/2/2022.