Pulmonary Hypertension (PH): Types, Causes, Symptoms, Diagnosis, and Treatment

Michael Rotman, MD, FRCPC, PhD | Medically reviewed by Robin Backlund, BHSc
Last update: October 7, 2024

According to the National Heart, Lung, and Blood Institute (NHLBI), pulmonary hypertension (PH) is a complex and often misunderstood condition that affects the arteries in the lungs and the right side of the heart.

Technically, it is characterized by elevated blood pressure in the pulmonary arteries, which drive blood supply from your heart to your lungs.

A 2010 study published in the European Heart Journal by Nazzareno Galiè et al. reported that before 1999, when the first drug was approved for treating PH, the life expectancy of patients diagnosed with PH was less than three years.

This condition, while relatively rare, should not be underestimated. Its prevalence, though challenging to pinpoint exactly due to its various types and classes, highlights a critical need for awareness and understanding.

For clinical relevance, the World Health Organization (WHO) has classified PH into various types based on their causative factors, as explained below.

• Type 1 (Pulmonary arterial hypertension – PAH)
• Type 2 (Left heart disease)
• Type 3 (Lung disease)
• Type 4 (Chronic thromboembolic pulmonary hypertension – CTEPH)
• Type 5 (Miscellaneous)

Each type progresses through various classes, from mild to severe, indicating the progression and severity of the condition.

A primary symptom of PH is shortness of breath, often first noticed during physical activity. This symptom is a key indicator and frequently why individuals seek medical attention.

The main causes of PH are diverse, ranging from genetic factors and heart diseases to lung conditions and blood clots.

Regarding diagnosis, echocardiograms are the primary non-invasive method that employs sound waves to reconstruct images of the heart, helping detect PH and assess its severity.

Treatment for PH is multifaceted and depends on the type and stage of the condition. Often, it involves medications, oxygen therapy, and, in severe cases, lung transplantation.

According to a 2023 STATPearl publication by Dr. Sean H. Oldroyd and colleagues, the main danger of untreated PH is right heart failure and impending death, underscoring the importance of early detection and treatment.

Prevention is tricky since many causes of PH are out of one’s control. However, adopting a healthy lifestyle, which includes maintaining a proper weight, quitting smoking, and engaging in regular exercise, can help alleviate the impact of certain risk factors.

Tracking blood pressure at home is crucial for those at risk. It involves regular monitoring using a blood pressure monitor.

Among the top blood pressure monitors healthcare professionals recommend are the Oxiline Pressure X Pro and CheckMe BP2.

Other options to consider are the Omron platinum blood pressure monitor, the A&D medical upper arm blood pressure monitor, and the Withings BPM connect.

Each offers a blend of accuracy, ease of use, and connectivity features, making them valuable tools for managing and understanding your heart health.

What are the types of pulmonary hypertension?

As mentioned earlier, the WHO has classified PH into five types based on their underlying causes and progressing symptoms. These five types are explained below the image in detail.

Pulmonary arterial hypertension (PAH) – Group 1

PAH is distinct due to its direct impact on the pulmonary arteries. It involves complex cellular-level changes leading to remodeling of the pulmonary arterial walls.

Increased resistance in these arteries burdens the right side of the heart, as it must work harder to pump blood through narrowed vessels.

PAH is unique among PH types in its pathophysiology and has been extensively researched, resulting in various targeted therapies.

Pulmonary hypertension due to left heart disease – Group 2

This type focuses on how conditions of the left heart affect the lungs. Increased pressure in the left heart due to various heart diseases eventually affects the pulmonary circulation.

This back-pressure plays a key role in developing PH in these patients.

Management strategies often revolve around addressing the underlying left heart conditions and understanding the interaction between heart and lung dynamics is crucial for treatment.

Pulmonary hypertension due to lung diseases – Group 3

This form of PH is closely related to lung health and pulmonary airways. Chronic changes in lung tissue and airway function can significantly impact pulmonary arteries.

The complex interplay between lung pathology and pulmonary arterial pressure requires optimizing lung function alongside managing pulmonary pressure.

Chronic thromboembolic pulmonary hypertension (CTEPH) – Group 4

CTEPH is unique among PH types due to its association with lung blood clots. These clots can lead to persistent obstructions in the pulmonary arteries, resulting in long-term lung changes.

Managing CTEPH often involves surgical interventions, such as pulmonary thromboendarterectomy, to remove obstructing clots.

Pulmonary hypertension with unclear and/or multifactorial mechanisms – Group 5

This group represents a diverse and complex category. Multiple underlying factors may contribute to PH in these cases, making diagnosis and management challenging.

The heterogeneity of this group necessitates highly individualized treatment plans, often requiring a multidisciplinary approach to address various contributing factors.

Common symptoms across all five types include breathlessness and exercise intolerance, which are characteristic of pulmonary hypertension.

According to the Pulmonary Hypertension Association (PHA), the diverse causes and pathophysiology of pulmonary hypertension are crucial for precise diagnosis and personalized treatments, while shared clinical features simplify communication and decision-making, justifying this classification.

The upcoming sections will provide further insights into the symptoms, causes, and various classes of this disease.

What are the symptoms of pulmonary hypertension?

The symptoms of PH can vary depending on the stage and severity of the condition. However, there are several common symptoms that many individuals with PH may experience, as explained below the image.

• Shortness of breath (dyspnea): This is often the most noticeable symptom, initially occurring during physical exertion and potentially progressing to occurring even at rest in more advanced stages.
• Fatigue: Patients frequently report feeling unusually tired or weak, which can significantly impact their daily activities.
• Chest pain or pressure: This can be experienced, particularly during physical activity, as the heart works harder to pump blood through narrowed pulmonary arteries.
• Dizziness or syncope (fainting spells): As PH progresses, reduced blood flow can lead to episodes of dizziness or fainting, especially after exertion.
• Swelling (edema): Swelling often occurs in the ankles, legs, and, eventually, the abdomen due to fluid retention. This is a sign of worsening right heart function.
• Palpitations: Irregular heartbeats (arrhythmias) can lead to a feeling of fluttering or thumping in the chest.
• Cyanosis: A bluish tint on the lips and skin is caused by reduced oxygen levels in the bloodstream.
• Exertional chest pain: Some patients may experience chest pain during exercise or strenuous activities.

These symptoms are not exclusive to PH and can be associated with various other health conditions.

Hence, it is vital for individuals with these symptoms to seek medical assessment to receive a precise diagnosis and suitable treatment.

Early detection and treatment of PH can significantly improve the quality of life and outcomes for those affected by this condition.

Indeed, a 2015 assessment conducted by researchers from the University of North Carolina recommends that when assessing patients experiencing breathlessness and exercise intolerance, healthcare providers should consistently include pulmonary hypertension as a potential diagnosis to consider.

They recommend using right heart catheterization, echocardiography, and other symptoms to determine the specific type of PH.

What are the classes of pulmonary hypertension?

According to the 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension, PH is often classified based on the severity of symptoms and their impact on a patient’s activities and bodily functions.

Originally derived from the functional classification established by WHO in 1998, it is divided into 4 classes or classes, as outlined below.

Class I

• This is the most severe class.
• Patients in this class maintain normal physical activity levels without symptoms.
• They can perform daily tasks like walking or climbing stairs without experiencing breathlessness, fatigue, chest pain, or near fainting.
• Their daily life is not restricted by PH.

Class II

• These patients experience a slight limitation in physical activities.
• While they feel comfortable during rest, engaging in routine activities such as light housework or moderate walking causes symptoms.
• This class indicates a mild impact of PH on daily life.

Class III

• Individuals in this class face significant limitations due to PH.
• They are symptom-free at rest, but performing less than ordinary activities, such as short walks or minimal exertion, leads to symptoms.
• This class represents a moderate to severe impact on PH, limiting daily activities considerably.

Class IV

• Patients are unable to perform any physical activity without experiencing symptoms.
• Symptoms are present even at rest, and there may be evidence of right heart failure.
• Simple activities like dressing or bathing can exacerbate symptoms, indicating a severe impact of PH on daily life and overall health.

These classes reflect the progression of PH and its impact on the patient’s lifestyle and activities, guiding treatment and management strategies.

It is worth noting that the categorization of PH serves two distinct purposes: types categorize PH based on underlying causes and pathophysiology, while classes assess its severity and impact on daily activities.

PH types encompass various causes, while classes range from no limitation of physical activity to an inability to engage in any activity without symptoms, indicating functional impairment.

 These classifications provide a comprehensive understanding of PH, addressing both its origins and the extent of its impact on patients.

By now, you may be familiar with the various grades of this disease, but how does it all occur? The next section will help you understand the causative factors of PH.

What are the causes of pulmonary hypertension?

The causes of each pulmonary hypertension group are well-documented in many studies.

Dr. Javier Perez-Fernandez, a Critical Care and Pulmonary Medicine Physician at Baptist Hospital explains that:

“It occurs due to factors like the lack of oxygen in the blood. In a normal lung, blood vessels distribute oxygenated arterial blood (represented in red) and return venous blood (in blue) to the heart.”

He adds:

“However, in pulmonary hypertension, certain vessels thicken and narrow, increasing pressure. This condition is particularly dangerous as lung blood vessels typically operate under low pressure.”

Furthermore, he states:

“The heightened pressure strains the heart and can lead to severe health issues, emphasizing the importance of managing pulmonary hypertension in pulmonary fibrosis patients.”

The NHLBI-NIH has made clear distinctions for each, as explained below.

Group 1 (pulmonary arterial hypertension – PAH)

• Group 1 PH, or pulmonary arterial hypertension (PAH), results from alterations in the small lung arteries.
• These alterations involve the constriction and rigidity of these arteries, impeding the smooth flow of blood.This can be due to genetic factors, certain drugs, or associated with other conditions like connective tissue diseases.
• The increased resistance in these small arteries means the heart must work harder to pump blood through the lungs, leading to increased pressure in the pulmonary arteries.

Group 2 (PH due to left heart disease)

• Group 2 PH is primarily caused by conditions affecting the left side of the heart.
• This includes issues such as heart failure where the heart muscle is weak and doesn’t pump effectively, and valvular heart diseases like mitral valve or aortic valve disease.
• In these conditions, the impaired function of the left heart leads to increased pressure in the pulmonary circulation (the blood vessels in the lungs), resulting in PH.
• Essentially, the problems in the left heart create a backlog of blood, increasing the pressure in the lungs.

Group 3 (PH due to lung diseases and/or hypoxia)

• Group 3 PH typically arises from lung diseases and conditions resulting in decreased oxygen levels, known as hypoxia.
• Common lung diseases within this category include Chronic Obstructive Pulmonary Disease (COPD), interstitial lung disease, and other chronic conditions that harm the lung’s airways or tissue.
• When lung damage occurs or the oxygen supply is inadequate, the blood vessels in the lungs may constrict and become less flexible, elevating the pressure within them.
• Consequently, the heart faces greater difficulty in propelling blood through the lungs, resulting in the development of PH.

Group 4 (chronic thromboembolic pulmonary hypertension – CTEPH)

• Group 4 PH, known as Chronic Thromboembolic Pulmonary Hypertension (CTEPH), is primarily caused by chronic blood clots in the lungs.
• These blood clots block or narrow the pulmonary arteries, the blood vessels in the lungs. When these arteries are blocked or narrowed, it becomes difficult for blood to flow through them, leading to increased pressure in these vessels.
• CTEPH can develop after a person has had acute pulmonary embolism, which are blood clots that travel to the lungs.

Group 5 (PH with unclear and/or multifactorial mechanisms)

• Group 5 PH includes cases where the exact cause is not clear or involves several different factors.
• This group might include PH due to blood disorders, systemic diseases like sarcoidosis, or metabolic disorders.
• The complexity here is that these conditions don’t directly target the lungs or heart, but they somehow contribute to increasing pressure in the pulmonary arteries.
• It’s a bit like a puzzle where different health issues combine to create a unique situation in each patient, leading to PH.

This condition might appear treatable, but there are several risk factors associated with it, and risk assessment is one crucial tool that doctors use before prescribing medicines. Below, you will find more information about it.

What are the risk factors of pulmonary hypertension?

The risk factors for PH encompass various health conditions and lifestyle factors.

A 2015 study by a team of researchers from the Centers for Disease Control and Prevention (CDC) analyzed data from 2001 to 2010 on PH mortality and hospitalization trends.

Some key risk factors identified in this analysis are listed below.

• Heart and lung diseases: Conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, and left heart disease can increase the risk.
• Family history: Genetic predisposition plays a role, especially in pulmonary arterial hypertension (PAH).
• Living at high altitudes: Residing in high-altitude areas for prolonged periods can lead to an increased risk due to lower oxygen levels.
• Use of certain drugs: Some diet drugs and illicit substances like stimulants can increase the risk.
• Connective tissue disorders: Diseases like scleroderma and lupus are associated with a higher risk of developing PH.
• Blood clots in the lungs: A history of pulmonary embolism can increase the risk of chronic thromboembolic pulmonary hypertension (CTEPH).
• Other Medical Conditions: Liver disease and sickle cell disease can also be risk factors. Additionally, conditions such as Down syndrome, Gaucher disease, HIV, and liver disease may contribute to the risk.
• Age: The risk increases with age, typically diagnosed between 30 and 60 years.
• Environment: Exposure to asbestos and certain infections caused by parasites.
• Genetics: Some genetic disorders, including Down syndrome, congenital heart disease, and Gaucher disease, can raise the risk.
• Lifestyle habits: Smoking and illegal drug use are lifestyle choices that can increase the risk.
• Sex: The condition is more common in women than in men.

A thorough understanding of these risk factors can help your doctors correctly assess your condition and decide on a treatment regimen that will be more effective.

If you are curious about how they diagnose PH, the upcoming section will help you understand that.

How to diagnose pulmonary hypertension & what are the tests for that?

Diagnosing PH is a multi-step process crucial for identifying the condition and determining the best treatment approach.

Below is an overview of its clinical diagnosis as reported in the European Respiratory Journal in an article titled “Diagnosis of pulmonary hypertension” by a team of US clinicians.

Initial assessment

• Medical history and symptoms review: Gathering information about the patient’s symptoms, medical history, family background, and risk factors.
• Physical examination: Checking for physical signs of PH, including a loud pulmonary valve sound, swollen legs or abdomen, and bluish skin color.

Diagnostic tests

• Echocardiogram: A non-invasive heart ultrasound assessing heart chamber dimensions, valve health, pulmonary artery pressure, and heart function.
• Chest X-ray: To detect enlarged pulmonary arteries and lung changes.
• Electrocardiogram (ECG or EKG): Evaluating heart electrical activity for irregularities.
• Pulmonary function tests: Assessing lung function and exercise capacity.
• Six-minute walk test: Measuring the distance walked in six minutes to evaluate exercise capacity and predict prognosis.

Confirmatory testing

• Right heart catheterization: The definitive PH diagnosis test involving catheter insertion into a vein to measure right heart and lung pressure.
• Blood tests: Identifying potential underlying conditions like HIV, liver disease, or autoimmune disorders.
• CT scan or MRI: Utilizing advanced imaging to search for lung clots or anomalies.
• Ventilation-perfusion (V/Q) scan: Specifically used to identify chronic lung blood clots.

Risk assessment

• After diagnosis, risk assessment determines disease severity and guides treatment.

It’s important to note that PH shares symptoms with other heart and lung conditions, emphasizing the need for accurate diagnosis. Approved hospital tests for diagnosing this condition are discussed further below.

Can pulmonary hypertension be cured?

According to NHS UK, there is currently no known cure for PH, meaning that no treatment can completely eliminate the condition or restore the pulmonary arteries to a fully normal state.

However, the ailment can be effectively managed through a variety of treatments aimed at alleviating symptoms, enhancing the quality of life, and slowing disease progression.

The nature of pulmonary hypertension makes it a particularly challenging disease to address.

It often emerges as a secondary condition, resulting from complications of other underlying health issues like heart disease, lung disease, or connective tissue disorders.

This multifaceted origin contributes to the complexity of finding a universal cure. Additionally, the disease can progress at different rates in different individuals, making it challenging to predict and treat uniformly.

Despite extensive research and advancements in understanding the disease, the medical community continues to face significant challenges in finding a cure.

The absence of a cure underscores the importance of early detection and diagnosis, which can aid in managing the condition more effectively.

The best part is that this condition can be treated in a way that alleviates the symptoms and helps you live with it. The upcoming section will help you learn more about treatments.

What are the treatments for pulmonary hypertension?

The treatment for pulmonary hypertension centers on symptom management, enhancing quality of life, and decelerating the disease’s advancement.

Since PH can be associated with a variety of conditions, treatment plans are often tailored to the individual’s specific type of PH and the underlying cause.

Below is a list of treatments explained in the American Lung Association guidelines.

This information comes from various studies, the most prominent one being published by a team of researchers from the University of Utah in Current Cardiovascular Risk Reports headed by Dr. Jennalyn D. Mayeux.

Medications

A variety of medications are used to manage PH.

• Prostacyclin analogs: These medications aid in the expansion of pulmonary blood vessels and enhance pulmonary blood circulation.
• Endothelin receptor antagonists (ERAs): These drugs inhibit endothelin, a substance responsible for narrowing blood vessels, thereby assisting in the relaxation of the pulmonary arteries.
• Phosphodiesterase-5 (PDE-5) inhibitors: These medications aid in muscle relaxation within the blood vessels, enabling them to expand.
• Guanylate cyclase stimulators: Similar to PDE-5 inhibitors, these drugs help relax the pulmonary arteries and increase blood flow.
• Anticoagulants: Often prescribed to prevent blood clots, which are a risk in PH patients.
• Diuretics: Utilized for eliminating surplus bodily fluids, which can alleviate the burden on the heart.

Oxygen therapy

For individuals experiencing reduced oxygen levels in their bloodstream, oxygen therapy can be beneficial in enhancing their symptoms.

Surgical and procedural interventions

In certain cases, more invasive treatments may be necessary.

• Lung or heart-lung transplant: This is considered for severe cases of PH when other treatments have failed.
• Atrial septostomy: A surgical procedure that establishes a connection between the upper left and right heart chambers (atria) in order to alleviate pressure on the right side of the heart.

Lifestyle changes and supportive therapies

Patients are often advised to do the following.

• Engage in supervised exercise programs tailored to their condition and tolerance level.
• Follow a diet plan devoid of excess fat.Avoid smoking and limit alcohol consumption.
• Get vaccinated against respiratory infections like influenza and pneumonia, which can exacerbate PH.

Pulmonary rehabilitation

A comprehensive program encompassing exercise training, education, and support for both patients and their families.

The treatment selection is influenced by various factors, such as the type and severity of PH, the presence of underlying conditions, and the patient’s overall health.

Typically, treatment plans incorporate a blend of medications and complementary therapies to address the condition effectively.

Patients with PH need to be managed by a team of specialists experienced in treating this complex condition. Regular follow-up and monitoring are crucial to adjust treatment plans as needed and address any complications.

However, there is a promising trend where many clinicians are increasingly exploring using natural methods. More details are explained below.

What are the natural ways to treat pulmonary hypertension?

There are several natural methods for managing PH, ranging from your daily habits to your diet.

The American Heart Association (AHA), the European Society of Cardiology (ESC), and the Pulmonary Hypertension Association (PHA) have conducted thorough studies on this matter and have provided a foundation for it.

It’s important to emphasize that these methods should complement rather than replace prescribed medical treatments.

Below are some natural strategies compiled from the AHA, ESC, and PHA guidelines that may benefit individuals with PH.

Dietary modifications

Lowering salt consumption can help lower blood pressure and prevent fluid retention, reducing the strain on the heart.

Consuming a diet rich in berries, citrus fruits, veggies, grains, lean proteins, and healthy fats contributes to cardiovascular health.

For instance, a 2020 study published in the journal Nutrients suggests that flavonoids such as quercetin, isoflavonoids such as genistein, and other dietary polyphenols including resveratrol found in berries, fruits, and nuts may slow the progression of PH.

Physical activity

Engaging in regular, moderate exercise can enhance cardiovascular fitness and endurance. However, exercise plans should be discussed with a healthcare provider to tailor them to individual capabilities and limitations.

A 2022 study published by a team of clinicians from St. Vincent’s Clinical School, Sydney, Australia, correlates exercise as a safe approach to improving the quality of life in PH patients.

Stress management

Engaging in techniques like deep breathing, meditation, yoga, and mindfulness can assist in effectively managing stress, which can be advantageous for heart health.

An alarming study published in the journal Vascular Health Risk Management in 2018 reports a 50% increased risk of PH in people with depression or anxiety, emphasizing the need for its management.

Weight control

Maintaining a healthy weight eases the burden on the heart and can help in managing PH symptoms. Healthcare providers can suggest safe weight management strategies.

One of the popular studies by Prof. Scott E. Friedman and Bruce W. Andrus, published in the Journal of Obesity, highlights an around 40% increased risk associated with PH and obesity.

Avoiding smoking

Smoking can exacerbate PH symptoms and damage the cardiovascular system, making quitting essential.

In a 2012 study reported in the journal Pulmonary Circulation, it was found that the mean arterial blood pressure increased by 25 mmHg in smokers who eventually developed chronic COPD, leading to lung transplantation.

Supplements and herbal remedies

Some supplements and herbs, including garlic, omega-3 fatty acids (from fish or flaxseed oil), and antioxidants, are believed to offer cardiovascular advantages.

However, their effectiveness for PH specifically is not firmly established, and some supplements can interact with medications.

A study conducted by Beijing University of Chinese Medicine in China examined how natural products such as Salvia miltiorrhiza Bunge, grapes, red wine, peanuts, soybeans, and various other plants can contribute to the management of PH.

Home oxygen therapy

Individuals with PH and low blood oxygen levels may find relief and enhanced quality of life through home oxygen therapy.

There are many supporting studies for this, such as a study published by Prof. Harrison W. Farber and his team in the Journal of Heart and Lung Transplantation. It was observed that the fatality rate for patients who used supplemental oxygen was comparatively lower than those who didn’t.

Pulmonary rehabilitation

While not strictly natural, participation in a pulmonary rehabilitation program encompassing supervised exercise, education, and support can substantially enhance endurance, symptom control, and quality of life for PH patients.

A 2023 Cochrane review, published by Dr. Morris NR, highlights how a rehabilitation approach could significantly improve the quality of life in PH patients.

Although medical interventions are vital for the management of PH, alternative methods and lifestyle modifications exist that can enhance overall health and potentially mitigate symptoms.

It’s essential for individuals with pulmonary hypertension to work in close partnership with their healthcare team to create a comprehensive treatment strategy that includes natural and lifestyle methods.

The effectiveness of natural treatments can vary among individuals, and what works for one person may not work for another.

Prioritizing safety and compatibility with existing PH treatments is of utmost importance. Having said that, let us learn why PH is such a dangerous condition, especially if left unattended.

Why is pulmonary hypertension dangerous?

PH is considered dangerous due to its serious impact on the heart and lungs, leading to significant health complications if left untreated. Here are the main reasons explained in a 2012 newsroom writeup from Harvard Health.

Right heart failure

PH causes high blood pressure in the pulmonary arteries, which are responsible for carrying blood from the right side of the heart to the lungs. Over time, the heart’s right ventricle must work harder to pump blood through these vessels.

This increased workload can lead to right ventricular hypertrophy (thickening of the heart muscle) and, ultimately, right heart failure, a condition where the heart can’t pump blood efficiently.

Decreased oxygen supply

Elevated pressure within the pulmonary arteries may hinder the exchange of oxygen and carbon dioxide in the lungs.

This inefficiency can lead to decreased oxygen levels in the blood (hypoxemia), affecting the oxygen supply to the body’s organs and tissues, impairing their function, and exacerbating fatigue and shortness of breath symptoms.

Progressive symptoms

Symptoms of PH, such as shortness of breath, fatigue, dizziness, and chest pain, tend to worsen over time, especially if the condition is not adequately managed.

This progression can significantly impair the quality of life, limiting physical activity and causing disability.

Risk of clotting and bleeding

People with PH are at an increased risk of forming blood clots in the small arteries of the lungs, which can further obstruct blood flow.

There’s also a risk of bleeding due to the strain on the circulatory system and the potential side effects of certain medications.

Complications from low blood pressure

Advanced PH can lead to low blood pressure (hypotension), especially in the systemic arteries. This can cause fainting and is particularly dangerous when it occurs during physical activity.

Due to these risks, early diagnosis and appropriate management of PH are crucial to improve outcomes and reduce the likelihood of severe complications.

Treatment strategies often involve medications to lower pulmonary blood pressure, oxygen therapy, lifestyle modifications, and, in some cases, surgical interventions.

Despite these treatments, managing PH remains challenging, and the condition can significantly affect life expectancy and quality of life, underscoring the importance of specialized care and ongoing research into more effective therapies.

But can this condition turn fatal? Let’s explore this in the next section.

Is pulmonary hypertension fatal?

PH can be a severe and potentially life-threatening condition if not properly managed. Over time, this strain can result in right heart failure, among other complications, significantly impacting survival and quality of life.

Prof. Marius M. Hoeper from Hannover Medical Center refers to this as a ‘life-threatening rare disease’ in a multi-author publication in Deutsches Ärzteblatt International.

The outlook for pulmonary hypertension varies depending on factors like its underlying cause, diagnosis timing, and treatment response.

Early stages of PH might be managed more effectively with current therapies, which can improve symptoms, enhance quality of life, and potentially slow the progression of the disease.

However, as the condition advances, it becomes more challenging to manage, and the risk of fatal complications increases.

The type of pulmonary hypertension also influences the prognosis. For example, pulmonary arterial hypertension (PAH, classified as Group 1 PH) has historically been considered among the more severe forms of the condition, with a significant impact on life expectancy if untreated.

However, advancements in treatment over the past few decades have improved the prognosis for many patients with PAH.

Despite these advancements, PH remains a serious condition that requires ongoing medical care. Regular follow-up with healthcare providers specializing in pulmonary hypertension is essential for monitoring the disease and adjusting treatment as necessary.

With appropriate management, many individuals with PH can lead active lives, although they may face limitations depending on the severity of their condition.

It’s important to approach information about pulmonary hypertension with an understanding that individual experiences can vary widely. Patients and families dealing with PH need medical attention, and you can learn when to call your doctor below.

When should you call your healthcare provider for pulmonary hypertension?

You should reach out to your healthcare provider in specific circumstances crucial for ongoing disease management and to prevent exacerbations.

Such instances include a noticeable increase in the severity or frequency of symptoms like shortness of breath, fatigue, or dizziness, which could indicate disease progression.

According to Mayo Clinic guidelines, the emergence of new symptoms, such as unexplained swelling or rapid weight gain, could suggest complications like fluid retention and also necessitate a call to your healthcare team.

Moreover, experiencing adverse reactions to PH medications or encountering signs of potential infections (fever, coughing, etc.) requires immediate communication with a healthcare provider to adjust treatment plans or address the infection, given the potential for worsening PH conditions.

If you’re considering adding any new medications or supplements to your regimen, it’s important to consult with your healthcare provider first to avoid negative interactions with your current PH treatments.

Life changes, such as planning for pregnancy, also call for a detailed discussion with your healthcare provider due to the significant risks and management complexities PH presents in such situations.

Similarly, informing your healthcare team about upcoming surgeries or procedures is critical, as these situations require special precautions for individuals with PH.

Maintaining regular contact with your healthcare provider for routine monitoring, even without acute changes, is essential for effectively managing PH.

This ensures that any necessary adjustments to your treatment strategy can be made promptly, aiding in stabilizing your condition and improving your overall well-being.

This guidance is geared towards managing your condition under normal circumstances and ensuring that your healthcare provider can provide tailored advice and interventions.

It’s distinct from scenarios that warrant immediate emergency room visits, focusing instead on the proactive management and prevention of potential complications associated with PH.

But there are certain scenarios you must be aware of that may require a visit to the ER. Read further to educate yourself.

When should you go to the ER for pulmonary hypertension?

For individuals with PH,  recognizing when to seek emergency medical attention is crucial due to the serious nature of the condition.

According to the guidelines with PHA, below are the cases when immediate ER visits are warranted.

• Severe shortness of breath: If you suddenly and severely struggle to breathe, and this persists even after resting or taking your regular medications, it may signal a significant deterioration of your condition or the possibility of a pulmonary embolism.
• Chest pain: Immediate attention is necessary if you experience severe or worsening chest pain, particularly if it differs from your usual symptoms and is accompanied by other symptoms.
• Syncope (fainting): Episodes of fainting or severe dizziness could signal a severe drop in oxygen levels or heart complications, necessitating urgent evaluation.
• Blue lips or skin (cyanosis): This symptom indicates a significant reduction in oxygen levels in your blood, requiring prompt medical intervention.
• Rapid heartbeat or palpitations: If accompanied by discomfort, shortness of breath, or dizziness, a rapid heartbeat can be a sign of worsening PH or cardiac complications.
• Uncontrollable symptoms: If your PH symptoms suddenly become unmanageable or significantly worsen, and you cannot stabilize them with your routine care at home, this indicates a need for emergency evaluation.
• Signs of a blood clot: Sudden leg swelling, pain in a single leg (typically the calf), warmth, redness, or changes in leg coloration may indicate a deep vein thrombosis, which is considered an emergency, particularly for those with PH, as it leads to fatal pulmonary embolism.

These situations represent potentially life-threatening complications of pulmonary hypertension and require immediate medical evaluation to provide necessary interventions that could be lifesaving.

Cleveland Clinic guidelines also suggest that a sudden episode of paralysis could potentially be associated with PH, and therefore recommends, an ER visit.

PH affects the body’s ability to circulate blood effectively, particularly through the lungs, and any acute worsening of symptoms or new cardiovascular symptoms can indicate a serious risk to your health.

Emergency departments are equipped to handle such crises, providing interventions that can stabilize your condition.

How to prevent pulmonary hypertension?

PH prevention involves a multifaceted approach that targets reducing risk factors and managing underlying health conditions that could lead to its development.

According to guidelines from leading health organizations such as the PHA, AHA, CDC, and Mayo Clinic, it’s essential to manage existing health issues like heart and lung diseases, connective tissue disorders, liver diseases, and HIV/AIDS with diligence.

Treatment and regular monitoring for conditions such as obstructive sleep apnea are also crucial, as they can significantly impact the risk of developing PH.

Additionally, lifestyle adjustments, including preventing blood clots through hydration, movement during long periods of sitting, and possibly using compression stockings or medication as recommended by a healthcare provider, are key preventative measures.

Exposure to harmful substances, including certain illegal drugs and diet medications, should be avoided to lower the risk of PH.

For women with PH or at high risk, discussing pregnancy plans with healthcare providers is vital due to the significant risks involved.

Prompt treatment and vaccination against respiratory infections also play a role in preventing exacerbations of underlying lung conditions that could lead to or worsen PH.

This compilation of preventive strategies reflects the consensus among major health organizations and underscores the importance of a proactive approach to reducing the risk of pulmonary hypertension.

Poorly managed systemic hypertension can contribute to overall cardiovascular disease, which can affect the heart’s ability to pump efficiently.

This inefficiency can indirectly influence the development of PH by altering heart function and the dynamics of blood circulation between systemic and pulmonary circulations.

The upcoming section will provide a thorough understanding of blood pressure monitors that can help you monitor cardiovascular health and prevent PH.

How to track blood pressure at home?

Tracking blood pressure at home is an effective way to monitor your cardiovascular health and can help manage conditions like hypertension.

Below elaborated is a step-by-step guide to accurately measure your blood pressure at home.

Choose the right equipment

• Use an automatic, cuff-style, bicep (upper-arm) blood pressure monitor.
• These monitors are often recommended for home use because they are easy to use and provide reliable readings.
• Make sure the cuff fits your arm properly; a cuff that’s too large or too small can give inaccurate readings.

Prepare for measurement

• Rest for 5 minutes before taking your measurement. Sit in a comfortable position with your back supported and feet flat on the floor.
• Refrain from consuming caffeine, engaging in physical activity, or smoking for a minimum of 30 minutes prior to measuring your blood pressure.
• Empty your bladder for a more accurate reading.
• Remove any tight-sleeved clothing from your arm.
• Position the cuff on your exposed upper arm, approximately one inch above the crease of your elbow.

Correct positioning

• Sit in a chair with your back straight and supported. Do not cross your legs.
• Place your arm on a flat surface, such as a table, with the upper arm at heart level.
• Make sure the cuff fits comfortably, neither too loose nor excessively tight, and is correctly positioned as per the monitor’s guidelines.

Take the measurement

• Adhere to the guidelines given for your blood pressure monitor. Press the button to initiate the inflation process.
• Stay quiet and still during the measurement. Moving or talking can affect your reading.
• Wait until the machine completes the measurement and displays your blood pressure reading on the screen, which will show both systolic (the higher number) and diastolic (the lower number) pressures.

Share your findings

Share your blood pressure log with your healthcare provider at regular visits to help manage your blood pressure more effectively.

Remember, while home blood pressure monitoring can provide useful information about your blood pressure trends and help you manage hypertension, it should not replace regular check-ups with your healthcare provider.

Below are some details on the best and clinically approved blood pressure monitors that you can incorporate into your daily routine.

Which blood pressure monitors are recommended as top choices?

The best blood pressure monitors come from Oxiline and CheckMe, two top-selling brands recommended by many clinicians.

According to a 2006 study by S. Provencher and a team published in the European Respiratory Journal, heart rate variations have significant correlations with PH, and both of these blood pressure monitors have dedicated sensors to detect accurate heart rate variation.

Below is a quick comparison between the top two contenders in the blood pressure monitor list.

More in this topic