Pulmonary Hypertension: Symptoms & Treatment
Pulmonary hypertension is a serious condition involving high blood pressure in the blood vessels that carry blood from the heart to the lungs. It can make the right side of the heart work harder than normal. Over time, this extra strain may weaken the heart and lead to heart failure.
The condition can develop for several reasons, including diseases of the heart, lung conditions and blood clots in the pulmonary arteries. Although some forms are uncommon, early diagnosis and appropriate treatment can help manage symptoms and reduce complications.
This form requires specialist assessment because its diagnosis and treatment differ from those of other types of pulmonary hypertension.
This guide explains the symptoms of pulmonary hypertension, its causes, how doctors diagnose it, treatment options and how HubPharm supports patients managing cardiovascular conditions.
What Is Pulmonary Hypertension?
Pulmonary hypertension (PH) means that pressure in the blood vessels supplying the lungs is abnormally high.
Normally, blood travels from the right side of the heart through the pulmonary arteries to the lungs, where it receives oxygen. It then returns to the left side of the heart to be pumped around the body.
When pressure in the pulmonary arteries rises, the right side of the heart must work harder to move blood through the lungs. Over time, this can cause the heart’s right pumping chamber to enlarge or weaken.
Pulmonary hypertension is different from ordinary high blood pressure. Ordinary hypertension affects the arteries throughout the body, while pulmonary hypertension affects the blood vessels between the heart and lungs. A person can have either condition or both.
Types of Pulmonary Hypertension
Pulmonary hypertension is not one single disease. Doctors classify it into five main groups based on its cause.
1. Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) affects the small arteries in the lungs. These blood vessels can become narrowed or stiff, increasing resistance to blood flow.
PAH may be associated with inherited conditions, certain connective tissue diseases, congenital heart disease or other specific causes. In some cases, the cause remains unknown.
This form requires specialist assessment because its diagnosis and treatment differ from those of other types of pulmonary hypertension.
2. Pulmonary Hypertension Caused by Left Heart Disease
Conditions affecting the left side of the heart can increase pressure in the blood vessels leading from the lungs to the heart.
Examples include heart failure and certain heart valve problems. Treatment usually focuses on managing the underlying heart condition.
3. Pulmonary Hypertension Caused by Lung Disease or Low Oxygen
Some lung diseases and conditions that reduce oxygen levels can contribute to pulmonary hypertension.
Examples include chronic obstructive pulmonary disease (COPD), certain interstitial lung diseases and sleep-related breathing disorders.
The treatment plan depends on the underlying lung condition and the severity of the pulmonary hypertension.
4. Chronic Thromboembolic Pulmonary Hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH) develops when persistent or organised blood clots obstruct the pulmonary arteries.
It may occur after pulmonary embolism, although not everyone who experiences a pulmonary embolism develops CTEPH.
Some patients may benefit from specialist procedures as well as medical treatment.
5. Pulmonary Hypertension With Other or Unclear Causes
The fifth group includes less common conditions associated with pulmonary hypertension, including certain blood disorders, systemic diseases and other complex medical conditions.
Correct classification matters because treatment that helps one type of pulmonary hypertension may not be appropriate for another.
What Causes Pulmonary Hypertension?
The causes depend on the type of pulmonary hypertension.
Common associated conditions include:
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Left-sided heart failure
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Heart valve disease
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Chronic lung diseases
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Previous blood clots in the lungs
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Certain inherited conditions
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Connective tissue diseases, including systemic sclerosis
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Some congenital heart conditions
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Conditions that cause persistently low oxygen levels
In some cases, medicines or other medical conditions may contribute to the development of pulmonary hypertension. A specialist investigates the likely cause before deciding on treatment.
Symptoms of Pulmonary Hypertension
The symptoms of pulmonary hypertension can develop gradually. Early signs may be subtle, and they can resemble symptoms of other heart or lung conditions.
Common symptoms include:
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Shortness of breath: Breathlessness during walking, climbing stairs or other everyday activities.
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Fatigue: Unusual tiredness or reduced stamina.
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Chest discomfort: Pressure or pain in the chest, particularly during activity.
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Dizziness: Feeling lightheaded, especially during exertion.
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Fainting: Some people may lose consciousness, particularly when the condition is advanced.
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Heart palpitations: A racing or pounding heartbeat.
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Swollen ankles and legs: Fluid may accumulate as the right side of the heart struggles to pump effectively.
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Abdominal swelling: Fluid may collect in the abdomen in more advanced disease.
These symptoms do not confirm pulmonary hypertension. Other conditions can produce similar problems, so medical assessment is essential.
When Is Pulmonary Hypertension an Emergency?
Seek emergency medical care if you experience severe or sudden difficulty breathing, persistent chest pain, collapse or fainting accompanied by concerning symptoms.
Sudden breathlessness and chest pain can also indicate a pulmonary embolism or another serious medical emergency.
Do not assume that new or severe symptoms are simply part of an existing diagnosis.
How Is Pulmonary Hypertension Diagnosed?
Diagnosing pulmonary hypertension can take time because its symptoms overlap with those of other conditions.
Doctors combine medical history, physical examination, imaging and specialised tests to establish the diagnosis and identify its cause.
1. Medical History and Examination
A healthcare professional may ask about breathlessness, fatigue, chest discomfort, fainting, family history and existing heart or lung conditions.
They may also examine the heart and lungs, check for swelling and measure oxygen levels.
2. Echocardiogram
An echocardiogram uses ultrasound to create images of the heart.
It can help estimate the likelihood of pulmonary hypertension, assess the right side of the heart and identify other heart conditions.
However, an echocardiogram alone does not definitively confirm pulmonary hypertension.
3. Right Heart Catheterisation
Right heart catheterisation measures pressure inside the heart and pulmonary arteries using a thin catheter.
It is the key test used to confirm pulmonary hypertension and assess the pressures involved. The procedure takes place in a specialist medical setting.
4. Electrocardiogram
An electrocardiogram (ECG) records the heart’s electrical activity. It can identify rhythm abnormalities and may reveal signs that the right side of the heart is under strain.
5. Lung Function Tests and Chest Imaging
Lung function tests assess how well the lungs work. Chest imaging can help identify lung disease, changes in the heart or other possible causes of symptoms.
6. Blood Tests and Additional Investigations
Blood tests may help identify associated conditions and assess general health.
Doctors may also request a ventilation-perfusion (V/Q) scan or other specialised imaging to investigate whether blood clots are obstructing the pulmonary arteries.
The exact investigations depend on the suspected type of pulmonary hypertension.
Pulmonary Hypertension Treatment
Treatment depends on the type of pulmonary hypertension, its cause, the severity of symptoms and the patient’s overall health.
Some patients need treatment for an underlying heart or lung condition. Others require specialist medicines or procedures directed at the pulmonary arteries.
There is no single treatment that works for every type of pulmonary hypertension.
1. Treatment for the Underlying Cause
When pulmonary hypertension results from another condition, managing that condition is an important part of care.
For example:
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Heart failure may require appropriate heart failure treatment.
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Lung disease may require specialist respiratory care.
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Certain cases of chronic blood clot obstruction may need assessment for surgery or other specialist procedures.
Treating the underlying condition can improve management, although the response depends on the cause and the extent of damage.
2. Specialist Medicines for Pulmonary Arterial Hypertension
Some medicines are designed to relax pulmonary blood vessels or target pathways involved in pulmonary arterial hypertension.
These treatments may improve symptoms and functional capacity in selected patients. However, they require specialist diagnosis and monitoring.
Examples of medicine classes used in selected cases include:
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Endothelin receptor antagonists, which act on pathways involved in blood vessel narrowing.
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Phosphodiesterase-5 inhibitors, which affect pathways that help relax blood vessels.
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Prostacyclin pathway therapies, which help widen blood vessels and affect processes involved in clotting.
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Soluble guanylate cyclase stimulators, which act on another pathway involved in blood vessel relaxation.
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Other specialist therapies, which may be considered for eligible patients under current specialist guidance.
The choice depends on the confirmed diagnosis, risk assessment and response to treatment. Not all these medicines are appropriate for every type of pulmonary hypertension.
3. Diuretics for Fluid Retention
Some patients develop swelling because fluid accumulates in the body.
Doctors may prescribe diuretics to help remove excess fluid and relieve symptoms. These medicines may require monitoring of kidney function, blood pressure and electrolytes.
Diuretics manage fluid retention; they do not directly treat every underlying cause of pulmonary hypertension.
4. Oxygen Therapy
Some patients have low blood oxygen levels and may benefit from prescribed oxygen therapy.
Oxygen is not necessary for everyone with pulmonary hypertension. A healthcare professional assesses oxygen levels and the underlying condition before recommending it.
5. Anticoagulants for Selected Patients
Anticoagulants may be used in certain patients, particularly when there is a specific indication involving blood clots.
They are not automatically required for everyone with pulmonary hypertension. The decision depends on the underlying condition, clotting risk and bleeding risk.
6. Specialist Procedures
Some patients need assessment for procedures that address particular causes of pulmonary hypertension.
For example, selected patients with chronic thromboembolic pulmonary hypertension may be considered for pulmonary endarterectomy, a procedure that removes obstructing material from the pulmonary arteries. Other specialist interventions may be appropriate for patients who meet specific criteria.
These decisions require assessment by a pulmonary hypertension specialist team.
Can Lifestyle Changes Help?
Lifestyle measures can support overall health, but they do not replace the medical treatment needed for pulmonary hypertension.
Follow Your Care Plan
Attend scheduled appointments and follow your healthcare team’s advice about activity, monitoring and medicines.
Balance Activity and Rest
Your safe level of physical activity depends on the severity and type of pulmonary hypertension. Ask your specialist about appropriate exercise and whether cardiac or pulmonary rehabilitation is suitable.
Avoid pushing through significant breathlessness, chest discomfort or dizziness.
Avoid Tobacco
Smoking harms the lungs and blood vessels. Avoiding tobacco supports respiratory and cardiovascular health.
Discuss Travel and High Altitudes
Some patients may need additional advice about flying or visiting high-altitude locations because oxygen levels can be lower in these environments.
Discuss travel plans with your healthcare team if you have pulmonary hypertension, particularly if your symptoms are significant or you use prescribed oxygen.
Keep Follow-Up Appointments
Regular reviews help the care team assess symptoms, treatment response, side effects and changes in heart or lung function.
Do not change prescribed medicines or oxygen settings without advice from your healthcare team.
What Happens If Pulmonary Hypertension Is Not Treated?
Untreated or poorly controlled pulmonary hypertension can progressively strain the right side of the heart.
Possible complications include:
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Worsening breathlessness and fatigue
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Reduced ability to carry out everyday activities
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Fluid accumulation in the legs or abdomen
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Right-sided heart failure
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Fainting episodes
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Serious complications related to the underlying cause
The outlook varies according to the type of pulmonary hypertension, how early it is diagnosed, the severity of the condition and the response to treatment. Early specialist assessment is therefore important.
Pulmonary Hypertension and Heart Failure
Pulmonary hypertension and heart failure are closely related, but they are not the same condition.
Pulmonary hypertension increases pressure in the blood vessels supplying the lungs. This can place extra strain on the right side of the heart.
Over time, the right ventricle may become enlarged or weakened, leading to right-sided heart failure.
Heart failure affecting the left side of the heart can also cause pulmonary hypertension. In these cases, treatment focuses on the underlying heart condition rather than automatically using medicines designed for pulmonary arterial hypertension.
This distinction is important because the correct diagnosis determines the appropriate treatment.
HubPharm Cardiovascular Care: Pulmonary Hypertension
Pulmonary hypertension can require complex, long-term care involving specialist consultations, ongoing monitoring and carefully selected medicines.
At HubPharm Africa, our cardiovascular-care service supports patients managing long-term cardiovascular conditions and prescribed treatments.
Medicines associated with pulmonary hypertension care may include:
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Bosentan
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Ambrisentan
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Macitentan
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Riociguat
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Epoprostenol
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Treprostinil
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Iloprost
These medicines have different roles and are not suitable for every patient. Some are used for selected forms of pulmonary arterial hypertension, while others have specific indications that require specialist assessment.
The medicine names above are provided for educational purposes and do not constitute a recommendation for individual treatment.
Why Specialist Pharmacy Support Matters
Pulmonary hypertension treatment requires careful coordination between the patient, specialist team and pharmacy professionals.
HubPharm’s pharmacy team can help patients understand their prescribed medicines, identify questions about potential interactions and reinforce the importance of attending recommended clinical reviews.
Support may include:
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Explaining the purpose of prescribed medicines.
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Helping patients understand their clinician’s instructions.
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Identifying potential medication-related concerns for professional review.
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Reinforcing the importance of follow-up and monitoring.
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Helping patients recognise when worsening symptoms require medical attention.
Some specialist pulmonary hypertension medicines require close monitoring. They should only be used under the appropriate specialist’s supervision.
Need Support With Your Cardiovascular Medication?
If you or a family member has been diagnosed with pulmonary hypertension, speak with a qualified healthcare professional about the treatment and monitoring plan.
HubPharm’s pharmacy team can provide appropriate guidance about prescribed medicines within the scope of pharmacy practice.
Do not start, stop or change a pulmonary hypertension medicine without advice from your specialist or prescribing healthcare professional.
Frequently Asked Questions About Pulmonary Hypertension
What is pulmonary hypertension?
Pulmonary hypertension is high blood pressure in the arteries that carry blood from the heart to the lungs. It can increase the workload on the right side of the heart.
What are the early symptoms of pulmonary hypertension?
Early symptoms often include breathlessness during activity, tiredness and reduced exercise tolerance. Some people also experience dizziness or chest discomfort. These symptoms can have other causes and require assessment.
Is pulmonary hypertension the same as high blood pressure?
No. Ordinary hypertension affects the body’s systemic arteries, while pulmonary hypertension affects the blood vessels supplying the lungs.
Can pulmonary hypertension be cured?
Some underlying causes may be treatable, and certain patients may benefit from specialist procedures. Many forms require long-term management. The outlook depends on the cause, severity and response to treatment.
Which medicines are used for pulmonary hypertension?
Treatment may include specialist pulmonary vasodilators, diuretics, oxygen therapy or anticoagulants in selected patients. The appropriate approach depends on the specific type of pulmonary hypertension and its underlying cause.
Can pulmonary hypertension cause heart failure?
Yes. It can increase the workload on the right side of the heart and eventually contribute to right-sided heart failure.
Is exercise safe for someone with pulmonary hypertension?
Activity recommendations depend on the patient’s condition. Ask the treating specialist about suitable physical activity and whether supervised rehabilitation is appropriate.
When should someone seek urgent medical help?
Severe or sudden breathlessness, persistent chest pain, collapse or fainting with concerning symptoms requires urgent medical assessment.
The Bottom Line
Pulmonary hypertension is a serious condition that affects the blood vessels supplying the lungs and can place significant strain on the heart.
Recognising symptoms, obtaining the correct diagnosis and receiving appropriate specialist treatment are essential parts of care. Treatment depends on the cause and may include medicines, oxygen therapy, management of underlying conditions or specialist procedures.
Do not ignore persistent breathlessness, unexplained fatigue, chest discomfort or fainting. Seek medical assessment, and obtain emergency help if symptoms are sudden or severe.
Medical Disclaimer
This article is for general health education and does not replace an individual assessment by a doctor, pharmacist or other qualified healthcare professional. Pulmonary hypertension diagnosis and treatment require individual clinical assessment, and specialist medicines should only be used under appropriate professional supervision.
References
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European Society of Cardiology and European Respiratory Society. 2022 Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension.
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American Lung Association. Pulmonary Hypertension: Symptoms and Diagnosis.
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American Lung Association. Treating and Managing Pulmonary Hypertension.
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European Respiratory Society. Definition, Classification and Diagnosis of Pulmonary Hypertension.