Pulmonary Hypertension: Causes, Symptoms, Risks & Life-Saving Treatments Explained

What is Pulmonary Hypertension?

Pulmonary hypertension occurs when the blood pressure in the pulmonary arteries is higher than it should be. [1] These arteries carry blood from your heart to the lungs to get oxygen. When they are too narrow, the heart has to work harder to push blood through them. Over time, this extra work can weaken the heart. [2,3]

Pulmonary hypertension can be caused by other diseases, such as Chronic Obstructive Pulmonary Disease (COPD), heart disease, or conditions like blood clots in the lungs. Oftentimes, the cause of pulmonary hypertension is unknown and is called idiopathic pulmonary hypertension. [4]

Types of Pulmonary Hypertension

Pulmonary hypertension is grouped into five main types based on its underlying cause.

1. Group 1, simply called pulmonary arterial hypertension (PAH), occurs when the blood vessels in the lungs become narrow or stiff, often due to genetic conditions, autoimmune diseases, or unknown causes.
2. Group 2 is linked to left heart disease, like heart valve problems or heart failure, where pressure builds up in the lungs because the heart can’t pump blood efficiently.
3. Group 3 is caused by lung conditions such as COPD or sleep apnea, where the lungs struggle to handle oxygen properly.
4. Group 4 occurs when blood clots block the arteries in your lungs, known as chronic thromboembolic pulmonary hypertension (CTEPH).
5. Group 5 includes cases where the exact cause is unclear but may be linked to rare conditions like blood disorders or metabolic diseases. [5, 6]

Risk factors and Causes of Pulmonary Hypertension

Pulmonary hypertension can develop due to various underlying conditions, including:

1. Family History

If someone in your family has pulmonary hypertension, you might be at a higher risk due to genetic factors. Genes like BMPR2 are linked to an increased risk of  PAH and can run in families. [7]

2. Heart Disease

Conditions that affect the left side of your heart, like heart valve problems or heart failure, can increase pressure in the blood vessels of your lungs over time, leading to pulmonary hypertension. Structural heart defects present from birth can also increase blood flow and pressure in your lungs, causing pulmonary hypertension. These defects include left to right shunt defects or left heart obstructive lesions such as ventricular septal defect (VSD), atrial septal defect (ASD), and persistent ductus arteriosus (PDA). [8, 9]

3. Lung Disease

Chronic lung problems such as COPD, interstitial lung disease, or sleep apnea can damage your lungs and make it harder for blood to flow through, raising the risk. [10]

4. Blood Clots in the Lungs:

A history of blood clots blocking the arteries in your lungs can lead to chronic high blood pressure in the lung’s vessels, especially if left untreated. [11]

5. Autoimmune Disease

Conditions like scleroderma, lupus, or rheumatoid arthritis can cause inflammation and damage to your lungs and blood vessels, increasing the chances of pulmonary hypertension. [12]

6. Liver Disease

Severe liver conditions, like cirrhosis, can affect blood flow in your body and lead to increased pressure in your lung arteries, a condition known as portopulmonary hypertension. [13]

7. Drug and Toxin Exposure

Using illegal drugs like methamphetamines or certain chemotherapy drugs has been linked to the development of pulmonary hypertension. [14]

8. Living at High Altitudes

If you live at a high elevation for an extended time, the lower oxygen levels can strain your lungs and increase the risk of developing pulmonary hypertension. [15, 16, 17]

Symptoms of Pulmonary Hypertension

Pulmonary hypertension can present with no symptoms, particularly in its early stages. This asymptomatic presentation can lead to delayed diagnosis. For instance, individuals with heritable pulmonary arterial hypertension may report no symptoms, and the condition might be suspected incidentally due to abnormal findings on physical examination on a routine doctor’s visit. The nonspecific symptoms of pulmonary hypertension, such as exertional dyspnea and fatigue, contribute to the diagnostic challenge, and some patients may remain asymptomatic until the disease progresses. Some common symptoms of pulmonary hypertension are as follows:

1. Shortness of Breath

You might notice that you get out of breath easily, especially when climbing stairs or walking short distances. Over time, this can happen even while you’re resting. [18]

2. Fatigue

Feeling drained is common because your heart and lungs work harder to pump blood. About 57% of patients with pulmonary hypertension experience significant fatigue, which is the second most debilitating symptom. [19]

3. Chest Pain or Tightness:

You may feel pressure or discomfort in your chest, especially during physical activity. This can be similar to the angina and compression of one of the arteries supplying the heart, which is found to be the cause of such pain. [20, 21]

4. Swelling

Your ankles, legs, or even your belly might swell. This happens because your heart struggles to pump blood effectively, causing fluid buildup. [21]

5. Dizziness or Fainting

You might feel lightheaded or even faint, particularly when you’re active. This occurs because your brain isn’t getting enough blood flow. [22]

6. Heart Palpitations

You may feel like your heart is racing, fluttering, or skipping beats due to the strain on your heart. [21]

7. Dry Cough

This is one of the symptoms caused by the compression of your airway by the dilated pulmonary arteries. This cough is generally persistent and increases with time. [23]

Diagnosis of Pulmonary Hypertension

Key Diagnostic Tests for Pulmonary Hypertension are:

1. Blood Test

Pulmonary hypertension is not linked to any particular abnormalities in your blood. However, the stretch on your heart might increase brain natriuretic peptide (BNP) levels, which can also be elevated in other conditions. [24]

2. Chest X-Ray

A chest X-ray provides a simple way to examine your heart and lungs. In pulmonary hypertension, the pulmonary arteries may appear enlarged, and the right side of the heart may look bigger. It can also help identify other lung conditions, such as interstitial lung disease or scarring, which might contribute to your symptoms. [25]

3. Electrocardiogram (ECG)

An ECG records the electrical activity of your heart. It’s a quick and painless test that can detect signs of strain on the right side of your heart caused by increased pressure in the pulmonary arteries. Your doctor will look for abnormalities, such as changes in heart rhythm or evidence of an enlarged right ventricle. [26]

4. Transthoracic Doppler Echocardiography (TTE)

This test is a key step in diagnosing pulmonary hypertension. It uses sound waves to create moving images of your heart. The echocardiogram helps your doctor estimate the pressure in your pulmonary arteries and assess how well your heart is pumping. It can also reveal structural abnormalities, such as thickened heart walls or valve problems, which can occur in pulmonary hypertension. [26]

5. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI)

Advanced imaging techniques like CT or MRI provide detailed pictures of your heart and lungs. A CT scan can detect blood clots in your lungs or structural abnormalities, while an MRI can provide a more detailed look at the heart’s structure and function. They also help to create a baseline assessment of the disease severity. [26, 27]

6. Ventilation-Perfusion (V/Q) Scan

A VQ scan, or ventilation-perfusion scan, is a test that helps diagnose pulmonary hypertension, especially when caused by CTEPH. It has two parts: inhale a harmless gas to check how air moves through your lungs (ventilation), and a small tracer is injected into your vein to track blood flow (perfusion). Comparing these scans helps identify blocked blood flow areas that may point to clots. [28, 29]

7. Right Heart Catheterization

This is the gold standard for diagnosing pulmonary hypertension. A thin tube (catheter) is inserted into a vein in your neck or groin and guided to your heart and pulmonary arteries. This procedure directly measures the pressure in your pulmonary arteries and assesses how well your heart functions. It also tests how your heart responds to medications or other treatments. [30, 31]

8. Pulmonary Function Tests (PFTs)

This test measures how well the lungs work and helps identify any lung diseases that might contribute to or occur alongside PH. [32]

9. Exercise Testing, Six-Minute Walk Test (6MWT)

This test measures how far a person can walk in six minutes. It’s a simple way to evaluate exercise tolerance and how well the lungs and heart work together. [33]

Treatment of Pulmonary Hypertension

Pulmonary hypertension (PH) is a chronic and potentially life-threatening condition that requires a comprehensive treatment approach. Treatment strategies vary based on the underlying cause and severity of PH, and they typically include medications, lifestyle modifications, and in some cases, surgical interventions.

1. Medications

Medications are the main treatment for pulmonary hypertension. These can include vasodilators, which relax and widen blood vessels, improving blood flow and reducing pressure in the lungs. [34] Drugs like prostacyclins, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors may be used to help lower pulmonary artery pressure. [35] Diuretics are also prescribed to reduce fluid buildup in the body, which can relieve symptoms like swelling and difficulty breathing. Additionally, blood thinners (anticoagulants) may be recommended to prevent blood clots, especially in Chronic thromboembolic pulmonary hypertension (CTEPH) cases. [36]

2. Oxygen Therapy

For patients with low oxygen levels, oxygen therapy can help improve breathing and reduce the strain on the heart and lungs. It involves using a portable oxygen tank or concentrator to provide supplemental oxygen, particularly during physical activity or sleeping. This treatment can prevent complications related to low oxygen, such as fatigue, dizziness, and organ damage. [37]

3. Lifestyle Changes

While medications are essential, lifestyle adjustments can also support treatment. A low-sodium diet helps prevent fluid retention, which can worsen symptoms of PH. Regular but light exercise can improve cardiovascular health and help manage symptoms. Avoiding high altitudes is also important because the lower oxygen levels at high altitudes can exacerbate breathing problems and increase stress on the heart. [38]

4. Surgical Interventions

In severe cases of pulmonary hypertension that do not respond to medications, surgical treatments may be necessary. Atrial septostomy is a procedure that creates a hole between the heart’s chambers to relieve pressure in the lungs, helping improve blood flow. [39, 40] For patients with CTEPH, surgery to remove blood clots or a pulmonary thromboendarterectomy may be an option. In the most severe cases, a lung transplant may be considered when other treatments are no longer effective. [41]

5. Treating Underlying Conditions

Treating the underlying condition is vital if pulmonary hypertension is secondary to another disease. For instance, managing heart diseases like left-sided heart failure or lung conditions such as COPD can significantly impact pulmonary hypertension. Additionally, treating conditions like autoimmune diseases or infections can help control pulmonary hypertension symptoms and prevent further complications. [42]

Complications of Pulmonary Hypertension

Pulmonary hypertension can lead to several serious complications if left untreated. As the pressure in the lungs’ blood vessels increases, the heart has to work harder to pump blood through the lungs, which can cause the right side of the heart to enlarge and weaken, a condition known as right heart failure. [7] Additionally, pulmonary hypertension can increase the risk of blood clots, leading to a higher chance of stroke or other clot-related issues. [4] In severe cases, it can cause organ damage due to reduced oxygen levels in the body. Left untreated, pulmonary hypertension can significantly impact the quality of life and shorten life expectancy.

Pregnancy and Pulmonary Hypertension

Pregnancy and pulmonary hypertension can be a risky combination because of the strain pregnancy places on your heart and lungs. Pulmonary hypertension causes high blood pressure in the blood vessels of your lungs, which forces your heart to work much harder to pump blood. During pregnancy, the body’s blood volume increases significantly, further stressing your heart and lungs. This can lead to serious complications such as heart failure, arrhythmias, or low oxygen levels, both for you and your baby. Pregnancy in women with pulmonary hypertension is associated with high maternal and fetal risks, and careful management by a multidisciplinary team is essential to mitigate these risks.  [43]

Questions to Ask Your Doctor

1. How can I manage stress or anxiety related to having Pulmonary Hypertension?
2. How often do I need to have follow-up appointments?
3. What should I do if I experience a sudden change in symptoms or feel unwell?
4. Are there lifestyle changes I should make to help manage my symptoms?
5. Are there any support groups or resources for people with pulmonary hypertension?

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