Not all types of high blood pressure are the same — some affect the whole body, while others target certain areas.

Pulmonary hypertension (PH) is a kind of high blood pressure that impacts the arteries which carry blood from the right side of the heart to the lungs. PH may be confused with systemic hypertension — high blood pressure that affects blood vessels throughout the entire body.

However, it’s important to note that the two are different conditions with their own causes and treatments.

What Are PH and Hypertension?

Pulmonary hypertension specifically refers to high blood pressure of the pulmonary arteries — the arteries that carry blood between the right side of the heart and the lungs.

Systemic hypertension, on the other hand, causes high blood pressure in the vessels that carry blood to all tissues in the body.

To understand PH and hypertension, it may be helpful to learn about how blood pressure works.

Your blood pressure measures how much blood your heart pumps, as well as how much resistance your blood faces while traveling through your body’s arteries. It’s made up of two numbers — your systolic blood pressure and diastolic blood pressure. These numbers are recorded in millimeters of mercury (mm Hg).

• Systolic blood pressure reflects how much force your heart puts on your arteries’ walls each time it beats and pumps blood. It’s the first, or top, number in your blood pressure measurement.
• Diastolic blood pressure reflects the pressure on your arteries’ walls in the space between beats when the heart relaxes. It’s the second, or bottom, number in your blood pressure measurement.

A normal blood pressure has a systolic reading below 120 mm Hg and a diastolic reading below 80 mm Hg. Hypertension occurs when a person’s systemic blood pressure is higher than 129 mm Hg systolic and 79 mm Hg diastolic.

Pulmonary artery pressure, however, is typically much lower than a person’s systemic blood pressure. Normal blood pressure in the pulmonary arteries falls between 11 mm Hg and 20 mm Hg when at rest. Doctors now say pulmonary hypertension happens when the average pressure in the lung arteries is more than 20 mm Hg.

Types of Pulmonary Hypertension and Hypertension

Systemic hypertension is classified as either primary or secondary, depending on whether it has an underlying cause or not. In contrast, the World Health Organization (WHO) has classified pulmonary hypertension into five types, according to the condition’s causes.

Group 1 Pulmonary Hypertension

Pulmonary arterial hypertension (PAH) is a type of PH that occurs when the lungs’ blood vessels have been damaged or have become narrow or blocked.

Group 1 PH refers to cases of PH with no known cause — referred to as idiopathic — as well as those that are inherited, caused by certain medications, or associated with connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, and schistosomiasis.

Group 2 Pulmonary Hypertension

Group 2 PH is typically caused by left-sided heart disease (conditions that affect the left side of the heart), such as heart valve disease or heart muscle weakness.

Group 2 PH occurs due to chronic (long-term) inability of the heart to pump the blood efficiently. This leaves a lot of blood behind in pulmonary circulation, damaging the pulmonary blood vessels. This type of PH is the most common.

Group 3 Pulmonary Hypertension

Group 3 PH refers to pulmonary hypertension caused by hypoxemia (low blood oxygen levels) due to preexisting lung diseases.

Hypoxemia may result from several health conditions, including:

• Chronic obstructive pulmonary disease (COPD)
• Emphysema (a lung condition that causes shortness of breath because of damaged lung tissue)
• Obstructive sleep apnea
• Abnormal lung development

Group 4 Pulmonary Hypertension

Group 4 PH — also known as chronic thromboembolic pulmonary hypertension (CTEPH) — occurs when blood clots in the lungs lead to blockages or narrowed arteries. CTEPH is a rare type of pulmonary hypertension.

People with a history of blood clots in the lungs, especially those with increased pressure in pulmonary vessels for six months or longer after their clot diagnosis, have an increased risk of CTEPH.

Group 5 Pulmonary Hypertension

Group 5 PH refers to PH caused by other disorders, including:

• Kidney disease
• Blood disorders
• Metabolic disorders — Conditions including hypothyroidism (underactive thyroid), hyperthyroidism (overactive thyroid), Gaucher disease, and glycogen storage disease
• Tumors that put pressure on the pulmonary arteries

Symptoms of Pulmonary Hypertension vs. Hypertension

Many people with systemic hypertension do not experience any signs or symptoms of the disease. This can be true even as hypertension progresses and their blood pressure rises to dangerous levels.

People with less advanced pulmonary hypertension also may not experience symptoms. Some symptoms of high blood pressure can be caused by both systemic and pulmonary hypertension while others are specific to one condition.

Symptoms Shared by Pulmonary Hypertension and Systemic Hypertension

1. Shortness of Breath

Shortness of breath is common for people with PH — around 86 percent of people with PAH (group 1 PH) reported this symptom. However, shortness of breath may also occur in some people with severe systemic hypertension.

2. Chest Pain

Another common symptom of PH, chest pain was reported in 22 percent of people with PAH (group 1 PH). Systemic hypertension can also result in sudden chest or back pain in people with dangerously high blood pressure.

Symptom Differences in Pulmonary Hypertension vs. Hypertension

1. Headaches (Systemic Hypertension)

People with severe systemic hypertension may have a headache, if they have symptoms at all. Severe headaches that come on suddenly, especially with very high blood pressure, are considered medical emergencies — call 911 or emergency services immediately.

2. Nosebleeds (Systemic Hypertension)

Nosebleeds may also occur in some people with systemic hypertension. Nosebleeds and high blood pressure are related, specifically in the case of systemic hypertension.

In fact, severe nosebleeds are more commonly associated with hypertension than any other disease.

3. Dizziness or Fainting (PH)

A person with later-stage PH symptoms may become dizzy or faint — particularly when doing physical activity. Around 15 percent of people with PAH (group 1 PH) report feeling light-headed or fainting.

4. Fatigue (PH)

Fatigue (severe tiredness that does not improve with rest) is another common symptom of pulmonary hypertension. Among people with PAH (group 1 PH), 27 percent report fatigue.

5. Swelling (PH)

Swelling — also called edema — of the ankles and feet can occur in people with pulmonary hypertension. This symptom was reported by 21 percent of people with PAH (group 1 PH).

6. Heart Palpitations (PH)

Heart palpitations — the sensation of an irregular heartbeat — was reported in 13 percent of people with PAH (group 1 PH). Palpitations can be felt in the neck or chest and may feel like your heart is racing, pounding, or skipping beats.

7. Changes in Skin Color (PH)

People with pulmonary hypertension may have skin discoloration called cyanosis, caused by low oxygen levels in the blood.

Cyanosis can look different depending on a person’s natural skin tone. It may appear blue or gray on lighter skin, and ashen or gray on darker skin. In individuals with darker skin, these changes may be more noticeable on the lips, gums, or palms.

What Causes PH and Hypertension?

Both pulmonary hypertension and systemic hypertension may have no identifiable cause. In some cases, however, underlying health conditions or medications may be the cause.

Causes of Pulmonary Hypertension

Pulmonary hypertension occurs when the blood vessels running between the lungs and the right side of the heart are damaged, stiffen, swell, or become narrowed. As a result, the lower right chamber of the heart (the right ventricle) must work harder to pump blood to the lungs.

PH can be caused by anything that raises the blood pressure in the pulmonary arteries of the lungs. PAH, in particular, is caused by the narrowing of the pulmonary artery — the vessel that delivers blood from the heart to the lungs.

Although pulmonary hypertension is often idiopathic, it can develop as the result of:

• Genetic variants
• Congenital defects (issues present at birth)
• Heart disease
• Lung disease
• Connective tissue diseases, such as lupus and scleroderma
• Blood cell disorders or blood clots
• Certain drugs, including methamphetamines, cocaine, and fenfluramine-phentermine (“fen phen”— now off the market) and other appetite suppressants

Causes of Systemic Hypertension

As with PH, many cases of hypertension have no identified cause. This is referred to as primary hypertension or essential hypertension. Primary hypertension typically develops slowly over the course of many years.

Secondary hypertension, on the other hand, refers to high blood pressure that can be attributed to an underlying health condition or problem. Several factors can cause secondary hypertension, including:

• Renal (kidney) disease, including narrowed kidney arteries
• Metabolic disease
• Obstructive sleep apnea
• Congenital defects in the blood vessels
• Primary aldosteronism (overproduction of the hormone aldosterone) and other hormonal disorders
• Certain medications, including over-the-counter pain medications and oral contraceptives
• Illegal substances, like amphetamines and cocaine
• Some types of tumors

Certain factors may increase a person’s risk of developing systemic hypertension, including using tobacco products, having a higher body weight, and having a family history of high blood pressure.

A person’s diet can also affect their blood pressure — too much sodium, too little potassium, and drinking alcohol in excess can increase the likelihood of hypertension.

As with pulmonary hypertension, the risk of systemic hypertension also increases with age.

Diagnosing PH and Hypertension

Pulmonary hypertension can be difficult to diagnose. Early symptoms like dizziness and tiredness typically come on slowly and go undetected, or they may be mistaken for symptoms of another condition. Consequently, people with pulmonary hypertension are often initially misdiagnosed.

Diagnosing Pulmonary Hypertension

If your doctor suspects you have PH, they will start by asking about your signs and symptoms and taking a health history. If they discover you have a family member with PH, for example, that may indicate that you’re at higher risk of developing the condition as well.

Several tests and procedures can be used to confirm a diagnosis of PH, determine its severity, and identify its underlying causes. Testing may include:

• Chest X-rays
• Echocardiography
• Electrocardiogram
• Right-heart catheterization

To determine the cause of your pulmonary hypertension, your doctor may request:

• Lung function tests
• Blood tests
• A CT or MRI scan of the chest

Your healthcare provider may also have you undergo a sleep study — known as polysomnography — if they believe a sleep-related issue is responsible for your PH.

Diagnosing Systemic Hypertension

As with PH, diagnosing high blood pressure typically begins with an assessment of your signs, symptoms, and medical history. Your doctor will measure your blood pressure using a gauge and an inflatable cuff placed around the arm. They may also determine the underlying cause of high blood pressure using one or more tests, including:

• A 24-hour blood pressure measurement test (ambulatory monitoring)
• Urine or blood tests
• Electrocardiogram
• Echocardiogram

Treating PH and Hypertension

Pulmonary hypertension and hypertension don’t have cures, but both conditions can be managed and potentially improved. Work with your doctor to assess your treatment options and determine the best way of managing your high blood pressure and symptoms.

Lifestyle Changes

Healthcare providers may recommend making some changes to your lifestyle as a first step to help lower your blood pressure. Strategies that may help keep your blood pressure in check with PH and hypertension, include:

• Maintaining a healthy weight through changes to diet or physical activity
• Eating a heart-healthy diet with plenty of fresh fruits, vegetables, and whole grains
• Limiting foods high in cholesterol, fats, sugar, and sodium
• Quitting smoking, which can help reduce your risk of atherosclerosis (when plaque builds up in the arteries, causing them to narrow)

Medication

Lifestyle changes are not always enough to manage pulmonary hypertension or hypertension. Your doctor may recommend taking medications to help lower or manage your blood pressure. A variety of medications may be used to treat high blood pressure.

Medications used to treat pulmonary hypertension include:

• Activin signaling inhibitors
• Digoxin
• Diuretics
• Endothelin receptor antagonists
• Oral prostacyclin receptor agonists
• Phosphodiesterase 5 (PDE5) inhibitors
• Prostacyclins or vasodilators
• Soluble guanylate cyclase (sGC) stimulators

Medications used to treat systemic hypertension include:

• ACE inhibitors
• Angiotensin-receptor blockers
• Beta-blockers
• Calcium channel blockers
• Diuretics
• Mineralocorticoid receptor antagonists

The treatment your doctor selects will often depend on the cause of your specific condition.

Find Your Team

On myPHteam, the social network for people with PH and their loved ones, more than 56,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Have you been diagnosed with pulmonary hypertension or systemic high blood pressure? Do you have any advice for others managing these conditions? Share your experience in the comments below, start a conversation by posting on your Activities page, or connect with like-minded members in Groups.