The term “hypertension” refers to high blood pressure. Pulmonary hypertension (PH) is a particular type of high blood pressure affecting the arteries that carry blood from the right side of the heart to the lungs. PH may be confused with systemic hypertension — high blood pressure in the blood vessels throughout the body. However, the two are different conditions with their own causes and treatments.
Pulmonary hypertension specifically refers to high blood pressure of the pulmonary arteries, the arteries carrying blood between the right side of the heart and the lungs. Systemic hypertension, on the other hand, causes high blood pressure in the vessels carrying blood to all tissues in the body — except those in the lungs. In order to understand PH and hypertension, it may be helpful to understand how blood pressure works.
Your blood pressure measures how much blood your heart pumps, as well as how much resistance your blood flow faces while traveling through your body’s arteries. It consists of two numbers: your systolic blood pressure and diastolic blood pressure. These numbers are recorded in millimeters of mercury (mmHg).
According to the Mayo Clinic, a normal blood pressure has a systolic reading below 120 mmHg and a diastolic reading below 80 mmHg. Hypertension, or high blood pressure, occurs when a person’s systemic blood pressure is higher than 129 mmHg systolic and 79 mmHg 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 8 mmHG and 20 mmHg when at rest. Pulmonary hypertension occurs when this pressure is higher than 25 mmHg when at rest or higher than 30 mmHg during exercise.
Systemic hypertension is classified as either primary or secondary, depending on whether it has an underlying cause or not. However, the World Health Organization has classified pulmonary hypertension into five types, according to the condition’s causes.
Pulmonary arterial hypertension (PAH) is a type of PH that occurs when the lung’s blood vessels have been damaged or have become narrow or blocked. Group 1 PAH 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 caused by other preexisting health conditions.
Group 2 PH is typically caused by conditions that affect the left side of the heart (left-sided heart disease), such as heart valve disease. Group 2 PH occurs when chronic (long-term) high blood pressure or heart disease damage the pulmonary arteries. This type is the most common cause of PH.
Group 3 PH refers to pulmonary hypertension caused by preexisting lung diseases — including interstitial lung disease or chronic obstructive pulmonary disease — or hypoxemia (low blood oxygen levels). Hypoxemia may result from several health conditions, including emphysema (a lung condition that causes shortness of breath) and sleep-related breathing problems such as sleep apnea.
Group 4 PH, also known as chronic thromboembolic pulmonary hypertension, occurs when blood clots in the lungs lead to blockages or narrowed arteries.
Group 5 PH refers to PH caused by other different disorders, including:
Many people with systemic hypertension do not experience any signs or symptoms of the disease — even as hypertension progresses and their blood pressure rises to dangerous levels. Symptoms a person may experience include shortness of breath, headaches, or nosebleeds. These symptoms typically occur only when hypertension has progressed far enough to become severe or life-threatening.
People with less advanced pulmonary hypertension also may not experience symptoms. Those who do most commonly experience shortness of breath, reported by 86 percent of people with pulmonary arterial hypertension. Other early symptoms of PH include:
As damage progresses, PH symptoms can become debilitating and impact day-to-day life. A person with later-stage PH symptoms may become faint or lightheaded — particularly when engaging in physical activity. They may even lose consciousness. They may also develop swollen legs or ankles and have discoloration in their skin or lips. This is called cyanosis, which results from low blood oxygen. In people with lighter skin, the skin takes on a dark bluish tint. In people with darker skin, cyanosis may present as gray or whitish skin and blue around the lips, gums, eyes, and nails.
Both pulmonary hypertension and systemic hypertension may be idiopathic, meaning they have no identifiable cause. In some cases, however, underlying health conditions or medications may be the cause.
Pulmonary hypertension occurs when the blood vessels running between the lungs and the right side of the heart are damaged, stiff, swollen, or narrow. As a result, the lower right chamber of the heart (the right ventricle) must exert more effort to pump blood to the lungs. PH can be caused by anything that raises the blood pressure in the lungs and pulmonary arteries. PAH, in particular, is caused by the narrowing of the pulmonary arteries.
Although pulmonary hypertension is often idiopathic, it can develop as the result of:
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:
Certain factors may increase a person’s risk of developing systemic hypertension, including using tobacco products, being overweight, 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.
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.
If your doctor suspects you have PH, they will start by asking about your signs and symptoms and by taking a health history. If they discover you have a family member with PH, for example, that may indicate that you are at higher risk of developing the condition as well.
Several diagnostic tests and procedures can be used to confirm a diagnosis of PH, determine its severity, and identify its underlying causes, including:
To determine the cause of your pulmonary hypertension, the doctor may request:
Your doctor may also have you undergo a sleep study — known as polysomnography — if they believe a sleep-related issue is responsible for your PH.
As with PH, diagnosing high blood pressure typically begins with an assessment of your signs, symptoms, and medical history. Your doctor will typically 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:
There is currently no cure for pulmonary hypertension or hypertension, 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 your symptoms.
Health care providers may recommend making some changes to your lifestyle as a first step to help lower your blood pressure. The following may help keep your blood pressure in check with PH and hypertension:
Generally, lifestyle changes are not enough to manage 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, including:
The treatment your doctor selects will often depend on the cause of your specific condition.
Living with pulmonary hypertension has its challenges. It can help to know that you’re not alone. By joining myPHteam, the social network for people with PH and their loved ones, you can connect with a growing team of nearly 35,000 members from across the globe who understand life with pulmonary hypertension.
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