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Pulmonary Hypertension

Reviewed by: HU Medical Review Board | Last reviewed: February 2024

Pulmonary hypertension (PH) is a heart condition that affects the blood vessels in the lungs. Having PH means you have high blood pressure in your pulmonary arteries. The pulmonary arteries are responsible for carrying blood from the heart to the lungs. In the lungs, the blood receives oxygen before being pumped back into the body.1,2

In people with PH, the pulmonary arteries narrow and thicken, making it harder for blood to flow through them. Symptoms of PH can include shortness of breath, lightheadedness, and chest pain. If left untreated, PH can cause heart failure.1-3

How common is pulmonary hypertension?

PH affects millions of people worldwide. The actual number is unclear, but experts believe that PH may affect about 1 in 100 people.2

The rate of PH in older adults is even higher. About 1 in 10 people over age 65 have PH.2

Who is at risk for pulmonary hypertension?

Pulmonary hypertension can affect people of all ages, but it is more common in adults. Women are diagnosed with this condition more often than men.1-3

Certain risk factors may increase the chances of developing PH. For example, a family history of PH and other heart or lung conditions can increase your risk. The following medical conditions also increase the risk of developing PH:2,3

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  • Mitral valve disease
  • Aortic valve disease
  • Scleroderma
  • Sickle cell disease
  • Chronic obstructive pulmonary disease (COPD)
  • HIV

Types of pulmonary hypertension

Not all cases of pulmonary hypertension are the same. PH can develop because of a pre-existing condition, or it can occur on its own. PH is broken down into 5 types:1,4

  • Pulmonary arterial hypertension (PAH)
  • PH due to left-sided heart disease
  • PH due to lung disease and/or low oxygen levels (hypoxia)
  • PH due to blood clots in the lungs
  • PH with no known causes and/or multiple causes

Pulmonary arterial hypertension (PAH)

In PAH, blood pressure in the pulmonary arteries is elevated. This makes it harder for blood to flow from the heart to the lungs. The heart must work harder to pump blood, leading to potential complications. PAH can be caused by various underlying causes. Symptoms include shortness of breath, fatigue, and chest pain.4

PH due to left-sided heart disease

In this condition, the left side of the heart has a tough time squeezing and relaxing. Or the heart valves on the left side may not be working properly. In simple terms, the left side of the heart has a hard time keeping up with the blood returning from the lungs. This elevates the pressure in the lungs.4

PH due to lung disease and/or low oxygen levels

When the lungs do not function properly, the blood vessels in the lungs may narrow. This makes it harder for blood to flow through and increases pressure in the blood vessels. Low oxygen levels in the blood can also contribute to this condition.4

This type of PH is closely linked to conditions like COPD and those that cause low oxygen levels.4

PH due to blood clots in the lungs

In this type of PH, high blood pressure in the lungs is caused by clots blocking the blood vessels. These clots are known as pulmonary embolisms. They restrict blood flow, making it difficult for the heart to pump blood through the lungs. This can lead to increased pressure in the pulmonary arteries, putting a strain on the heart.4

PH with no known causes and/or multiple causes

This simply means that experts do not know the exact cause of PH. It could be triggered by a pre-existing condition or something else.4

Your treatment plan will depend on the subtype of PH you have and how severe it is.1,2,4

Stages of pulmonary hypertension

At the beginning stage of PH, you may not notice any symptoms, or symptoms may be mild. But as PH progresses, symptoms get worse.2,3

The stages of PH, also referred to as “functional classes,” depend on a person’s symptoms and the severity of those symptoms.2

  • Stage 1 – There are no noticeable symptoms.
  • Stage 2 – Symptoms occur when doing everyday tasks such as household chores and climbing stairs. There are no symptoms when resting.
  • Stage 3 – Symptoms occur more often and make everyday tasks much harder.
  • Stage 4 – Symptoms occur even when you are resting.

What is the prognosis of pulmonary hypertension?

The prognosis of PH depends on many factors, including:1-3

  • How quickly you are diagnosed
  • The severity of your condition
  • Presence of underlying conditions
  • How you respond to treatment

Because symptoms come on slowly over time, diagnosis of PH is often delayed. The sooner a person gets treated for PH, the better their outcomes. Thanks to advancements in medical treatments like medicines and surgeries, people with PH can lead fulfilling lives.1-3