Functional Classes | Types and Prognosis | Outcomes With Different Treatments | Get Support

Pulmonary hypertension (PH) is caused by high blood pressure in the arteries (known as pulmonary artery pressure) that are responsible for carrying blood from the right side of the heart to the lungs.

The life expectancy of an individual with PH depends on several factors, including the class of PH, the type of PH, and any underlying conditions. The disease prognosis has improved greatly over the years thanks to better medications and treatments.

For example, the life expectancy of someone with pulmonary arterial hypertension (PAH), one type of PH, used to be 2.5 years. Now it has been extended to more than a decade.

Functional Classes of Pulmonary Hypertension

Currently, there is no one cure for PH. This disease is caused by many factors and can worsen over time at different rates for different people. Left untreated, PH can cause permanent damage to the heart and lungs and can become life-threatening.

The World Health Organization (WHO) divides cases of PH into four functional classes depending on how much the disease limits activities. The classes are as follows:

• Class 1 PH — Diagnosed when there are no PH symptoms at either rest or with exercise
• Class 2 PH — Diagnosed when there are no PH symptoms at rest, but there is shortness of breath during everyday activities such as shopping for groceries or climbing stairs
• Class 3 PH — Diagnosed when there may not be PH symptoms at rest, but household chores and everyday activities are greatly limited due to fatigue or shortness of breath
• Class 4 PH — Diagnosed when there are PH symptoms both at rest and during everyday activities; swelling of the ankles and feet — known as edema — is common, and fainting (or feeling like you are going to faint) is also possible

Classes of Pulmonary Hypertension and Survival

Survival times can vary depending on numerous factors, including sex, age, overall health, any underlying conditions, and more. Your doctor will discuss your outlook and treatment options with you once you receive your diagnosis.

Types of Pulmonary Hypertension and Prognosis

The type of PH a person has can affect disease outcomes. Some types are more severe than others and can affect survival. The WHO divides cases of PH into five groups depending on the cause of the disease.

Group 1: Pulmonary Arterial Hypertension

Group 1 PH, also known as pulmonary arterial hypertension, can be caused by many factors. In some cases, there is no known cause of the disease (idiopathic PAH). In other cases, it can be caused by genetics (familial PAH), drugs and toxins (anorexigenic-associated PAH), or other underlying diseases such as:

• Autoimmune conditions, such as scleroderma
• Liver disease
• Congenital heart disease
• HIV infection

Underlying conditions that cause PH can also affect disease outcomes. For example, according to an article in the journal Annals of Thoracic Medicine, individuals with both PH and scleroderma have a 40 percent chance of living two years after diagnosis.

Group 2: PH Due to Left Heart Disease

Group 2 PH is caused by heart disease on the left side of the heart. This is also known as pulmonary venous hypertension and is the most common type of PH. Heart diseases that cause this type of PH include:

• Coronary artery disease
• Long-term high blood pressure throughout the body (chronic hypertension)
• Heart defects that formed before birth
• Mitral valve problems

Women have a better five-year survival rate than men, at 61.8 percent compared to 47.1 percent. Another study found that people with congenital heart disease have better outcomes than those without.

Group 3: PH Due to Lung Disease and Hypoxia

Group 3 PH is caused by a lack of oxygen over extended periods of time, known as hypoxia. This can be caused by several conditions, including sleep apnea, interstitial lung disease, and chronic obstructive pulmonary disease (COPD). People who live at higher altitudes with lower oxygen levels can also develop group 3 PH.

People with COPD and PH have been found to have better survival rates than those with interstitial lung diseases and PH. The five-year survival rates for group 3 PH are 44 percent for women and 34.3 percent for men. It has also been found that survival rates are better for those with less severe heart failure symptoms using the New York Heart Association scale.

Group 4: PH Due to Chronic Thromboembolic Disease

Group 4 PH is caused by blood clots that become stuck in the lung’s arteries. This type is also known as chronic thromboembolic pulmonary hypertension (CTEPH). Those with CTEPH who undergo surgery to remove the blood clot and affected tissues have a three-year survival rate of 89 percent.

Group 5: PH Due to Miscellaneous Causes

Group 5 PH is caused by multiple factors or other underlying conditions, and it is not well understood. These are mainly diseases that disrupt the blood flow between the heart and lungs, leading to increased blood pressure. Conditions can include:

• Chronic kidney failure
• Cancer
• Thyroid disease
• Chronic hemolytic anemia

Group 5 PH is rare, and few studies have looked at survival rates and disease outcomes due to the many underlying causes.

Outcomes With Different Pulmonary Hypertension Treatments

Although there is no cure for PH, it can be managed with medication. Some people will respond better to treatments than others, depending on the type of PH and the underlying conditions. Some PH treatments come with side effects.

Prostaglandins

Prostaglandins mimic hormones in the body that are responsible for dilating blood vessels (known as a vasodilator). They can also help prevent blood vessel scarring.

However, some people with PH will respond to prostaglandins and other vasodilators better than others. They can be broken up into responders and nonresponders. Most people with idiopathic PAH are nonresponders.

Calcium Channel Blockers

Calcium channel blockers are another type of vasodilator used to treat PAH. Although they are effective in responders, they are often not used as a long-term treatment.

Endothelin Receptor Antagonists

Endothelin receptor antagonists reduce the amount of endothelin in the blood, which stops blood vessels from constricting. These drugs have the potential to slow the progression of PH and can even reverse some of the damage caused.

These drugs have been found to improve survival in those with idiopathic PAH, with one- and two-year survival rates around 87 percent to 90 percent.

Phosphodiesterase-5 Inhibitors

Phosphodiesterase-5 (PDE-5) inhibitors work to inhibit the PDE-5 enzyme, which regulates blood flow and dilates arteries in the lungs. PDE-5 inhibitors have been found to improve disease outcomes and survival in people with PAH.

Lung Transplantation

People who do not respond well to vasodilator therapy can be recommended for a lung transplant. People with PAH who received a double lung transplant had a better survival rate than people who received just a single lung transplant. Overall, lung transplants have given people longer survival times and a better quality of life.

Talk With Others Who Understand

On myPHteam, the social network for those who live with PH, more than 46,000 members come together to ask questions, give advice, and share their experiences of life with pulmonary hypertension.

Are you or a loved one living with pulmonary hypertension? Share your experience in the comments below, or start a conversation by posting on your Activities page.