Pulmonary hypertension life expectancy and prognosis

Pulmonary hypertension life expectancy varies broadly, depending on factors such as disease type and treatment access. Still, the outlook has greatly improved with modern treatments, allowing many people with the rare disease to live full and active lives.

Abnormally high pressure in the blood vessels that carry blood from the heart to the lungs, called the pulmonary arteries, is the hallmark of pulmonary hypertension, or PH. This high pressure makes the heart work harder to pump blood through the lungs, compromising oxygen delivery to the body. The heart is also strained, and right heart failure can develop.

Because PH is a progressive disease, its symptoms generally get worse over time and can substantially affect daily life. While there is no cure, a combination of PH therapies, supportive care, and lifestyle changes can help control symptoms and improve quality of life.  

Factors that affect pulmonary hypertension life expectancy

It’s difficult to give a universal estimate on PH prognosis, because there are many factors that can affect PH survival rate, including:

• disease type
• treatment approach and access
• time to diagnosis
• disease severity
• presence of other coexisting conditions
• demographic factors, such as age and sex

For any given patient, a combination of these factors can influence disease outcomes.

Type of PH

The World Health Organization (WHO) classifies PH into five types depending on the underlying causes. Each disease type has different symptoms and treatment options, which can influence clinical outcomes.

• Group 1: pulmonary arterial hypertension (PAH). A severe disease type caused by narrowing of the pulmonary arteries with a historically poor prognosis, but there are now several targeted therapies available to slow disease progression.
• Group 2: PH due to left heart disease. The prognosis varies, depending on the severity of the underlying heart disease.
• Group 3: PH caused by lung disease or chronic oxygen insufficiency. The prognosis is influenced by the severity of the underlying lung disease, but this PH group generally has some of the poorest outcomes.
• Group 4: chronic thromboembolic pulmonary hypertension (CTEPH). Outcomes vary in this form of the disease caused by blood clots in the lungs, but some people are able to receive potentially curative surgery.
• Group 5: PH associated with other health conditions. Prognosis is highly variable, depending on the underlying disease.

Even within the same WHO group, outcomes can vary. For example, PAH due to underlying connective tissue disease generally has worse outcomes than idiopathic PAH, which has no known cause. The idiopathic PAH prognosis may also be more favorable than that of heritable PAH, which is associated with genetic mutations.

Access to treatment

Prompt treatment can substantially alter the natural course of the disease. In general, PH life expectancy with treatment is notably longer than without, particularly for PAH, which has the most available treatment options.

The timing of when a person starts treatment, as well as the specific type of treatment, can also affect outcomes. It is generally recognized that the earlier initiation of therapies to slow disease progression leads to better outcomes. However, diagnostic delays can hinder the initiation of earlier treatment and lead to worse outcomes.

Moreover, not everyone has equal access to the necessary care. High therapy costs and limited access to medications in certain geographical areas can restrict treatment options. People of lower socioeconomic status and racial minority groups are disproportionately affected by this disparity.

Other clinical factors

Other clinical factors, including disease severity and progression, as well as the presence of other health conditions, can also affect life expectancy.

The WHO defined four functional classes, or stages, of PH based on a classification system developed by the New York Heart Association (NYHA) to categorize the severity of heart failure. Class 1 disease has the least impact on daily function, while class 4 is the most severe. Life expectancy typically decreases as the severity of the condition increases.

Coexisting medical conditions, particularly those involving the heart, lungs, or connective tissue, can also decrease life expectancy, as can clinically noticeable progression of symptoms.

Higher pulmonary artery pressure and other indicators of impaired blood flow can also negatively affect prognosis.

Demographic factors

In general, older people and men tend to have a poorer PH outlook.

Historically, PH life expectancy for children was poor, with lower survival rates than adult patients, but with modern advances in treatment and care, outcomes have improved.

Pulmonary hypertension life expectancy statistics

Overall, life expectancy is substantially reduced with PH relative to the general population. About 85%-90% of people with PH survive for at least a year after their diagnosis, and two-thirds to three-quarters survive for at least three years. However, statistics vary widely, and data are not uniformly available across disease types and severities. Survival rates may also vary by geographical region due to differences in treatment availability and other factors.

• Group 1: Data generally show that PAH survival has substantially improved since the earliest registry studies in the 1980s. After targeted therapies became available, the median life expectancy for PAH patients in the U.S. increased from about 2.8 years to about 7 years.
• Group 2: Data indicate that approximately two-thirds of Group 2 PH patients remain alive after five years.
• Group 3: Reported survival rates for Group 3 PH are generally lower than for other PH types. For example, one study found that 37% of patients were still alive after five years, although this rate can vary depending on the specific underlying lung disease.
• Group 4: In CTEPH, outcomes vary depending on whether patients can undergo surgery to remove disease-causing blood clots successfully. One international study found that without surgery, the three-year survival rate was 71%, while with surgery, it was 92%-94%.
• Group 5: Survival data for Group 5 PH is highly variable. One study showed five-year survival rates of around 82%.

For people with PAH who have numerous treatment options, a combination of multiple treatments as a first-line therapy approach may substantially decrease the death rate compared with a single therapy approach.

Few studies have directly compared life expectancy for people with different types of PH in a single report. A German study that examined this found that the overall five-year survival rate was 53.6%, ranging from 22.5% in patients with PH and interstitial lung disease (Group 3) to 66.7% in those with CTEPH (Group 4).

Influence of disease severity

While PH type gives some information about life expectancy, outcomes can still vary substantially within the same group, with disease severity having a substantial impact. In general, people with more severe disease have worse outcomes than people with milder PH.

• Scientists have begun to utilize risk scoring systems to predict mortality in PH, particularly in PAH. These systems consider multiple factors, including functional assessments, blood biomarkers, and heart imaging. Data suggest that around 90% or more of PAH patients who achieve a low-risk status survive for five years, compared with 50% or fewer of individuals at an intermediate or high risk.
• Another study showed that stage 3 PH mortality is 1.7 to 3.5 times higher than stage 2, depending on the disease type. Likewise, stage 4 PH increased the risk of death by nearly eight times relative to NYHA functional class 2.
• In a U.K. study, people with milder elevations in pulmonary artery pressure had significantly better outcomes than people with higher pressure. Mild PH survival at three years was 85.1%, while more severe PH survival at three years was 68%.

Life expectancy decreases further once the disease reaches an advanced stage and causes right heart failure. PH with right heart failure prognosis was poor in one U.S. study, which demonstrated a 16% mortality rate within 90 days of hospitalization.

Outlook and quality of life

While there is no cure for PH, prompt and appropriate treatment, along with a multidisciplinary supportive care plan, can improve the overall PH outlook and boost quality of life.

Lifestyle changes — such as adherence to a healthy diet, regular exercise, and quitting smoking — can help people with PH live as healthy and active a life as possible.

Managing a lifelong condition like PH can take an emotional toll. Mental health support, such as talk therapy or support groups, is also a key aspect of living well with PH.

End-stage PH life expectancy is typically poor, but supportive and palliative care may help ease pain and increase comfort at the end of life. In some cases, a lung transplant or combined lung-and-heart transplant may be an option to increase the likelihood of survival.