Life Expectancy And Prognosis With Pulmonary Hypertension

You may already be familiar with hypertension, which refers to having high blood pressure. It’s associated with an increased risk of having a heart attack or stroke. However, being diagnosed with pulmonary hypertension (PH) is a separate, rare lung disorder. Pulmonary hypertension still involves high blood pressure, but “pulmonary” pertains to the lungs.

The American Lung Association estimates that 500–1,000 new cases of PH are diagnosed in the US each year¹. Most cases of pulmonary hypertension are incurable. However, proper diagnosis and treatment help make the symptoms manageable and prolong life expectancy.  Drug therapies are the primary treatment. This article provides a general outlook and broad statistics regarding how long one can live with pulmonary hypertension.

What is pulmonary hypertension?

Your blood pressure is the force of your blood pushing against your blood vessel walls. When those levels are consistently too high, it's referred to as high blood pressure (HBP). In contrast, pulmonary hypertension is HBP in the arteries connecting the right side of your heart to your lungs (pulmonary vessels). This form of hypertension narrows the arteries, thereby reducing blood flow to your lungs and lowering the amount of oxygen in your bloodstream, impacting the function of all cells.

Life expectancy with pulmonary hypertension

When the heart is working harder than usual to push the blood through the arteries, it weakens over time, making pulmonary hypertension a fatal condition, especially if undiagnosed.

In 2021, new clinical trial data² was released by The Janssen Pharmaceutical Companies of Johnson & Johnson demonstrating the benefits of using a drug called selexipag to delay the progression of pulmonary hypertension, especially if given shortly after the initial PH diagnosis.

Furthermore, a 2021 medical review regarding advances in PH management states that 1-year survival rates have increased to 86%–90% (from 65% in the 1980s). Average long-term survival has increased from 2.8 to 6 years³.

Suppose you or someone you know has been diagnosed with pulmonary hypertension. In that case, the prognosis will depend on many variables, such as the progression of the disease at the time of diagnosis, the underlying cause of the disease, the course of treatment, personal medical history, and any additional health problems experienced. A doctor will provide a more personalized prognosis after considering these variables.

How is pulmonary hypertension diagnosed?

Blood pressure in the lung arteries isn't as easy to measure as your system-wide blood pressure. So pulmonary hypertension diagnosis starts with a physical examination, clinical assessment, risk factor analysis and gradually moves to more specialized tests.

These may include blood work, genetic testing, echocardiograms, and electrocardiograms. To better understand how the area around the heart is functioning, the doctor may also order x-rays (coronary angiography or coronary arteriography) or an MRI. The most reliable way to measure blood pressure in the pulmonary arteries is by cardiac catheterization⁴ (sometimes called cardiac cath or heart cath). This procedure involves inserting a small, flexible tube into a vein in your arm or leg, guided to your heart. A cardiac cath takes place under IV sedation and usually takes about an hour.

Types of pulmonary hypertension

The World Health Organization (WHO) classifies pulmonary hypertension into the following groups:

Group 1

In Group 1, pulmonary hypertension and narrowing of the arteries may be due to an unknown cause (idiopathic), genetic, drug-induced (by methamphetamines, for example), or from certain other diseases, like congenital heart disease, liver disease, portal hypertension, connective tissue disease, HIV, or chronic hemolytic anemia.

In this group, the right side of the heart is affected.

Treatment may involve medication in the form of pills or inhalers.

Group 2

Group 2 is for pulmonary hypertension caused by left heart disease (LHD). According to the Pulmonary Hypertension Association, the heart is “unable to keep up with the blood returning from the lung — causing a backup of blood that raises the pressure in the lungs⁵.”

This group has to do with the valves on the left side of the heart.

Treatment usually is tailored to the underlying lung disease.

Group 3

With this form of pulmonary hypertension, lung disease or low oxygen levels are the cause. Examples of lung diseases that could cause pulmonary hypertension are chronic obstructive pulmonary disease (COPD), obstructive sleep apnea, interstitial lung disease, or other lung diseases that lower blood oxygen.

Similar to Group 2, interventions will focus on treating underlying lung disease.

Group 4

Chronic thromboembolic pulmonary hypertension (CTEPH) is the body’s inability to dissolve a blood clot that travels to one of the pulmonary vessels, blocking and increasing blood pressure.

Treatment may involve surgical removal of a clot. In cases when surgery is not an option, oral medication may be used.

An important distinction for Group 4 is that surgery may provide a cure. In a 2020 study examining life expectancy after pulmonary endarterectomy (PEA), they found that “life expectancy following PEA was shorter compared to the general population, but the difference was relatively small⁶.” For patients who survived the operation and early postoperative phase, survival at 15 years was 59%⁶ compared to 71% in the matched general population.

Group 5

This category is for pulmonary hypertension with an unknown cause or due to multiple factors that are systemic, metabolic, or blood disorder-related.

Therapy will usually focus on treatment for the underlying disease or disorder.

How does pulmonary hypertension progress over time?

The World Health Organization also categorizes the stages of pulmonary hypertension into four distinct classes, that are distinguished by health, functioning, and wellbeing over the course of time.

Class 1

Symptoms are not yet noticeable, and the condition doesn’t impose limits while at rest or being physically active.

Class 2

You may be somewhat limited in physical activities. Symptoms may arise while being active, but not while you’re resting.

Class 3

Physical activity is limited by symptoms at may be noticeable during slight exertion or ordinary activities, but not while you’re resting.

Class 4

Noticeable symptoms are experienced while resting or being active. Heart failure is a risk.

Symptoms at the beginning of pulmonary hypertension

In the early stages of PH, you might experience:

• Shortness of breath

• Dizziness (particularly during physical activity)

• Fatigue

Advanced symptoms

As the condition advances, symptoms become more noticeable, and may include: 

• Shortness of breath at rest 

• Dizziness and fainting 

• Chest pain

• Ankle and abdominal swelling

• Bluish tint to the skin

• Quickened pulse or heart palpitations

Lifestyle changes

In addition to medical interventions for pulmonary hypertension, doctors are likely to recommend lifestyle changes. The goal will be to improve overall heart health and eliminate any harmful lifestyle factors that could affect your heart.

Regular exercise

While fatigue related to pulmonary hypertension can reduce the motivation to exercise, exercise itself can help improve energy levels over time. Mild to moderate exercises, such as walking or cycling is recommended. However, it is important to discuss with your doctor which types of exercise are suitable.

Sleep schedule

Getting the proper amount of sleep at night will give your heart time to recover from the day's activities and help minimize feelings of daytime fatigue.

Nutrition

Just as with having systemic high blood pressure, certain foods are best avoided with pulmonary hypertension. Processed foods, foods high in fat and cholesterol foods, and foods high in sodium do not support overall heart health. Also, a 2009 study⁷ indicates that a lack of iron can worsen PH, while an iron infusion reduced symptoms.  Consider speaking to your doctor or a dietitian about building a suitable meal plan.

Butt out cigarettes

When your lungs are having a hard time providing the proper amount of oxygen to your blood, it becomes especially dangerous to smoke. In the US, smoking cessation help and resource referrals are available through 1-800-QUIT-NOW (1-800-784-8669).

The lowdown

Untreated, the prognosis for pulmonary hypertension is poor. Heart failure is a major cause of death. However, following a proper treatment protocol can have a significant positive impact on prolonging life expectancy. While there's no cure for the condition, there are steps that can be taken to slow pulmonary hypertension progression, minimize its effects, and manage the symptoms.

In the last two decades, medical advances have increased survival rates. Some individuals may live 20 years from the time of diagnosis. However, rates depend on early detection and treatment, usually in the form of medication, surgery, or supplemental oxygen therapy.