Pulmonary hypertension (PH) develops when the blood pressure in the vessels between the heart and lungs is too high. This extra pressure puts a strain on both the heart and lungs. Over time, PH can worsen, leading to dysfunction of the lungs and their blood vessels.

Because PH gets worse over time, it can eventually result in end-stage PH. At this point, chronic (long-term) lung dysfunction and vessel damage lead to heart failure. Although PH can’t be cured, treatment can help manage it.

Additionally, medications that help control symptoms in the early stages may not work as well later on. Lung transplantation may be an option for some people when medications no longer work. Others may require a heart-lung transplant, due to the extent of heart disease or failure.

Unfortunately, many people are not eligible for a transplant due to strict criteria that need to be met. Extended wait times may also prevent a person from being able to undergo one of these operations.

Although there is no true cure for PH, there are treatments and supportive care options that improve quality of life for people with end-stage PH and comfort those with the disease.

What Is End-Stage Pulmonary Hypertension?

End-stage PH is an advanced heart and lung disease in which chronic damage to the lungs and the lungs’ blood vessels leads to heart failure. When the disease is advanced, it becomes untreatable, either because the treatments available are no longer working or because the heart and lungs are too damaged. Ultimately, end-stage PH leads to death.

End-stage PH is not an official stage of PH. The World Health Organization (WHO) has a classification system for the condition that doctors use to describe how advanced a person’s PH is based on symptoms and clinical testing.

Functional classes of PH range from class 1 to class 4 (sometimes written in Roman numbers, e.g., class I to class IV). Class 1 describes people with few symptoms, even during physical activity. Class 4 describes people with symptoms of right heart failure at rest. People with class 4 PH do not necessarily have end-stage PH, as they can sometimes improve with treatment.

Risk Factors in Developing End-Stage Pulmonary Hypertension

PH that develops on its own without a known cause is called idiopathic pulmonary arterial hypertension. However, PH frequently occurs because of one or more underlying conditions. Having associated conditions can make managing and treating PH difficult and can increase a person’s risk of developing end-stage PH.

Conditions that commonly cause PH include:

• Chronic obstructive pulmonary disease (COPD)
• Interstitial lung disease — A group of conditions, including pulmonary fibrosis, that cause scarring of the lungs, making it hard to breathe and get enough oxygen into your blood
• Scleroderma and other autoimmune diseases
• Heart valve disease
• Obstructive sleep apnea

Having one or more of these conditions can increase a person’s risk for PH, and having PH alongside other illnesses is associated with worse outcomes.

Monitoring End-Stage Pulmonary Hypertension

Many people wonder, what are the symptoms of end-stage PH? As PH progresses, a person may notice worsening symptoms, such as shortness of breath, fluid retention, and chest pain. Impaired lung function can lead to hypoxia (low blood oxygen), which can cause dizziness, light-headedness, and syncope (fainting).

Studies have found the most common causes of death in end-stage PH include right heart failure, noncardiac causes, and sudden death.

Additionally, as PH progresses, it can affect other parts of the body. For example, people with advanced PH often develop kidney or liver problems. Because of this, people with end-stage PH need regular monitoring by doctors or health care teams to ensure the best care.

By monitoring heart function, you and your doctors can have a good sense of whether your PH is getting worse. An echocardiogram is a noninvasive way to measure the hemodynamics (blood flow) of the heart. An echocardiogram can also measure blood pressure in the heart and lungs, which can help track the progression of PH.

Additionally, echocardiographic assessment can be used to estimate the cardiac index, which determines how well the heart is pumping blood to the body.

End-Stage Pulmonary Hypertension Treatments

According to StatPearls, an online resource for health care providers, the average length of time that someone with class 4 PH lives is less than six months. However, this estimate depends on their treatment and health characteristics.

The goal of treating end-stage PH is to manage a person’s symptoms and provide relief and support. The type of PH treatment that a doctor or supportive team may prescribe depends on a person’s other chronic conditions, symptoms, and other factors contributing to their overall health.

Palliative Care

Palliative care is supportive care aimed at helping people living with chronic conditions like PH manage their symptoms and improve their quality of life. Palliative care can help a person with PH manage pain and other physical symptoms like shortness of breath — as well as emotional, social, and spiritual burdens they may carry.

Although palliative care is often associated with end-stage PH, it can help at any stage of the condition. A person with PH can seek palliative care while undergoing other treatments to manage PH and can continue to receive palliative care after stopping those treatments.

Some doctors recommend that people diagnosed with PH begin palliative care as soon as possible to manage their chronic condition and mental well-being.

Invasive Palliative Care for End-Stage Pulmonary Hypertension

Health care providers sometimes recommend surgery for people with end-stage PH to relieve symptoms, improve heart or lung function, and in some cases improve survival rates.

Atrial Septostomy

For people with pulmonary arterial hypertension (PAH) who are waiting for a transplantation, or for those who aren’t eligible for transplantation, a doctor may perform an atrial septostomy. During this procedure, a doctor creates a shunt (an opening) in the upper chambers of the heart, called the atria. This septal shunt helps some of the blood in the heart skip past the lungs, where there may be too much pressure.

Although this causes a decrease in blood oxygen, research has shown that the shunt can improve blood flow, symptoms, and possibly survival rates in those with end-stage PAH.

Potts Anastomosis

Potts anastomosis may represent a future direction for palliative surgical treatment in PH. In the past, this procedure was performed in children with heart defects but is now being studied in adults with PAH.

The procedure entails creating a direct connection, called an anastomosis, between two blood vessels: the left pulmonary artery and the aorta. The goal of this surgical detour is to decompress the pressure in the right ventricle of the heart in order to relieve it from overworking and to provide better blood flow to the body.

While extreme, a Potts anastomosis may be effective at improving symptoms without decreasing blood oxygen to the upper body.

Right Ventricular Assistive Device

A right ventricular assistive device (RVAD) can be attached to the heart to improve ventricular function by helping the heart pump blood. An RVAD is usually implanted temporarily. It has been used in cases of PAH. However, more studies on this device are still needed.

Noninvasive Palliative Care for End-Stage Pulmonary Hypertension

Noninvasive palliative care strategies can take different forms. A health care provider will recommend a strategy based on what the person with end-stage PH might need. It could mean additional medications, as well as therapy or social support.

Palliative Drug Therapies

A doctor might prescribe prostacyclin or prostanoids, like epoprostenol (Flolan), as part of end-stage PH treatment. These medications are effective at vasodilating — opening up blood vessels — and have anticlotting properties. This treatment can temporarily decrease blood pressure in the lungs and can be combined with other therapies.

Other potential drug therapies can help, depending on the needs of the person with end-stage PH. These could include pain medications, treatments for nausea and shortness of breath, and medications for anxiety or depression.

Counseling and Group Support

Creating a strong support system is an important and often overlooked part of managing chronic illness. Anxiety and depression are common among people living with PH. Palliative care teams often include counseling services to help people with end-stage PH process their illness and grief. Professional therapists, social workers, and pastoral counselors offer emotional, practical, and spiritual support to the person with PH and their family.

Group therapy or PH support groups can also provide comfort. These groups unite people experiencing the same physical challenges and who are processing similar types of grief. Group support can be found in online communities such as myPHteam, which can help connect people with PH who are navigating similar journeys.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 55,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

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