Pulmonary hypertension (PH) can arise from many underlying causes and conditions. Regardless of its origin, the condition ultimately puts an increased strain on the heart and lungs. Specifically, PH increases pressure in the blood vessels that take blood from the right side of the heart to the lungs. The condition is progressive and incurable, and it can eventually result in end-stage PH: a point at which long-term damage to the lungs and their blood vessels results in heart failure.
Due to the progressive nature of PH, medications that may initially help control symptoms can become less effective over time. Lung transplantation may be an option for some people when medications no longer work, while others may require a heart-lung transplant, due to the extent of heart disease or failure. Unfortunately, transplant eligibility criteria are strict and exclude many people. 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.
End-stage PH is an advanced heart and lung disease in which long-term damage to the lungs and the lung’s blood vessels leads to heart failure. When the disease is advanced, it becomes untreatable, either because the treatments available are insufficient 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.
PH that develops on its own without an etiology (underlying 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:
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.
As PH progresses, a person may notice a worsening of their 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).
Additionally, PH’s progression can affect other systems in the body. For example, it’s common for those with end-stage PH to also develop kidney or liver disease. As such, end-stage PH requires ongoing monitoring by doctors or health care teams to provide the best support.
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.
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, the presentation of their symptoms, and other factors contributing to their overall health.
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 pursue palliative care after stopping those treatments.
Some doctors advocate for people diagnosed with PH to begin palliative care as soon as possible to manage their chronic condition and their mental well-being.
Health care providers sometimes recommend surgical interventions for people with end-stage PH to provide symptom relief, improve heart or lung function, and in some cases improve survival rates.
For people with pulmonary arterial hypertension (PAH) who are awaiting transplantation, or for those who aren’t eligible for transplantation, a doctor may perform an atrial septostomy. During this procedure, a doctor makes an opening (a shunt) in the upper chambers of the heart, called the atria. This septal shunt helps some of the blood in the heart bypass the lungs, where there may be too much pressure. Although this causes a decrease in blood oxygen, research has shown that it can improve blood flow, symptoms, and possibly survival rates in those with end-stage PAH.
Potts anastomosis may represent a future direction for palliative surgical treatment in PH. This procedure was historically performed in children with heart defects but is now being studied in adults with PAH. The procedure entails creating an anastomosis (direct connection) between two blood vessels: the left pulmonary artery and the aorta. The goal 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.
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 strategies can take different forms. A health care provider will recommend a strategy based on what the person with end-stage PH might require. It could mean additional medications, as well as therapy or social support.
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 individual 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.
Creating a strong support system is an important and often overlooked component of managing chronic illness. Anxiety and depression are common among people living with PH. As such, 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 the myPHteam, which can help connect people with PH who are navigating similar journeys.
On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 46,000 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.