What are the symptoms of end stage pulmonary hypertension

Pulmonary hypertension develops when the vessels that bring blood from your heart to your lungs become narrowed.

Blood is moved from the heart to the lungs to be replenished with oxygen, so this narrowing can push oxygen-starved blood back into your heart. The result is shortness of breath and breathing problems from a lack of oxygen and eventually heart problems from the strain this backup causes.

In this article, we discuss the symptoms and possible complications of pulmonary hypertension.

What are the symptoms of end stage pulmonary hypertension

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Frequent Symptoms

If you get winded climbing stairs or become short of breath while you go about your daily activities, it could be a sign of pulmonary hypertension. Shortness of breath with activity—also called dyspnea on exertion—is the primary symptom that leads people to visit their doctor and receive a diagnosis of pulmonary hypertension.

Your symptoms may gradually develop over time without you noticing until they become more severe. Some people overlook developing symptoms because many people have other chronic conditions that share similar symptoms to pulmonary hypertension.

The most common symptoms of pulmonary hypertension are:

  • Shortness of breath
  • Chest pain
  • Fainting
  • Dizziness
  • Fatigue
  • Weakness

About 80% to 99% of people with pulmonary hypertension experience shortness of breath and chest pain.

Other symptoms of pulmonary hypertension may develop as the condition gradually worsens over time.

Advanced symptoms of pulmonary hypertension can include:

  • Throbbing in your chest
  • Racing heartbeat
  • Palpitations
  • Extreme shortness of breath, even while resting
  • Swelling (edema) in your face, abdomen, legs, or ankles

Recap

Symptoms of pulmonary hypertension—like shortness of breath and chest pain—are common to a number of other conditions. For many people, a diagnosis isn't made until the condition has reached advanced stages.

Rare Symptoms

Pulmonary hypertension is not curable and gradually worsens. As this happens, you may develop more severe symptoms. These symptoms tend to be more individualized based on the severity and stage of the disease. These aren't as much rare as they are less common.

Advanced signs and symptoms that may not be seen until later stages of the disease or alongside other complications include:

  • A cough that brings up blood (hemoptysis)
  • An enlarged heart
  • An enlarged liver
  • Low blood pressure (hypotension)
  • A hoarse voice
  • Cyanosis, or a blue tone to your skin

Complications/Sub-Group Indications

Other chronic medical problems are often present in people with pulmonary hypertension, either as a consequence or as an underlying cause of this condition. Examples include:

  • Chronic liver disease or cirrhosis, where scar tissue replaces healthy liver tissue
  • Scleroderma, which causes hardening of the skin and connective tissues
  • Lupus, where your immune system attacks its own tissue
  • Chronic obstructive pulmonary disease (COPD) (a group of lung diseases that block airflow and make it difficult to breathe)
  • Pulmonary fibrosis (scarring of the lungs)
  • Aortic valve disease (narrowing of the valve in the large blood vessel branching off the heart [aorta])
  • Left-sided heart failure
  • Congenital heart disease (heart disease that presents at birth)
  • Mitral valve disease, where the mitral valve (controls blood flow from the left atrium into the left ventricle) becomes damaged or diseased
  • Blood-clotting disorders
  • Obesity
  • Sleep apnea, where breathing suddenly stops and starts while you are sleeping
  • Lung tumors
  • Thyroid disease
  • Metabolic disorders, where abnormal chemical reactions in your body disrupt your metabolism
  • Sickle cell disease
  • HIV infection
  • Parasitic infections

Women are more likely than men to develop pulmonary hypertension, and your risk increases with age too.

As pulmonary hypertension worsens, a number of complications can develop. One of the most common complications is heart failure. When the pulmonary artery, which moves blood from the heart to the lungs, narrows, blood backs up into the right ventricle. The right ventricle receives blood from all over the body. A backup here could lead to heart failure and other problems throughout the body

As this backup continues over time, the right atrium can become stretched and weak. It also has to work harder to pump blood through the narrowed vessel to the lungs. This develops into right-sided heart failure.

The left side of your heart may also try to compensate for the lack of oxygen-rich blood coming from the lungs, working harder to pump blood out to your body. This leads to left-sided heart failure. These conditions—separately or together—are called congestive heart failure.

Other complications that could develop with pulmonary hypertension include:

  • Vascular disease (problems in your blood vessels)
  • Anemia
  • Blood clots
  • Abnormal heart rhythms (arrhythmia)
  • Liver damage
  • Pericardial effusion (collection of fluid around the heart)

In advanced stages, you could end up experiencing several complications or becoming so short of breath that you struggle to do even basic tasks. Cor pulmonale describes extreme pulmonary disease in both the heart and lungs.

When to See a Doctor/Go to the Hospital

If you get short of breath once in a while or have symptoms that come and go, you should schedule an appointment with your primary care provider. Dietary and lifestyle changes can help improve your general health and prevent cardiovascular problems like pulmonary hypertension.

If you are already being treated for pulmonary hypertension, you doctor will also teach you how to watch for progression of the disease. Call the doctor who is managing your condition if you have:

  • Weight gain of more than 5 pounds in a single week
  • Increased swelling in your abdomen, arms, or legs
  • Worsening shortness of breath, especially at rest
  • Worsening cough
  • Restlessness
  • Confusion
  • Constant dizziness
  • Nausea
  • Poor appetite

Sometimes, having a chronic condition can make it difficult to tell when things are getting worse. Your doctor will tell you when to call and when to seek emergency care. If you become so short of breath that you are unable to speak in a full sentence or carry on with your normal daily activities, this is a red flag.

Other symptoms that could signal an emergency include:

  • New-onset, severe chest pain
  • A heart rate that's above 150 beats per minute
  • Sudden weakness or paralysis
  • Sudden, severe headaches
  • Loss of consciousness

Recap

Don't hesitate to call your doctor if you notice new symptoms or your existing symptoms get worse. Call 911 immediately for things like new or severe chest pain, intense shortness of breath, or loss of consciousness.

Summary

Pulmonary hypertension is a severe, chronic disease that typically occurs alongside similar chronic conditions like heart failure and COPD. Because these conditions share many symptoms, like shortness of breath, it can be difficult to diagnose pulmonary hypertension until it reaches advanced stages. Talk with your doctor about any new or worsening chronic symptoms you may have.

A Word From Verywell

Pulmonary hypertension usually develops as a result of other chronic health conditions like obesity, high blood pressure, and heart disease. Be sure to see your doctor for wellness visits to address these issues before they progress into more serious conditions. There are many causes for shortness of breath, and your doctor is the best person to pinpoint the cause.

Frequently Asked Questions

  • What is the most common symptom of pulmonary hypertension?

    Shortness of breath is the most common symptom of pulmonary hypertension. You'll typically notice it especially while performing daily activities like walking and climbing stairs.

  • Could my symptoms be caused by something else?

    Symptoms that are common in pulmonary hypertension, like shortness of breath, can happen in a number of other conditions. Talk to your doctor about how long you have been having these symptoms and when they happen to help them make an accurate diagnosis.

  • Can I prevent pulmonary hypertension?

    For some people, pulmonary hypertension is genetic. There are a lot of lifestyle choices you can make—like eating well and exercising—that can help prevent a number of cardiovascular problems, including pulmonary hypertension.

  • Can pulmonary hypertension get worse over time?

    Pulmonary hypertension is a chronic, progressive disease that gets worse in most cases, leading to other problems like heart failure. However, with early diagnosis and treatment, you can slow disease progression and improve your symptoms.

What are the symptoms of end stage pulmonary hypertension

By Rachael Zimlich, BSN, RN
 Rachael is a freelance healthcare writer and critical care nurse based near Cleveland, Ohio.

Thanks for your feedback!

What happens in end stage pulmonary hypertension?

Pulmonary arterial hypertension (PAH) is a condition characterised by increased pulmonary vascular resistance which can lead to right heart failure and premature death. It imposes a significant burden on patients' lives, affecting their physical, emotional and social wellbeing.

Can pulmonary hypertension cause sudden death?

Sudden cardiac death is now encountered more often in PAH patients. In the American National Institute of Health registry, 106 deaths were reported in a cohort of 194 patients with idiopathic PAH, of which 26% were sudden.

How do you know when pulmonary hypertension is getting worse?

Symptoms of pulmonary hypertension As the disease gets worse, symptoms can include the following: Increased shortness of breath, with or without activity. Fatigue (tiredness) Chest pain or pressure.

What is the life expectancy of someone with severe pulmonary hypertension?

Because the disease often isn't diagnosed until later stages, pulmonary hypertension survival rates are low. Some studies have shown that pulmonary hypertension life expectancy is as little as one year after diagnosis but can be five years or more.