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How Long Can You Live With Pulmonary Hypertension

Which Diseases Are Common In People With The Condition

How long can a Person live with Pulmonary Hypertension

There are no clear statistical data on the prevalence of pulmonary hypertension. Since pulmonary hypertension is seen in multiple different conditions, it is perhaps better to look at the prevalence in each condition. It is fairly common in patients with heart failure, COPD, and sleep apnea. It occurs in 7% to 12% of patients with collagen vascular disease, and in less than 5% of HIV patients.

  • People may also complain of chest pain and angina.
  • Depending on the underlying associated disease, pulmonary artery hypertension can have other manifestations. For example, characteristic skin changes seen in scleroderma, or the signs of liver disease seen in portopulmonary hypertension.
  • Signs of pulmonary hypertension may include:

  • Rapid breathing, hypoxia , and swelling in the legs.
  • In severe pulmonary hypertension, the health care professional may hear louder than normal components of heart sounds when he or she listens to the heart with a stethoscope .
  • The doctor may also feel elevation of the chest wall when the heart pumps and this may indicate enlargement of the right side of the heart suggestive of pulmonary hypertension .
  • What Causes Pulmonary Hypertension

    The following are some known causes of pulmonary hypertension:

    • The diet drug “fen-phen.” Although the appetite suppressant “fen-phen” has been taken off the market, former fen-phen users have a 23-fold increase risk of developing pulmonary hypertension, possibly years later.
    • Liver diseases, rheumatic disorders, lung conditions. Pulmonary hypertension also can occur as a result of other medical conditions, such as chronic liver disease and liver cirrhosis rheumatic disorders such as scleroderma or systemic lupus erythematosus and lung conditions, including tumors, emphysema, chronic obstructive pulmonary disease , and pulmonary fibrosis.
    • Certain heart diseases. Heart diseases, including aortic valve disease, left heart failure, mitral valve disease, and congenital heart disease, can also cause pulmonary hypertension.
    • Thromboembolic disease. A blood clot in a large pulmonary artery can result in the development of pulmonary hypertension.
    • Low-oxygen conditions. High altitude living, obesity, and sleep apnea can also lead to the development of pulmonary hypertension.
    • Genetics. Pulmonary hypertension is inherited in a small number of cases. Knowing that someone in the family had or has pulmonary hypertension should prompt you to seek early evaluation should symptoms occur.

    Pulmonary hypertension may also be caused by other conditions, and in some cases, the cause is unknown.

    There Are Four Types Of Pah

    According to Galié, “most types are considered idiopathic,” meaning there’s no known cause or major precipitating factor.

    About 6% of cases are heritable. They’re passed down from a parent, most likely due to a mutation in a protein receptor that affects tissue growth. “If you’re carrying this gene it does not mean you’re definitely going to develop PAH, but you are at a higher risk,” Maresta says. “We think you need more than one trigger,” such as a pre-existing condition.

    The idiopathic and heritable forms of PAH are at least twice as common in women compared to men. In fact, PAH is most often diagnosed in women between the ages of 30 and 60.


    In some cases, PAH can be drug-induced. The medication most famous for causing PAH is fen-phen , a weight loss drug that was popular in the 1960s and 1970s but is no longer on the market.

    Lastly, associative cases occur when PAH goes hand in hand with another pre-existing condition, such as HIV, congenital heart disease or scleroderma.

    All four typesidiopathic, heritable, drug-induced and associativeare treated similarly, but the prognosis might vary as a result of which one you have.

    For example, notes Maresta, patients who have associative PAH because they also have scleroderma are already more prone to renal disease, GI complications and heart complications. “That can impact your overall life expectancy,” Maresta adds.

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    Group : Ph Due To Lung Disease And Hypoxia

    Group 3 PH is caused by a lack of oxygen over extended periods of time, known as hypoxia. This can be caused by several conditions, including sleep apnea, interstitial lung disease, and chronic obstructive pulmonary disease. People who live at higher altitudes with lower oxygen levels can also develop group 3 PH.

    People with chronic obstructive pulmonary disease and PH have been found to have better survival rates than those with interstitial lung diseases and PH. The five-year survival rates for group 3 PH are 44 percent for women and 34.3 percent for men. It has also been found that survival rates are better for those with less severe heart failure symptoms using the New York Heart Association scale.

    Life Expectancy And Prognosis With Pulmonary Hypertension

    How long Do You Live with Pulmonary Hypertension?

    You may already be familiar with hypertension, which refers to having high blood pressure. Its associated with an increased risk of having a heart attack or stroke. However, being diagnosed with pulmonary hypertension is a separate, rare lung disorder. Pulmonary hypertension still involves high blood pressure, but pulmonarypertainsto the lungs.

    The American Lung Association estimates that 5001,000 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.

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    Pulmonary Hypertension Definition And Facts

    • Pulmonary hypertension is abnormally elevated pressure in the pulmonary circulation.
    • The classification of primary and secondary pulmonary hypertension has been reclassified, and now is based on the main underlying disease or condition, symptoms, and treatment options for pulmonary hypertension. There are five classes or groups in this new classification system.
    • Pulmonary hypertension can have no identifiable cause and is then referred to as idiopathic pulmonary hypertension .

    Pulmonary hypertension occurs when the pressure in the pulmonary arteries increases abnormally. To help explain this condition further, here is how the pulmonary arteries work.

    What are pulmonary arteries?

    The human body has two major sets of blood vessels that distribute blood from the heart to the body. One set pumps blood from the right heart to the lungs, and the other from the left heart to the rest of the body . When a doctor or a nurse measures the blood pressure on a person’s arm, he/she is measuring the pressures in the systemic circulation. When these pressures are abnormally high, the person is diagnosed as having high blood pressure .

    The portion of the circulation that distributes the blood from the right side of the heart to the lungs and back to the left side of the heart is referred to as the pulmonary circulation.

    What are normal pulmonary artery pressure ranges?

    Group : Ph Due To Left Heart Disease

    Group 2 PH is caused by heart disease on the left side of the heart. This is also known as pulmonary venous hypertension and is the most common type of PH. Heart diseases that cause this type of PH include:

    • Coronary artery disease
    • Long-term high blood pressure throughout the body
    • Heart defects that formed before birth
    • Mitral valve problems

    Women have a better five-year survival rate than men, at 61.8 percent compared to 47.1 percent. Another study found that people with congenital heart disease have better outcomes than those without.

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    Adapting And Moving Forward

    Even after years of relatively stable health, every long-term survivor needs to be prepared for new health challenges. Many PH patients talk about their new normal as an empowered term to describe their ability to factor PH into a foundation for a new life different from their old life, but no less full or meaningful. If youve been living with PH for years, you may have to adjust to another new normal on a regular basis. This constant reinvention of self can be physically and emotionally draining and requires an enormous amount of resilience.

    Palliative Care And Lung Transplant

    Can I live with Pulmonary Hypertension

    Referral for lung transplant in PAH is recommended for patients with New York Heart Association functional class of III or IV despite maximal medical therapy, patients on parenteral prostanoids and patients with rapidly progressive disease, as evidenced by worsening 6MWT, NT-proBNP, right ventricular function and haemodynamics . Patients with PAH whose mortality risk is high based on comprehensive assessment of multiple variables should be considered for lung transplantation . Given the symptom burden and the sometimes prolonged waiting time for lung transplant, concomitant palliative care services are crucial to help improve HRQoL and minimise the suffering of PAH patients.

    Palliative care for patients on the lung transplant waiting list involves taking care of symptoms, planning for advance care and providing emotional and spiritual support . A retrospective cohort study of lung transplant candidates referred for palliative care by Colmanet al. showed that palliative care could be provided without harming the chances of a successful transplant. Palliative care should not be delayed until the moment when a patient is disqualified for transplantation. Pulmonary hypertension referral centre clinics should manage these end-stage patients in multidisciplinary clinics where they can get support from social workers, pastoral counsellors and palliative care physicians.

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    Risk Factors For Pulmonary Hypertension

    Because the disease is so wide in scope, there are many potential risk factors for pulmonary hypertension. Along with the specific factors related to genetics, lifestyle, and medications, having any of the diseases or conditions known to cause pulmonary hypertension is a significant risk factor.

    These are some of the risk factors that may increase your chances for developing pulmonary hypertension:

    • A family history of pulmonary hypertension
    • Being overweight
    • Illegal drug use
    • Prolonged exposure to high altitudes
    • Having any of the diseases or conditions that can cause pulmonary hypertension

    What Is Pulmonary Arterial Hypertension

    Pulmonary hypertension is a life-threatening condition that gets worse over time, but treatments can help your symptoms so you can live better with the disease. It may take some planning, but plenty of people who have it find ways to do all the things they love, just as they did before they were diagnosed.

    Having pulmonary arterial hypertension means that you have high blood pressure in the arteries that go from your heart to your lungs . Its different from having regular high blood pressure.

    With PAH, the tiny arteries in your lungs become narrow or blocked. Its harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries, and after a while the heart muscle gets weak. Eventually, it can lead to heart failure.

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    Group : Pulmonary Arterial Hypertension

    Group 1 PH, also known as pulmonary arterial hypertension, can be caused by many factors. In some cases, there is no known cause of the disease . In other cases, it can be caused by genetics , drugs and toxins , or other underlying diseases such as:

    • Autoimmune conditions, such as scleroderma
    • Liver disease
    • Congenital heart disease
    • HIV infection

    Underlying conditions that cause PH can also affect disease outcomes. For example, individuals with both PH and scleroderma have a 40 percent chance of living two years after diagnosis.

    What Is The Life Expectancy Of Someone With Cll

    How long can a person live with pulmonary hypertension?

    Patients with CLL have a wide range of prognoses when they are diagnosed. Because to CLL problems, some individuals die quickly, within 2-3 years of diagnosis. Most patients live 5â10 years, with a relatively benign early course followed by a fatal, progressive, and resistant phase lasting 1-2 years. The average life expectancy for people with CLL is 10 years.

    Why does mortality increase after 10 years? Many factors contribute to increased mortality after 10 years, including disease progression, treatment side effects, and comorities associated with aging. Patients who survive longer than 10 years tend to do so because their cancer has been in complete remission for many years. These survivors represent a group that can be studied over time to learn more about what causes CLL death and how it can be prevented.

    Survival rates improve if the disease is found and treated early. The better your response to therapy at the time of diagnosis, the greater your chance of survival. As well, patients who start treatments sooner after diagnosis appear to do better than those who don’t get treatment until later on.

    There are several types of therapies available for patients with CLL. Some treatments work best if given alone while others work best when used in combination with other drugs. It is important for patients to discuss options with their doctor. This process helps identify the most effective treatments for each patient.

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    Diseases Of The Lungs

    In some cases, increased pressure in the lungs may be triggered by diseases affecting the lungs. Such diseases may include chronic obstructive airway disease, interstitial lung disease, pulmonary fibrosis , pneumonia, tracheobronchial disease, pulmonary thromboembolism and cancer.

    Considering Several Factors

    As mentioned, the life expectancy of dogs with pulmonary hypertension can vary due to individual factors. The pulmonary hypertension can be caused by different conditions as seen above and each of them can have a different impact on the dog’s overall life span. How advanced the pulmonary hypertension is can also play a role.

    Pulmonary hypertension is divided in different functional classes based on the dog’s clinical signs. Dogs categorized under Functional class I do not have exercise intolerance and are capable of exercising without manifesting fatigue, trouble breathing, chest pain or fainting. Functional class II patients do fine during rest, but exercise can trigger trouble breathing, fatigue, chest pain or fainting. Functional class III patients do fine during rest but show significant signs with minimal activity. Signs include severe labored breathing, fatigue, chest pain or fainting. Finally, Functional class IV patients cannot tolerate any activity. Even at rest, these dogs show symptoms and develop right-sided heart failure as a result of their severe pulmonary hypertension.

    When To See A Gp

    See a GP if you have any symptoms of pulmonary hypertension. They may ask you about your symptoms and medical history, and they may carry out a physical examination.

    Correctly diagnosing pulmonary hypertension can sometimes take time because its symptoms are similar to those of many other heart and lung conditions.

    Tests you may have include a type of heart scan called an echocardiogram, and right heart catheterisation, where a thin, flexible tube is inserted into your pulmonary artery.

    The changes in the pulmonary arteries that lead to pulmonary hypertension can be caused by:

    • problems with the smaller branches of the pulmonary arteries
    • conditions that affect the left side of the heart
    • lung diseases or a shortage of oxygen in the body
    • blood clots that cause narrowing or a blockage in the pulmonary arteries

    Read more about the causes of pulmonary hypertension.

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    So How Do You End Up With This Exceptional Team

    Shortness of breath is a typical first symptom, as are fatigue, heart palpitations, dizziness, swollen ankles in other words, the same symptoms as too many other illnesses. Some patients go several years undiagnosed before finding a specialist who has worked with PH patients.

    My first symptoms showed up three years ago when I thought I had been hit with 24-hour flu during a Thanksgiving visit to a mountain town in North Carolina, 4,118 feet above sea level. I had spent vacations there for years, with no problems.

    My second night there, I threw up, my heart raced, I was nauseated, and didnt feel like eating. It lasted 24 hours.

    I returned to the mountains over New Years, and it happened again, also on my second night.

    In the spring, my husband and I flew to Denver, the mile-high city, 5,280 feet above sea level. I felt woozy in the airport and came down with similar issues by early evening. The next day, we drove up to Minturn, CO, 7,861 feet. I went to bed for three days, vomited, my mind confused. I need to go to Urgent Care, I finally murmured.

    A nurse put a pulse oximeter on my index finger to measure the oxygen level in my blood. It was 61. Is that bad? I asked. She shared a glance with another nurse. Healthy people usually have 95-99 percent oxygen, but anything over 90 is OK.

    High altitude is not my friend.

    Linda had just returned from Iceland when I met her, which was encouraging. Travel is my passion, and my job was writing about travel.

    Questions For Your Doctor

    What it means to live with pulmonary hypertension today

    You may want to write down a list of questions before your appointment, so you can make sure you ask your doctor everything you want to. It can also help to have a friend or family member with you to help you get the answers you want.

    Some possible questions are:

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    Other Facts And Figures

    There are some other important facts and figures for patients and caregivers that can help to answer the question, can I die from pulmonary hypertension. These include the following:

    • This condition typically occurs along with another condition or disease, such as lung diseases, pregnancy, sleep apnea, thyroid diseases, using illicit drugs or certain diet medications, blood vessel and heart diseases, liver diseases, connective tissue diseases such as scleroderma and lupus, or HIV infection.
    • The majority of patients are older women, however, this condition can affect all ages, races and ethnic groups.
    • Before 1995, patients lived on average for less than three years after being diagnosed. Now, quality of life and survival rates have improved with new treatments.
    • This condition resulted in 260,000 hospital visits and 15,668 deaths in the United States in 2002.
    • Between the years 2000 and 2002, 807,000 patients had to be hospitalized due to this condition. Of these patients, 66 percent were age 65 or older and 61 percent were women.


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