What Are The Symptoms Of Pulmonary Hypertension
Symptoms of pulmonary hypertension do not usually occur until the condition has progressed. The first symptom of pulmonary hypertension is usually shortness of breath with everyday activities, such as climbing stairs. Fatigue, dizziness, and fainting spells also can be symptoms. Swelling in the ankles, abdomen or legs, bluish lips and skin, and chest pain may occur as strain on the heart increases. Symptoms range in severity and a given patient may not have all of the symptoms.
In more advanced stages of the disease, even minimal activity will produce some of the symptoms. Additional symptoms include:
- Irregular heartbeat
- Racing pulse
Eventually, it may become difficult to carry out any activities as the disease worsens.
Who Gets Pulmonary Hypertension
Pulmonary hypertension is a rare condition that can affect people of any age. According to the NHS, its more common in people who have another lung or heart condition. PH can also be associated with other medical conditions such as connective tissue conditions, congenital heart disease, HIV, genetic causes, or it can happen by itself. Read more about the different types of PH and what causes it.
According to the Pulmonary Hypertension Association UK, PH is more common in women. Studies have shown that the female sex hormone is a risk factor for pulmonary arterial hypertension , with PAH around four times more likely in women.
How Pulmonary Hypertension Affects Your Body
Oxygen-poor blood returning to the heart from all body organs and tissues travels from the right side of the heart through the pulmonary arteries into the lungs. Within the lungs, these arteries divide into smaller vessels in order to take in oxygen and get rid of carbon dioxide. The oxygen-rich blood then returns to the left side of the heart from where it is pumped back to all body organs and tissues.
Normally, the blood flows relatively easily through these vessels in your lungs but in PH, the walls of the arteries become stiff, damaged and narrow. This makes blood flow much more difficult and causes the blood pressure in the lungs to rise and the right side of your heart to work harder. Over time, this added stress to the right side of your heart can lead to other medical problems, specifically heart failure.
Pulmonary hypertension is classified by the World Health Organization according to its causes or associated underlying conditions:
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Life Expectancy With Pulmonary Arterial Hypertension
PAH is a progressive condition. This means it gets worse over time. Some people may see symptoms get worse faster than others.
A 2015 study examined the 5-year survival rates for people with different stages of PAH. Researchers found that as the condition progresses, the 5-year survival rate decreases.
Here are the 5-year survival rates researchers found for each stage, or class, of PAH:
- Class 1: 72 to 88 percent
- Class 2: 72 to 76 percent
- Class 3: 57 to 60 percent
- Class 4: 27 to 44 percent
PAH is divided into four stages based on the severity of symptoms.
These stages are based on criteria established by the World Health Organization :
- Class 1. The condition doesnt limit your physical activity. You dont experience any noticeable symptoms during periods of ordinary physical activity or rest.
- Class 2. The condition slightly limits your physical activity. You experience noticeable symptoms during periods of ordinary physical activity, but not during periods of rest.
- Class 3. The condition significantly limits your physical activity. You experience symptoms during periods of slight physical exertion and ordinary physical activity, but not during periods of rest.
- Class 4. Youre unable to carry out any type of physical activity without symptoms. You experience noticeable symptoms, even during periods of rest. Signs of right-sided heart failure tend to occur in this stage.
PAH is one of five types of pulmonary hypertension . Its also known as group 1 PAH.
Most Common Ph Symptoms
Pro Tip
“Pulmonary hypertension can cause shortness of breath with exertion, fainting, low oxygen levels, and/or lower extremity swelling. I listen for patients telling me they are having trouble doing things they used to be able to do because they are now short of breath. For example, patients now having trouble walking to the store or going upstairs.” Dr. Benjamin Ranard
Low oxygen levels in the blood cause general fatigue and shortness of breath when youre physically active. It can also lead to lightheadedness and fainting.
When the heart isnt receiving enough oxygen, you may feel pain in the chest.
Pulmonary hypertension increases pressure in the blood vessels between the heart and the lungs. Over time, this stresses the heart, making it harder for the heart to pump. It may lead to other symptoms, such as fluid build-up in the legs and belly and a lack of appetite.
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What Is The Main Cause Of Pulmonary Hypertension
Pulmonary hypertension is a form of high blood pressure in the vessels that connect the heart to the lungs. There are many reasons that it may develop.
- Left heart disease. This is the most common cause of pulmonary hypertension in the United States. Blood travels from the right side of the heart to the lungs and out to the left side of the heart and then to the body. Problems with the left heart such as a weak squeeze can cause blood to back up, leading to high blood pressure in the lungs.
- PAH . Blood pressure rises as blood tries to move through the narrowed vessels.
- Lung disease and low oxygen
- Blockage of the pulmonary artery
Group 5 Pulmonary Hypertension
Group 5 includes pulmonary hypertension triggered by other disorders. Examples of such diseases or conditions are blood disorders such as polycythemia vera and essential thrombocythemia systemic disorders such as sarcoidosis and vasculitis metabolic disorders such as thyroid and glycogen storage disease and other conditions such as kidney disease and tumors that press on the pulmonary arteries.
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Investigation Into Pulmonary Hypertension
The series of tests required to confirm a diagnosis of pulmonary hypertension can be quite extensive. Your first appointment with your specialist might just be spent organising these tests, which may include:
If your doctor asks you to have any tests before the appointment, please do so because it will speed up the process of determining what the nature of your pulmonary hypertension may be, and how to treat it.
Different Symptoms For Different People
Each person with PAH will experience a different assortment of symptoms. The severity of the symptoms will also differ from person to person. One persons journey with having and treating PAH wont necessarily be helpful to another person because the path with PAH and the treatment options are so individualized.
However, you can draw support from others who have PAH, learn from their experiences, and shape your approach to treating PAH accordingly. Read more about medications used to treat PAH.
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Pah Is Difficult To Diagnose
Given how rare PAH is, when a patient complains about such symptoms as exhaustion and labored breathing, its not unusual for a doctor to first order testing for conditions like asthma and congestive heart failure.
While that makes sense on some levelthose issues are much more common than PAH, explains Galiéit also means that getting diagnosed with PAH may sometimes take a while.
You might see a general practitioner, then a specialist for asthma, then another specialist and so on before finally seeing a pulmonologist or cardiologist who diagnoses the PAH, Maresta explains. It usually takes one year from the presentation of symptoms to get a correct and final diagnosis of PAH.
Plus, notes Galié, your doctor will also need to rule out all other forms of pulmonary hypertension before giving a diagnosis of PAH.
But the good news is that the timeline to PAH diagnosis is starting to speed up a little. Things are slowly changing, as more doctors now know what PAH is and how to identify it, Maresta explains.
Once your physician suspects PAH, your doctor will order a series of tests that may include a chest X-ray, pulmonary function test and exercise tolerance test. If all signs still point to PAH following the results of these tests, youll probably require a right-heart catheterization, which measures the pressure inside the pulmonary arteries.
The Pulmonary Hypertension Life Expectancy
The problem of people who are suffered from final stages pulmonary hypertension is the worse condition. Mostly, the health condition will be decreased constantly although they are taking a proper treatment. Of course, it doesnt mean that you dont do anything. Taking a proper treatment determines the life expectancy. In the earlier phase or people with PHA less than 1 year, they still have up to 85% of life expectancy. If they are suffered from this disease for 3 years the life expectancy is reduced up to 65%. In the final stages pulmonary hypertension or up to 5 years, the chance to live is around 57%. It can be concluded that the life expectancy is still bigger as long as you follow the medication constantly. The life expectancy is also depending on your health condition and also the type of pulmonary hypertension you have.
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Risk Factors For Pulmonary Hypertension
Risk factors for pulmonary hypertension include:
- family history of pulmonary hypertension especially a first-degree relative such as a parent, sibling or children
- connective tissue disorders or autoimmune diseases, including scleroderma, lupus, and rheumatoid arthritis
- living at high altitudes for extended periods of time
- obesity
- chronic liver disease
- infectious diseases including human immunodeficiency virus , hepatitis B and hepatitis C
- use of certain drugs and medications including methamphetamines, chemotherapy, and some diet drugs or appetite suppressants.
Preparing For Your Heart Catheterization
Before the heart catheterization a doctor will explain the risks and benefits of this procedure. You will remain without food or drink 12 hours before the procedure. You may take you regular a.m. medications on the day of the procedure however you should hold any blood thinners or diabetic medications.
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Persistent Pulmonary Hypertension Of The Newborn
This is a type of pulmonary hypertension estimated to affect two in every 1,000 newborn babies.
It has been linked to the mother taking non-steroidal anti-inflammatory drugs during the third trimester of the pregnancy .
Conditions that may also be associated with persistent pulmonary hypertension of the newborn include:
- severe pulmonary hypoplasia when your babys lungs are seriously underdeveloped
- hypoglycaemia an abnormally low level of glucose in the babys blood
- a life-threatening illness caused by the babys body overreacting to an infection
- meconium aspiration syndrome when a newborn baby breathes in a mixture of amniotic fluid and meconium , which can block their airways
Why Do The Pulmonary Arteries Narrow
Scientists believe that the process starts with injury to the layer of cells that line the small blood vessels of the lungs. This injury, which occurs for unknown reasons, may cause changes in the way these cells interact with the smooth muscle cells in the vessel wall. As a result, the smooth muscle contracts and grows more than is normal and narrows the vessel.
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How Is It Different From Systemic Blood Pressure
Systemic blood pressure is a measure of how forcefully blood flows through other arteries in the body.
The Centers for Disease Control and Prevention note that normal systemic blood pressure is below 120/80 mm Hg, where the top number represents systolic blood pressure and the bottom diastolic.
A person has hypertension if their blood pressure is 130/80 mm Hg or higher, and doctors may recommend treatment when a persons blood pressure is consistently 140/90 mm Hg or higher.
Systemic hypertension is more common than PAH. Although both are risk factors for heart disease, PAH is more likely to cause heart failure. Over time, systemic hypertension may damage the heart, causing PAH.
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Research Into Pulmonary Hypertension
Pulmonary hypertension is an area of intense research, particularly with respect to:
- the correct type of medication to use in the different groups of pulmonary hypertension, and in which combinations
- management of right heart failure, and possible new treatment targets or therapies
- treatments at the molecular level, such as monoclonal antibodies, which may be useful tools to slow disease progression
- further genetic markers that may identify people at high-risk of developing pulmonary hypertension.
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What Is The Best Treatment For Pulmonary Hypertension
Dr. Rx
“If you are prescribed oxygen or breathing machines to use all the time, use the oxygen as much as possible. Sometimes patients stop using oxygen because they dont think they need it. You may feel fine without the oxygen. However, even if not symptomatic, low oxygen levels in the blood can be bad for your heart and make pulmonary hypertension worse.” Dr. Ranard
How pulmonary hypertension is treated depends on the cause. Whether its an underlying heart disease, lung disease, low oxygen, blood clots, or bodywide diseases. For instance, if its from blood clots, a blood thinner may be recommended. If you have sleep apnea, that would be addressed.
The one condition that is treated directly with specific medications is PAH. They include calcium channel blockers, endothelin receptor antagonists, prostacyclin pathway agonists and nitric oxide enhancers. are a group of medications that dilate blood vessels. The medications may be pills, inhaled, or intravenous.
Pulmonary hypertension may go away if the underlying cause can be treated. For example, if the cause is weak pumping of the left side of the heart , treating that can improve pulmonary hypertension.
Many causes of pulmonary hypertension are chronic conditions that require long-term care. If you have severe pulmonary hypertension or it is not easily reversible by treating the underlying issue, follow up with a cardiologist or pulmonologist .
How Common Is Pulmonary Hypertension
There are many different types of pulmonary hypertension and some are associated with underlying health conditions, particularly conditions affecting the heart or lungs. Pulmonary hypertension may be more common in people with:
- sickle-cell anaemia
- chronic obstructive pulmonary disease
Generally, pulmonary hypertension can affect people of almost any age, although some types are more common in young women and some in people aged over 40.
One type of pulmonary hypertension, called pulmonary arterial hypertension , is more common in women and affects two to four people in every million each year.
More about the causes of pulmonary hypertension
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Pah Associated With Other Conditions
PAH can be associated conditions that affect your blood vessels, such as:
- pulmonary veno-occlusive disease a rare condition that causes high blood pressure in the lungs
- pulmonary capillary hemangiomatosis when capillaries grow within the lungs, causing blockages
Other conditions or treatments associated with PAH include:
- a condition affecting the structure or composition of your body tissue, such as scleroderma
- surgery to artificially connect the aorta to the pulmonary artery this is often done to treat congenital heart disease and increase the blood supply to the lungs
- portal hypertension a condition where the blood pressure inside the liver is abnormally high, leading to the development of swollen veins
- HIV one in 200 people with HIV will develop PAH
- certain medications or drugs such as fenfluramine, which is now no longer available, and amphetamines, a type of illegal drug
- disorders of the thyroid gland
- glycogen storage disorder
- splenectomy when you have had your spleen surgically removed
- sickle cell anaemia
Sickle-cell anaemia is an inherited disorder where your red blood cells are abnormal and die prematurely, leading to a shortage of red blood cells. The lack of red blood cells means that haemoglobin, a protein normally found in these cells, is released into the blood.
Irregular Heartbeat And Chest Pain
Increased pressure in the heart makes the hearts muscles work harder than they should. Over time, these muscles grow weaker. A weakened heart cant beat as well or as regularly as it once did. Ultimately, this may cause an erratic heartbeat, a racing pulse, or heart palpitations.
Increased blood pressure in the heart and arteries may cause chest pain or pressure. An overworked heart may also cause unusual chest pain or chest pressure.
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Factors That Can Affect Pulmonary Hypertension
While pulmonary hypertension has no cure, you can live an active, fulfilling life by working with your doctor to manage your symptoms. Talk to your doctor before taking over-the-counter medicine, as some can worsen symptoms of pulmonary hypertension or interfere with prescriptions and keep a list of medicines with you. Ask whether you should get a pneumonia vaccination or flu shot. Additionally, pregnancy can pose serious risks for women with pulmonary hypertension, so be sure to discuss birth control options with your physician.
What Is Primary Pulmonary Hypertension
Primary pulmonary hypertension is high blood pressure in the lungs. Its a rare lung disorder in which the blood vessels in the lungs narrow and the pressure in the pulmonary artery rises far above normal levels. The pulmonary arteries carry blood from your body to the lungs where carbon dioxide is traded for oxygen.
Pulmonary hypertension is a serious, chronic disease that can lead to heart failure if its not treated.
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Pregnancy And Pulmonary Hypertension
If you have pulmonary hypertension, before considering conception, please talk to your specialist. Pregnancy in women with pulmonary hypertension is very high-risk, both to the mother and the baby. Many of the medications used to treat pulmonary hypertension are toxic to developing foetuses or untested in pregnant women. Unfortunately, for many women with pulmonary hypertension, carrying a baby to term may not be possible.