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Living with Pulmonary Hypertension:
All You Need to Know

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1.What is Pulmonary Hypertension?

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It is a rare form of high blood pressure due to which pulmonary arteries and capillaries get damaged. The pulmonary arteries carry blood from the lower right chamber of your heart (right ventricle) into your lungs. Due to the increased pressure in the blood vessels, the heart is forced to pump harder to supply blood to the lungs. This causes severe damage to the heart which may often lead to heart failure.

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2.Symptoms of Pulmonary Hypertension

Pulmonary hypertension symptoms are not evident in its early stages when you are suffering from mild pulmonary hypertension, however, the symptoms become very clear as the condition worsens. Some of the common symptoms of pulmonary hypertension are-

  • Difficulty in breathing
  • Tiredness
  • Giddiness
  • Chest Pressure and Chest pain
  • Increase in pulse rate
  • Heart palpitations
  • Evident swelling in ankles and legs eventually spreading to the abdomen at the final stage.
  • The lips and skin colour turns blue.

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3.Possible Causes and Risk Factors

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The heart consists of four chambers which are divided into two upper chambers called atria and two lower chambers called ventricles. When the blood passes through the heart every time, the right ventricle pumps out blood to the lungs through a huge blood vessel called the pulmonary artery.

Once the blood is passed to the lungs it gets purified by releasing carbon dioxide and picks up oxygen. This purified blood is later passed on to pulmonary arteries, capillaries and veins which eventually reaches the left side of the heart. During this process, the blood flow maintains a low pressure in the lungs.

Pulmonary hypertension is caused due to the changes in cells lining pulmonary arteries which leads to thickening and inflammation of the artery walls. This change leads to blockage of blood vessels leading to high blood pressure. Pulmonary hypertension is further divided into five groups depending upon its causes

Group 1: Pulmonary arterial hypertension - It is a condition that appears due to defects during childbirth or through a genetic mutation passed on through generations. It also occurs due to chronic liver disease or infections such as HIV or schistosomiasis.

Group 2: Pulmonary hypertension caused by left-sided heart disease due to the failure of the left ventricle and left-sided valvular such as mitral valve and aortic valve.

Group 3: Pulmonary hypertension is also caused by disorders in the lungs such as pulmonary fibrosis, emphysema. This group is commonly found in people living in higher altitudes.

Group 4: Pulmonary hypertension caused by chronic blood clots - Blood clots in the arteries of the lung causes severe pulmonary hypertension.

Group 5: In this group, pulmonary hypertension occurs due to various unclear reasons - It could be caused due to blood disorders, metabolic disorders or pressure caused due to tumours beside pulmonary arteries.

Risk factors

The risk of pulmonary hypertension increases in -

  • Young adults as the blood pressure in the lungs are usually high
  • Obesity
  • A family medical condition of hypertension
  • Usage of illegal drugs like cocaine
  • Intake of appetite-suppressant medicines
  • People living in higher altitudes with a family history of pulmonary hypertension

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4.Complications that May Arise from Pulmonary Hypertension

Pulmonary Hypertension can cause multiple complications like-

Enlargement of the right-sided heart- The right ventricle of the heart is expanded to pump more blood through narrow arteries causing a heart failure called cor pulmonale. In this condition, the heart initially thickens its walls to release pressure from arteries but eventually, this leads to suppress the blood flow.

Arrhythmia- The heart starts to beat irregularly from the upper or lower chambers which leads to palpitations and dizziness. This, later on, leads to fatal heart failures.

Bleeding- At advanced stages, Pulmonary Hypertension causes internal bleeding in the lungs. This results in hemoptysis (coughing sessions with blood). This condition is fatal.

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5.Diagnosis and Treatment of Pulmonary Hypertension

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Diagnosis:

Pulmonary hypertension cannot be diagnosed at early stages unless it is found during your regular health check-up. On the presence of Pulmonary Hypertension is diagnosed further checks are performed to know the stage of the disease. Pulmonary Hypertension diagnosis is conducted in the following ways.

Echocardiogram- In an echocardiogram, the condition of the right ventricle of the heart is monitored with images created by the sound waves of the beating heart. This test also helps the doctor to analyse the condition of the heart chambers and valves. The pressure on the pulmonary arteries can also be measured under this test. Sometimes doctors recommend an exercise echocardiogram to observe the performance of lungs and heart under pressure.

Echocardiogram- In an echocardiogram, the condition of the right ventricle of the heart is monitored with images created by the sound waves of the beating heart. This test also helps the doctor to analyse the condition of the heart chambers and valves. The pressure on the pulmonary arteries can also be measured under this test. Sometimes doctors recommend an exercise echocardiogram to observe the performance of lungs and heart under pressure.

Electrocardiogram(ECG)- An abnormal rhythm caused due to the heart's electrical pattern can be observed using ECG. Depending on these patterns doctors diagnose pulmonary hypertension.

Right heart catheterization- Once your echocardiogram shows signs of pulmonary hypertension you are likely recommended to undergo right heart catheterization recommended by your doctor to check the severity of the issue. This procedure is conducted by inserting a thin flexible tube called a catheter into the vein. This catheter is then passed to the right ventricle and pulmonary artery. This procedure helps the doctor to identify the swelling in the arteries.

Blood Tests- Blood tests are recommended to find out substances that cause pulmonary hypertension and its complications. In some cases, the other conditions causing pulmonary hypertension can also be identified.

CT Scan- CT scan is performed by doctors to produce sectional X-ray images of your heart. The doctor injects a dye into the blood vessels to get clear images of the arteries and can also observe the function of the heart and its size. This procedure also helps them in identifying blood clots.

Magnetic resonance imaging (MRI)- This procedure is conducted by using a magnetic field and pulses of radio wave energy to take pictures of the body. This procedure allows the doctor to check the blood flow between the right ventricles and lung arteries.

Pulmonary function test- This is a non-invasive test that checks the capacity of your lungs by measuring how much air it can hold. This procedure is done by asking you to blow into a simple instrument called a spirometer.

Polysomnogram- This procedure is conducted while you are asleep to observe the brain activity, heart rate, oxygen level and blood pressure level.

Ventilation/perfusion (V/Q) scan- A VQ scan is usually performed to identify blood clots that cause symptoms of pulmonary hypertension. This procedure is conducted by injecting a tracer into veins of the arm which tracks the air and blood flow into the lungs.

Open-lung biopsy- An open-lung biopsy is very rarely recommended by doctors to obtain a small sample of tissue from the lungs under general anaesthesia. This procedure is conducted to check the cause of secondary pulmonary hypertension.

Pulmonary Hypertension Treatment:

There is no cure for pulmonary hypertension, however, there are treatments recommended to ease tension from pulmonary arteries to decrease the progress of pulmonary hypertension. The treatment methods keep changing depending on the effectiveness of medicines given. Treatments are complicated and patients will require thorough check-ups by their doctors to observe changes in the heart rate or blood flow pattern. Some of the treatments that ease tension from pulmonary hypertension are-

Blood vessel dilators (vasodilators)- Medicines such as epoprostenol (Flolan, Veletri) are commonly prescribed to dilate narrowed blood vessels in pulmonary hypertension. However, the effects of epoprostenol do not last more than a few minutes, hence it is constantly injected in between intervals through an IV catheter via a small pump covered in a pack that can be worn on your belt or shoulder. This medicine does have some side effects such as nausea, leg cramps, diarrhoea and jaw pain.

Iloprost (Ventavis)- This is another form of vasodilators that helps in easing pulmonary hypertension. This medicine needs to be inhaled six to nine times a day through a nebulizer to help the medicine reach the lungs directly. The side effects of this drug are headache, nausea, chest pain and breathlessness.

Treprostinil (Tyvaso, Remodulin, Orenitram)- This drug can be inhaled, injected or orally consumed 4 times a day. Its side effects include nausea, headache and diarrhoea.

Endothelin receptor antagonists- These drugs help in the backward effect of endothelin by increasing energy levels. Endothelin is a substance found in blood vessels that narrows the walls of blood vessels. This drug cannot be consumed during pregnancy and continuous intake can lead to liver damage. Regular observation of liver function is required every month.

Sildenafil and Tadalafil- Sildenafil (Revatio, Viagra) and tadalafil (Cialis, Adcirca) are rarely used to increase blood flow to the lungs by breaking blocks in the blood vessels. These drugs include side effects such as headache, vision problems and stomach upset.

Soluble guanylate cyclase (SGC) stimulator- Soluble guanylate cyclase help in clearing the pulmonary arteries by easing the pressure inside the arteries. This happens when the drug responds to nitric oxide in the body. This drug causes dizziness and nausea sometimes and should not be taken by pregnant women.

Anticoagulants- Anticoagulant such as Warfarin may be prescribed by doctors to avoid the formation of blood clots inside the small pulmonary arteries. This drug interferes with normal blood coagulation process and causes internal bleeding. Hence, it cannot be taken very often. Arrange for regular blood check-ups and increase intake of herbal supplements and other food that ease the effects of Warfarin.

Digoxin- Digoxin (Lanoxin) helps in pumping out more blood by increasing stronger heartbeats. This also controls heart rate during arrhythmias.

Oxygen- Doctors recommend inhalation of pure oxygen to patients suffering from pulmonary hypertension living in higher altitudes. This treatment is known as oxygen therapy.

Surgeries such as Atrial Septostomy and Transplantation is required when medications fail to work.

Atrial Septostomy is conducted by surgeons to create more space between the upper left and right chambers of your heart called atria to ease tension on the right side of the heart. However, Atrial Septostomy may have serious complications that can show abnormalities in heart rhythm.

Transplantation is recommended in younger people suffering from idiopathic pulmonary arterial hypertension, where only the lung or both heart and lung is transplanted. The patient's body can show rejection to transplantation or cause serious infection leading to severe complications. If the transplant surgery is successful, immunosuppressive drugs are prescribed for life to avoid the chances of rejection in future.

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6.How to Adapt your Lifestyle

There are no medical treatments available to cure pulmonary hypertension, however, the medications mentioned above can ease symptoms and discomforts caused by pulmonary hypertension. Along with modern medicines, one has to make healthy changes to their lifestyle to improve the disorder.

Do not overstrain- Take adequate rest, do not overstrain the body by lifting heavy objects or exercising too much. By resting your body often it reduces the heartbeat and eventually releases pressure from pulmonary arteries.

Walk to Maintain a Healthy Heart- Walking helps in maintaining a healthy heart, however in case of pulmonary hypertension brisk walking is not allowed. You can take a stroll at the park or walk slowly in the neighbourhood with portable oxygen cans. This has shown some beneficial results.

Quit Smoking- Smoking causes severe damage to the lungs. In the case of pulmonary hypertension, the condition of lung and heart worsens due to smoking which leads to death. Consult a doctor to prescribe nicotine supplements or other exercises programmes that can help you quit smoking as soon as possible.

Avoid pregnancy and birth control pills- Pulmonary hypertension in women during the childbearing age can cost pregnancy. Bearing a child with pulmonary hypertension can cause severe damage to the mother and the baby which is fatal. Intake of birth control pills complicates the condition of pulmonary hypertension as these pills increase the chances of blood clots that interrupts the blood flow. Use different options for birth control and consult a gynaecologist if you get pregnant for better suggestions.

Avoid travelling by flight and living at higher altitudes- Travelling and living at altitudes higher than 8000 feet can worsen the condition of pulmonary hypertension. Relocating to lower altitudes are recommended by doctors.

Avoid exposure to warm climates- Exposure to warmer and hot temperature reduces blood pressure which may lead to fainting episodes and even death in case of pulmonary hypertension. Avoid taking hot showers for long, avoid sitting in a hot tub or sauna at all costs.

Follow a healthy diet chart- Eat a balanced diet with all healthy nutrients that can help you feel energetic. Your meal should consist of fruits and vegetables, low-fat dairy products and lean meat. Do not eat food that contains saturated fat, trans fat and cholesterol as they increase fat deposits in blood vessels. Also, reduce the intake of salt and maintain a healthy weight.

Do not take unprescribed medicines- Always take medicines suggested by your doctor. If you have heard about a new medicine to ease symptoms of pulmonary hypertension from a friend or a well-wisher, please consult your doctor before taking them.

Clear your doubts during follow-ups- Do not shy away from clearing your doubts about discomforts faced during pulmonary hypertension. Do mention the occurrence of unexpected side effects to your doctor during follow up appointments. By doing this you can work on improving your quality of life.

Get vaccinated- People living with a pulmonary hypertension condition are more prone to diseases such as influenza and pneumonia. Your doctor will advise you to get vaccinated against these diseases to avoid severe complications.

Ask for help or support- Ask for help from your family and friends and also speak to a psychological counsellor for help in coping with stress and anxiety. Participate in activities arranged by support groups to help ease your stress levels.

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7.Stages of Pulmonary Hypertension

Pulmonary hypertension is divided into four stages depending on the severity and symptoms faced by the patients.

Class 1 - Symptoms are not evident at this stage and does not stop you from performing regular activities.

Class 2 - The symptoms occur lightly during physical activities and no symptoms are observed during the resting period.

Class 3 - You are unable to perform your regular activities at this stage as you get exhausted very easily.

Class 4 - Symptoms are more evident and severe at this point where even normal activities are performed with great difficulty. Signs of exertion are present even during the resting period. Most often right-sided heart failure occurs during this stage.

What are the Survival rates and Pulmonary Hypertension Prognosis?

Pulmonary hypertension life expectancy is unknown as there is no treatment found yet. However, individuals suffering from it can prolong their lifespan by making the necessary lifestyle changes. It is a disease that gets progressively worse over time, hence timely intervention is absolutely necessary to improve quality of life and avoid any life threatening complications.

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8.Pulmonary Hypertension FAQs: All your concerns addressed.

Q. How will pulmonary hypertension affect my life?

It is a known fact that pulmonary hypertension stops you from exerting energy through regular physical activities over time. It is important to listen to your doctor and follow the instructions and medications given to you. Taking rest is the prime requirement of the body, take an adequate amount of rest and do not overstrain your body. Pulmonary hypertension can bring difficulties into every part of your life from work, travel and exercise. It can be dealt with good coordination and a great deal of patience.

Q, What questions should I be asking my medical team?

Do not hesitate to clear all your doubts. Keep your list of questions ready before meeting your doctor, your questions should probably involve-

  • How often should I visit the doctor?
  • What are the side effects of my medication?
  • What are the changes should I bring in my daily activities and what should I eat?
  • How do I need to alter my daily routine to reduce the risk of complications?

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