Are you aware of Pulmonary Hypertension? Let's talk about it!

Have you ever heard of a disease in which the pressure in the lungs increases? This is a little different from the high blood pressure we usually talk about. Doctors call this `Pulmonary Hypertension` (PH) , or high blood pressure in the lungs. Simply put, this is when the blood pressure, or pressure, inside the veins that carry oxygen-poor blood from your heart to your lungs (we call these `pulmonary arteries`), increases unnecessarily. This is a rather serious condition, because it has a big impact on our heart and lungs.

Why is Pulmonary Hypertension (PH) so dangerous? What happens to our body?

Think about what happens when the pressure inside the veins that carry blood to the lungs increases. Just as when a water pipe is blocked, it makes it difficult for the water to flow, these veins become narrow, sometimes thickening. Then, the heart has to work harder to pump blood to the lungs. The right side of the heart (the `right ventricle`) in particular feels this strain the most. Over time, the heart starts to weaken as it continues to work harder. This condition can also cause various problems in the body. For example:

• Decreased hemoglobin in the blood (` Anemia` ).
• Irregular heartbeat (`Arrhythmias`).
• Blood clots in the veins of the lungs .
• A fluid collection around the heart (` Pericardial effusion `).

If a pregnant woman develops this condition, it can be dangerous for both the mother and the baby. If left untreated, this condition can lead to progressive heart failure (`right-sided heart failure`) and can even be life-threatening. Therefore, it is very important to diagnose the disease at an early stage and start treatment .

What are the main types of Pulmonary Hypertension (PH)?

The World Health Organization (WHO) has divided this `Pulmonary Hypertension` (PH) condition into five main categories according to the causes that cause it. Let's see what they are. 1. Category 1: PH caused by `Pulmonary Arterial Hypertension (PAH). In this, the blood vessels that carry blood to the lungs (`pulmonary arteries`) become narrowed, thickened, or stiffened. This can have various causes. Some medical conditions, even some medications, can affect this. 2. Category 2: PH caused by problems with the left side of the heart. The left side of our heart is the one that pumps blood to the whole body. So, if there is a problem with the left side of the heart, it affects the right side and the system that carries blood to the lungs. The blood does not flow properly and becomes blocked, and the pressure in the pulmonary veins increases. 3. Category 3: PH caused by lung disease or lack of oxygen (`hypoxia`).Some lung diseases cause the blood vessels in the lungs to narrow. This causes blood flow to be obstructed, and the pressure to rise. 4. Category 4: PH caused by obstructions in the lungs. Blood clots in the lungs or scarring from them obstruct the flow of blood. This puts more strain on the right side of the heart, increasing blood pressure in the lungs. 5. Category 5: PH caused by other diseases. This PH condition can occur with other diseases, such as some blood diseases and metabolic disorders. However, it is not yet clear exactly how these diseases cause PH.

Who is most affected by this condition `Pulmonary Hypertension` (PH)?

Pulmonary Hypertension (PH) can affect adults of any age. People with heart or lung disease are at a higher risk of developing it. It can also be more common in people with other conditions. For example:

• Almost 100% of people with severe mitral valve disease.
• About 65% of people with aortic valve disease (another heart valve disease).
• About 30% of people with `Scleroderma` (a disease of hardening of the skin and connective tissue ).
• About 20% - 40% of people with sickle cell disease.
• About one in 200 people infected with HIV .

It usually affects adults. However, very rarely, newborn babies can also develop this condition (`Persistent Pulmonary Hypertension of the Newborn - PPHN`). If this happens, the baby will need to be treated in the intensive care unit. Worldwide, it is estimated that about one in 10 adults over the age of 65 has this PH condition. This means that it may be more common than we think.

What are the early symptoms of Pulmonary Hypertension (PH)?

Often the first symptom is shortness of breath, or wheezing . You may experience this shortness of breath during normal daily activities, such as climbing stairs, carrying groceries, or exercising. You may not have any symptoms at first. When symptoms do appear, they may be very mild. However, over time, these symptoms can become more severe and make it difficult to do your normal activities.

What are the symptoms that appear as the disease progresses?

As your pH level increases, you may experience shortness of breath even when you are just standing still. This may be accompanied by symptoms such as:

• Blue lips or skin.
• Chest pain or tightness.
• Feeling dizzy or faint.
• Feeling tired all the time (`Fatigue `).
• The food is tasteless.
• Pain in the upper right side of the stomach.
• Feeling like your heart is beating fast.
• Swelling of the ankles, legs, or abdomen (`Edema`).

These symptoms make it very difficult to exercise and perform daily tasks.

Are there stages of Pulmonary Hypertension (PH)?

Yes, the World Health Organization (WHO) has identified four main stages of PH. These are called "functional classes." These are determined by the symptoms you experience and how much you can do your daily activities. As the disease progresses, the symptoms become more pronounced and interfere with your daily life.

• Class 1: No symptoms.
• Class 2: No symptoms at rest, but some discomfort or pain during normal activities, such as doing household chores or climbing stairs.
• Class 3: You may still feel fine when you're just sitting around. But it's very difficult to do normal tasks now. You're too tired and stressed.
• Class 4: Symptoms are present even when just standing still. Symptoms become worse when you try to do any normal activity.

What are the causes of Pulmonary Hypertension (PH)?

There are many different causes of PH. It depends on the type of PH you have. Various illnesses, underlying health conditions, and even environmental factors (toxins, certain medications) can cause it.

Category 1: Causes of `Pulmonary Arterial Hypertension (PAH)`

• Congenital heart disease .
• Some weight loss medications (e.g., the drug "fen-phen," which can cause PAH even after years of use).
• Genetic mutations .
• `Glycogen storage diseases`.
• HIV infection.
• Liver disease .
• Lupus disease.
• Portal hypertension (increased pressure in the veins leading to the liver).
• Rare pulmonary vein diseases such as `Pulmonary capillary hemangiomatosis` and `Pulmonary veno-occlusive disease`.
• Schistosomiasis (a disease caused by a parasite).
• Scleroderma disease.
• Use of drugs such as `Methamphetamine`.

Some people can develop PAH without an obvious cause. This is called `idiopathic`.

Category 2: Causes of PH caused by disorders of the left side of the heart

Heart disease is a common cause of PH. Because the left and right sides of the heart work together, a problem on the left side will affect the right side as well.

• Aortic valve disease: This is a problem with the valve between the heart's main pumping chamber (the `left ventricle`) and the aorta (the body's largest blood vessel).
• Left-sided heart failure : The left side of the heart is unable to pump blood properly.
• Left ventricular hypertrophy (thickening of the walls of the left ventricle) : This makes it difficult for the heart to pump blood efficiently.
• Mitral valve disease : A problem with the valve that allows blood to flow from the left atrium to the left ventricle of the heart.

Category 3: Causes of PH caused by lung disease or lack of oxygen (`hypoxia`)

• Chronic obstructive pulmonary disease (COPD) : This includes diseases such as chronic bronchitis and emphysema. These make it difficult to breathe.
• Interstitial lung disease : Scarring (fibrosis) occurs in the lung tissue.
• Obstructive sleep apnea : This causes the airway to become blocked periodically during sleep, reducing the amount of oxygen the body receives.

Category 4: Causes of PH due to obstruction in the lungs

This is mainly caused by a condition called `Chronic Thromboembolic Pulmonary Hypertension (CTEPH) .` This is when blood clots form and scar tissue in the veins of the lungs. Sometimes, this condition can be caused by scarring caused by `pulmonary embolism` (a blood clot that gets stuck in the lungs).

Category 5: Causes of PH caused by other diseases

PH can be caused by a number of other diseases, and scientists still don't fully understand how it happens.

• Gaucher disease (a metabolic disease).
• Kidney disease.
• Langerhans cell histiocytosis (a disease that causes scarring and tumors in the lungs of adults).
• Sarcoidosis (an inflammatory condition, most commonly affecting the lungs and lymph nodes).
• Thyroid disease.
• Tumors : Tumors, whether cancerous or benign, can cause pressure on the veins in the lungs.

How is Pulmonary Hypertension (PH) diagnosed?

Your doctor will examine you and, if necessary, perform several tests to determine whether you have PH. First, your doctor will do a physical exam . There,

• They ask about your health history and symptoms.
• Check for jugular venous distention (this can be a sign of right-sided heart failure).
• To check the size of the liver, feel the upper right side of the abdomen.
• The heart and lungs are listened to with a stethoscope.
• Check the abdomen, ankles, and legs for swelling (edema).
• Blood pressure is measured.
• A pulse oximeter measures the oxygen level in the blood.

PH symptoms can be difficult to diagnose because they are similar to those of other conditions. Therefore, after a physical examination, your doctor may perform additional tests. You may also be referred to a lung specialist (pulmonologist) or a heart specialist (cardiologist).

What tests are used to diagnose PH?

• Right heart catheterization: Also called pulmonary artery catheterization, this measures the pressure in the pulmonary veins and the amount of blood the heart can pump per minute. This is the main test to confirm PH.
• Doppler echocardiogram: This is a scan using sound waves. It can show how the right ventricle of the heart is working and how blood flows through the valves. This allows the doctor to calculate the systolic pulmonary artery pressure.
• Blood tests: These check for things like organ function, hormone levels, and infections. These include tests like the ``Complete metabolic panel`` and ``complete blood count.``
• Chest CT scan: Checks for blood clots and other lung problems.
• Chest X-ray: This can be used to see if the right side of the heart or the pulmonary veins are larger than normal.
• Sleep test (`Polysomnogram - PSG`): An overnight test to see if you have `sleep apnea`.
• Pulmonary ventilation/perfusion (VQ) scan: Checks for blood clots in the lungs.

Your doctor may also perform a six-minute walk test . This will measure how much exercise you can tolerate and how much oxygen is in your blood during exercise. This can give you an idea of ​​whether your PH is mild or severe.

What are the treatments for Pulmonary Hypertension (PH)?

Treatment for PH depends on the type of PH you have and your other health conditions. Your medical team will develop a treatment plan that is best for you. Currently, there are only two types of PH that can be treated directly:

• ``Pulmonary Artery Hypertension (PAH)''
• ``Chronic Thromboembolic Pulmonary Hypertension (CTEPH)''

Other types of PH are treated by controlling the underlying medical conditions that cause them. Treatment for `Pulmonary Arterial Hypertension (PAH):

• Calcium channel blockers: These medications help reduce blood pressure in the pulmonary veins and throughout the body.
• Diuretics ("water pills"): Help remove excess fluid from the body.
• Oxygen therapy: This may be necessary if there is not enough oxygen in the blood.
• Pulmonary vasodilators:These medications relax the pulmonary veins and open them up better. This improves blood flow and reduces the workload on the heart.

Treatment for `CTEPH`:

• Anticoagulants (medicines that prevent blood clotting).
• Balloon atrial septostomy (BAS): Although this is usually done for babies with serious heart defects, it is also used to stabilize adults with PH until a lung transplant can be obtained.
• Balloon pulmonary angioplasty (BPA): This is a procedure that uses a catheter to widen the pulmonary arteries. It is done for people who cannot undergo major surgery.
• Medication: A medication called `Soluble guanylate cyclase stimulator (SGCS)` can help control the progression of the disease.
• Pulmonary endarterectomy (PEA): This is a surgery. It removes blood clots from the lungs. This is currently the only way to completely cure PH. However, it can only be done in people with CTEPH.

PH caused by heart or lung disease is treated by controlling the underlying disease, so treatment plans can vary greatly from person to person.

Remember, you need to talk to your doctor and decide what treatment is best for you.

Some people with severe PH may need to undergo a lung transplant as a last resort.

How to manage medications for Pulmonary Hypertension (PH)?

You may be taking multiple medications for PH and other conditions. It can be difficult to remember all of this and do it properly. Here are a few tips:

• Know the names of all the medicines you take and how they work. Make a list and always keep it with you.
• Take your medicine at the same time every day. If you miss a dose, do not take two doses to make up for it. Call your doctor and ask what to do.
• Do not take any over-the-counter medications without consulting your doctor. Some cold, cough, and fever medications, as well as pain relievers, can cause problems for people with heart disease.
• Do not take any medication that is labeled as not suitable for people with high blood pressure.
• Do not stop taking any medication or change the dosage without talking to your doctor.
• Avoid using herbal supplements and herbal teas. They may interact with some medications you are taking.

What is the outlook for someone with Pulmonary Hypertension (PH)?

This varies from person to person. It depends on:

• The cause of PH.
• How quickly the disease was diagnosed.
• Severity of symptoms.
• Other concomitant diseases.

Many types of PH cannot be cured completely. Doctors prescribe medications to reduce symptoms, improve quality of life, and control the progression of the disease. However, some people with CTEPH can be cured with surgery. If left untreated, Pulmonary Hypertension (PH) can lead to right-sided heart failure and eventually death. Therefore, getting the right treatment can help you live longer and maintain a good quality of life.

What are the things that increase the risk of developing Pulmonary Hypertension (PH)?

• Exposure to asbestos.
• Having a family history of blood clots.
• Having a family member with Pulmonary Hypertension (PH).
• Living at high altitudes above sea level.
• Smoking and use of tobacco products.
• Some medications used for weight loss (e.g. "fen-phen" - `dexfenfluramine` and `phentermine`).
• Some medications for cancer and depression.
• Drug use.

Also, people with these conditions are at higher risk:

• Blood clots in the veins of the lungs.
• Connective tissue disease.
• `Down syndrome`.
• Gaucher disease.
• Heart disease.
• `HIV`.
• Liver disease.
• Lung diseases.

Can Pulmonary Hypertension (PH) be prevented?

PH cannot always be prevented. Some risk factors are beyond our control. If you have risk factors, your doctor may recommend certain tests to check your heart and lung function. You can reduce your risk of PH by doing everything you can to prevent or control other medical conditions.

• Make an exercise plan. Ask your doctor what exercises are safe for you.
• Follow a heart-healthy diet. Avoid foods high in salt, oil, sugar, and artificial foods.
• Stop smoking and tobacco use. These are major risk factors for heart and lung disease.
• Take medications for blood pressure and other diseases exactly as prescribed by your doctor.

What kind of follow-up care should I receive?

Talk to your doctor about how to manage and monitor your condition. Your doctor will tell you when you need to come in for tests and check-ups. Tell your doctor right away if you develop any new symptoms or if your symptoms change. Also, talk to your doctor about:

• Family planning methods and whether pregnancy is safe. Pregnancy is risky for people with PH.
• Build an emergency kit. People with PH need to keep certain items and information close at hand at all times.
• Exercise.Ask what's safe for you, how to incorporate exercise into your daily life, and what exercises to avoid.
• Seasonal vaccines (such as flu, pneumonia).
• Support groups or counseling. As you adjust to living with PH, you may experience a variety of emotions.
• Travel plans. You may need to take special precautions if you are traveling by plane. You should also be careful when traveling to high altitudes.

What dietary changes should I make?

Your doctor will give you specific recommendations. One important thing is to reduce your salt (sodium) intake. This means:

• Stop adding salt at the table and using "seasoning salt."
• Avoid smoked, salted, and canned meats and fish.
• Choose foods that are labeled "low sodium" (low salt).
• Limit fast foods and take-out food.

Other dietary changes:

• Eat more fiber-rich foods (whole grains, bran, fruits, vegetables).
• Eat foods rich in potassium (raisins, bananas, oranges).
• Eat foods rich in magnesium (peanuts, tofu, broccoli).
• Limit foods containing refined sugar, saturated fat, and cholesterol.

When should I see the doctor?

Talk to your doctor if you have any of these questions:

• Rapid heartbeat (more than 120 beats per minute).
• If you have a respiratory infection or a worsening cough.
• If you often feel dizzy or faint.
• Chest pain or discomfort with physical activity.
• Extreme fatigue or inability to perform normal tasks.
• Nausea or loss of appetite.
• Restlessness or confusion.
• If you have increased shortness of breath, especially when you wake up in the morning.
• If there is increased swelling in the ankles, legs, or abdomen.
• If you have difficulty breathing during normal activities or even just standing still.
• Weight gain (about 1 kilo in a day, or about 2.5 kilos in a week).

When should I go to the emergency room (ER)?

If something like this happens, go to the emergency room immediately, or call 1990:

• If the heart rate is too fast (120-150 beats per minute) and does not slow down.
• If you lose consciousness and faint.
• If you are taking intravenous medications such as prostacyclins through a Hickman catheter, there are risks such as infection, catheter movement, medication leakage, bleeding, or IV pump failure.
• If the shortness of breath does not decrease even when you just stand still.
• If you suddenly experience severe chest pain.
• If you suddenly get a severe headache.
• If you suddenly experience weakness or numbness in your limbs.

Finally, the most important message

Being diagnosed with Pulmonary Hypertension (PH) can be a confusing experience. It takes time to understand what it is, what is happening inside your body, and how you will live with it going forward. Talk to your doctor to get the support you need. Involve your family and friends in your lifestyle changes. Let them know about your condition. Also, as you adjust to this new normal, never hesitate to ask for help and rely on others. You are not alone. You can manage this condition well with proper medical advice and a positive attitude. Pulmonary Hypertension, PH, Pulmonary Hypertension, Heart Disease, Lung Disease, Breathing Difficulty, Respiratory Disease