Do you also have narrowed blood vessels in your lungs? Let's talk about Pulmonary Arterial Hypertension (PAH)!

Do you sometimes feel a little short of breath? Or do you feel unusually tired even when walking a short distance? Have you ever noticed that your fingertips and lips turn blue along with some tightness or pain in your chest? Don't dismiss these as just normal things. Because sometimes, behind these symptoms, there may be a health condition that needs some attention. One such condition is Pulmonary Arterial Hypertension (PAH) . Although the name may sound a little scary, let's talk about it simply and in a way that you can understand.

What is Pulmonary Arterial Hypertension (PAH)? Simply put...

Simply put, Pulmonary Arterial Hypertension (PAH) is a condition in which the small blood vessels in your lungs (pulmonary arteries) become narrowed. Think of it like a water pipe that is blocked, and it is difficult for the water to flow through it. When these blood vessels become narrow, the blood cannot flow properly through your lungs. This causes the pressure inside these blood vessels, or blood pressure, to become too high . These blood vessels are the ones that carry oxygen-poor blood from your heart to your lungs, where it is cleaned.

So what happens when this happens? The right side of your heart has to work harder than usual to pump blood to the lungs. It's like lifting a heavy weight and climbing a mountain. When the heart has to carry this extra load over time, it gradually starts to weaken. If not treated properly, this condition, PAH, can cause a series of problems not only for your heart and lungs, but also for your entire body.

Remember, PAH is just one specific form of a larger category called Pulmonary Hypertension . Pulmonary Hypertension is an increase in pressure in the blood vessels of the lungs due to any cause.

How does PAH affect your body?

Pulmonary Arterial Hypertension (PAH) mainly puts pressure on the right side of your heart. This is because this part of the heart pumps oxygen-poor blood to the lungs. Over time, this high pressure can lead to right-sided heart failure .

Not only that, PAH also slows down the flow of blood between the heart and lungs. This means that less blood is getting to the lungs to get fresh oxygen. As a result, less oxygen is getting to the other organs and tissues in your body. In fact, every part of our body needs oxygen to survive. If it doesn't get enough, problems can arise.

How serious is this condition called PAH?

Pulmonary Arterial Hypertension (PAH) is a very serious, sometimes life-threatening condition . However, if it is diagnosed early and treated promptly, you can live a longer, better life. That's why it's important to pay attention to the symptoms.

Who is most affected by PAH?

PAH can affect adults of any age. However, it is more common in women , who are usually diagnosed between the ages of 30 and 60. It has also been found that men over the age of 65 who develop PAH may have a higher risk of developing the disease.

This condition can also affect young babies. When it occurs in newborns, we call it Persistent Pulmonary Hypertension in the Neonate (PPHN) .

How common is PAH?

Compared to other types of pulmonary hypertension, such as pulmonary hypertension caused by heart disease or lung disease, PAH is not a very common condition . Even in a country like the United States, only 500 to 1,000 new cases of PAH are diagnosed each year. In Western countries, the incidence is about 25 per million.

What are the symptoms of Pulmonary Arterial Hypertension (PAH)?

You may not experience any symptoms in the early stages of PAH. Most people only start to experience symptoms as the disease progresses. Some of these symptoms include:

• Blue discoloration of the fingertips or lips: As if the blood is lacking oxygen.
• Chest pain or tightness: As if someone is squeezing your chest.
• Dizziness or fainting: This can occur when blood flow to the brain is reduced.
• Fatigue: Feeling extremely tired , not just tired.
• Racing or pounding heartbeat.
• Shortness of breath: This increases over time. Even doing small tasks can cause shortness of breath.
• Swelling (Edema): Swelling may begin first in the legs and ankles, then spread to the abdomen and neck.

If left untreated, these PAH symptoms will get worse over time. You may find yourself unable to perform even the most basic daily tasks without having to catch your breath or rest.

What are the causes of Pulmonary Arterial Hypertension (PAH)?

The main cause of PAH is damage to the lining of the blood vessels in your lungs. However, it's not always clear what causes this damage.

Cases where no cause can be found (Idiopathic PAH)

Sometimes, no specific cause can be found . We doctors call it “idiopathic” pulmonary arterial hypertension . It's like a mystery.

Known causes

However, at other times, there are clear causes. Some of the known causes of PAH include:

• Some medical conditions:
• Congenital heart disease
• Glycogen storage diseases
• HIV infection
• Liver disease
• Autoimmune diseases such as lupus
• Portal hypertension (increased pressure in the blood vessels leading to the liver)
• Pulmonary capillary hemangiomatosis (abnormal growth of capillaries in the lungs)
• Pulmonary veno-occlusive disease (blockage of veins in the lungs)
• Schistosomiasis (an infection caused by a parasite)
• Scleroderma (thickening of the skin and connective tissue)

• Genetic mutations:
• Most often, this is due to a mutation in your BMPR2 gene . This gene controls the number of cells in some tissues in our body. So, when this gene is mutated, cells in the small blood vessels in your lungs can grow too much. When these cells fill up, the openings in the blood vessels become narrow.
• PAH can run in families. We call it heritable PAH . About 80% of people with this condition have mutations in the BMPR2 gene.
• Some people carry this altered gene, but they never develop PAH.
• Some people can develop PAH even though no one in their family has it, due to a genetic mutation. This is called sporadic PAH .

• Some medicines and drugs:
• Some diet pills , such as the old drug “fen-phen,” are now banned, but can still cause PAH years after use.
• Recreational drugs , for example cocaine and methamphetamine.

How is Pulmonary Arterial Hypertension (PAH) diagnosed?

PAH can be difficult to diagnose because the symptoms of PAH are similar to those of many other diseases. Your doctor will do a physical exam and talk to you about your symptoms and medical history. They will do a number of tests (including imaging tests and blood tests) to find out if you have pulmonary hypertension and, if so, what type it is.

Your doctor may also refer you to a pulmonologist or cardiologist . These specialists will perform special tests to check the function of your heart and lungs. They will determine whether you have PAH or another type of pulmonary hypertension. They will also assess how severe your condition is.

There are specific treatments for PAH that are not available for other types of pulmonary hypertension. Therefore, your medical team needs to know as much as possible about what is happening in your lungs and heart. This information will help them develop the best treatment plan for you.

What tests are used to diagnose PAH?

Your medical team will use a combination of tests to rule out other diseases and diagnose PAH.

If your doctor suspects you may have PAH after your physical examination, the first test they will order is a transthoracic echocardiogram . This test evaluates the overall structure and function of your heart.

Other tests may include:

• Blood tests: Check organ function, hormone levels, and identify underlying diseases. Especially tests like the complete metabolic panel and complete blood count .
• Chest CT scan: To check for and rule out other conditions such as kidney disease. (Perhaps the kidney disease in the original article should be lung disease. A CT chest scan looks at the lungs and chest organs. Let's say we're looking at the condition of the lungs.
• Chest X-ray: To see if your heart or pulmonary arteries are larger than normal.
• Heart MRI: Assess the condition of your heart's right ventricle (this is the chamber of the heart that pumps blood to the lungs).
• Polysomnogram (PSG): Checks for sleep apnea, which can worsen PAH. A PSG is a type of sleep test that is performed overnight.
• Pulmonary function tests: See how well your lungs are working.
• Pulmonary ventilation/perfusion (VQ) scan: Checks for blood clots in the lungs. This test can rule out a condition called chronic thromboembolic pulmonary hypertension .
• Right heart catheterization: Measures the pressure in the blood vessels in your lungs (pulmonary artery pressures). This test is essential to definitively diagnose PAH.
• Six-minute walk test: See how much exercise you can tolerate and how much oxygen circulates in your blood when you move.

Talk to your doctor about what tests you need to have and how to prepare for them.

What are the criteria for diagnosing Pulmonary Arterial Hypertension?

Doctors diagnose pulmonary hypertension by measuring the pressure in the blood vessels in your lungs. The diagnostic criteria are that the pulmonary arterial pressure in your lungs is greater than 20 millimeters of mercury (20 mmHg) when you are at rest . This value is measured by right heart catheterization .

What are the treatments for Pulmonary Arterial Hypertension (PAH)?

The main goals of treatment for PAH are to control the progression of the disease and to give you a better quality of life. There is no one-size-fits-all treatment for PAH. Your doctor will work with you to determine the best treatment for your specific needs.

Your PAH treatment plan may include:

• Balloon atrial septostomy (BAS): This procedure is usually done for babies with congenital heart defects, but is sometimes used for adults with PAH. A septostomy helps reduce pressure on the right side of the heart, allowing more oxygen to circulate in the blood. It can also be done as a bridge to a lung transplant.
• Calcium channel blockers: These drugs help lower blood pressure in the blood vessels of your lungs and throughout your body.
• Diuretics: Also known as "water pills," these drugs help remove excess fluid from the body and reduce swelling.
• Oxygen therapy: This treatment may be needed if you don't have enough oxygen in your blood. Extra oxygen can help when you're at rest, sleeping, or exercising.
• Pulmonary vasodilators: These drugs help the blood vessels in your lungs (pulmonary arteries) relax and open wider. This reduces the strain on your heart and helps relieve your symptoms.

For some people with severe PAH , a lung transplant may be the last option. This surgery can give you one or two new lungs. A heart-lung transplant can also give you a new heart.

What are the medications given for PAH?

Medications to treat PAH come in different forms:

• Oral medications: These medications help relax blood vessels in the lungs and prevent them from narrowing. These medications can also help you be physically active.
• Inhaled medications: Treat breathing difficulties.
• Portable infusion pump: This can help open up blood vessels and increase blood flow. This may help improve your symptoms.
• Intravenous (IV) medications: Open up blood vessels and relieve symptoms such as chest pain and difficulty breathing.

Here are some medications that the US Food and Drug Administration (FDA) has approved for PAH patients:

• Ambrisentan (oral)
• Bosentan (oral)
• Epoprostenol (IV)
• Iloprost (inhalation)
• Macitentan (oral)
• Riociguat (oral)
• Selexipag (oral)
• Sildenafil (oral)
• Tadalafil (oral)
• Treprostinil (oral, inhalation, pump, IV)

Side effects of PAH medications

Some common side effects of medications used to treat PAH include:

• Feeling dizzy or faint.
• Feeling of warmth in the face, neck, or hands (flushing).
• Gastrointestinal symptoms such as bloating, nausea, vomiting, and diarrhea.
• Headache (headache).
• Low blood pressure (Hypotension) .
• Swelling of the legs and ankles (Pedal edema) .
• Skin rash.
• Feeling of upper respiratory tract congestion.

Certain medications may have additional side effects. Talk to your doctor about how to manage any side effects. In some cases, your doctor may need to change the dosage of your medication.

Can Pulmonary Arterial Hypertension (PAH) be completely cured?

Although current medications can control the progression of PAH, they cannot reverse the damage that has already occurred . However, researchers are investigating new drugs that they think may reverse PAH. Such drugs can repair the damage to the endothelial cells that line the blood vessels in your lungs.

Talk to your doctor to learn more about the latest research and clinical trials for PAH treatments.

What factors increase a person's risk of developing Pulmonary Arterial Hypertension (PAH)?

Several factors that increase the risk of developing PAH are:

• Connective tissue disease .
• Down syndrome .
• Family history of pulmonary hypertension.
• HIV infection.
• Using weight loss drugs such as “fen-phen” (dexfenfluramine and phentermine).
• Use of illegal drugs (street drugs).

If someone in your family has PAH, ask your doctor about genetic testing. If you are diagnosed with PAH, it is a good idea to ask your family to consider genetic testing as well.

How to prevent Pulmonary Arterial Hypertension (PAH)?

We can't control some risk factors for PAH (such as genetic mutations). However, staying away from illegal drugs can reduce your risk of not only PAH, but many other health problems. Also, talk to your doctor before taking any weight loss medication.

Depending on your risk factors, your doctor may recommend preventive screenings for PAH.

What is the future outlook for people with Pulmonary Arterial Hypertension (PAH)?

With advances in treatment, people with PAH are living longer than ever before. Your life expectancy depends on many factors, including the severity of your condition and how early you are diagnosed. Talk to your doctor about your specific condition.

The most important thing is to continue your treatment and follow your doctor's instructions carefully. There are a few things you can do to improve your chances of survival:

• Make an emergency kit. There are certain supplies and information you should always have with you. Ask your doctor what to include in your kit, and never leave home without it.
• Get seasonal vaccines as recommended by your doctor. It is very important to protect yourself from flu and pneumonia.
• Keep all follow-up appointments. Regular check-ups are essential to check your lung and heart function and measure the progress of your treatment.
• Take your medications exactly as your doctor prescribes, at the same time every day. Do not change the way you take your medications without your doctor's advice.

What lifestyle changes do I need to make?

Follow your doctor's advice about any lifestyle changes you want to make. In general, here are some tips that are important:

• Avoid hot tubs, saunas, and traveling to high altitudes.
• Consider birth control. Pregnancy can be dangerous for people with PAH. Talk to your doctor if you are planning to become pregnant or if you may become pregnant.
• Exercise and stay active as much as possible. Talk to your doctor about what exercises are safe for you and how much activity you should do each day. Ask your doctor before starting a new exercise program.
• Follow a heart-healthy diet. This includes foods low in saturated fat, trans fat, and sodium.
• Stop smoking and using tobacco products. Also, avoid secondhand smoke.
• Seek support, don't try to go through your PAH journey alone. Ask your doctor about support groups and resources.

When should I call my doctor?

Call your doctor if you have any of these questions:

• If the heart rate is more than 120 beats per minute (fast heart rate).
• If a respiratory infection or cough gets worse.
• If you often feel dizzy or faint.
• If you experience chest pain or discomfort with physical activity.
• If you experience extreme fatigue or a decrease in your ability to perform normal tasks.
• Nausea or loss of appetite.
• Restlessness or confusion.
• If your shortness of breath gets worse, especially if you feel short of breath when you wake up.
• If swelling in your ankles, legs, or abdomen gets worse.
• Difficulty breathing during normal activities or at rest.
• Weight gain (2 pounds per day or 5 pounds per week).

When should I go to the Emergency Treatment Unit (ETU) ?

If you have any of these things, go to the emergency room or call your local emergency number:

• If the heart rate (120-150 beats per minute) does not decrease.
• Fainting, causing loss of consciousness.
• Complications from your IV or infusion pump (such as infection, catheter dislodgement, solution leakage, bleeding, IV pump malfunction).
• If the shortness of breath does not go away even after resting.
• Sudden and severe chest pain.
• A sudden and severe headache.
• Sudden weakness or paralysis of the arms or legs.

Finally, a message to keep in mind

Pulmonary Arterial Hypertension (PAH) is a life-changing diagnosis. If you or a loved one has been newly diagnosed with this disease, take the time to learn about the condition and what you can expect. Ask your doctor about resources that explain how PAH works and how treatments can help.

One of the most valuable resources you can have is the support of others in your life. Don't try to manage this condition alone. If your family and friends aren't around, ask your doctor about support groups and community resources.

If your loved one has been diagnosed with this disease, do everything you can to make them feel supported and help them connect with others. Illness can make us feel isolated in some ways, but it can also bring us closer together. Use this time to build a community that can help you or your loved one move forward.

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