Has your heart changed like this? Let's talk about Hypertrophic Cardiomyopathy (HCM)!

Have you ever heard of a heart condition where the heart muscle just thickens, a bit complicated? Sometimes you may not even know you have it, because some people don't have any symptoms. But it's important to be aware of it because it affects the heart. Today we're going to talk about this condition called hypertrophic cardiomyopathy, or `HCM' among doctors. Don't worry, we'll talk about it simply, in a way that you can understand.

Let's first see what HCM is?

Simply put, hypertrophic cardiomyopathy (HCM) is a complex condition that affects the muscles of your heart. It can be caused by a few main things:

• Thickening of the heart muscle: Especially the lower chambers of the heart, which we call the ventricles, have thickened walls.
• Left ventricular stiffness: The main chamber on the left side of the heart (left ventricle) stiffens, becoming less elastic, like rubber.
• Mitral valve changes: Changes in the function of the mitral valve, an important valve in the heart, can occur.
• Cellular changes: Certain abnormalities occur in the heart muscle cells (cellular changes).

These things can cause some disruptions in the functioning of the heart.

How does this HCM condition affect your body?

Okay, now let's see what these changes actually do to the body.

Myocardium thickening

This is the main symptom seen in HCM. Most often, this thickening occurs in the wall that separates the left and right sides of the heart, called the `(septum).` Imagine, when this septum between the lower chambers of the heart `(ventricles)` thickens, it can block the way blood flows from the left ventricle to the aorta, the main blood vessel that carries blood throughout the body. We call this `(outflow tract obstruction)`. Then the heart has to work harder to pump blood past this blockage. The type of HCM that blocks the way blood flows is called `(Hypertrophic Obstructive Cardiomyopathy - HOCM)`.

Sometimes other parts of the heart, such as the apex, the right ventricle, or the entire left ventricle, can become thickened. In these cases, the blood flow is not blocked. However, the amount of blood that the ventricle can handle is reduced. This is called Hypertrophic Nonobstructive Cardiomyopathy.

Left Ventricular Stiffness

When the heart muscle thickens, changes in those cells cause the left ventricle to not relax properly and fill with blood. What happens then? Because there is less blood in the chamber, less oxygen-rich blood is sent to the other organs and muscles in the body. This hardening also increases the pressure inside the heart, and you may experience symptoms such as:

• Chest pain
• Shortness of breath
• Dizziness or fainting
• Feeling like your heart is beating fast (palpitations)

Mitral Valve Changes

As mentioned earlier, when the left ventricular outflow tract becomes blocked, the mitral valve in the heart may not function properly. This can obstruct the flow of blood and increase the pressure in the left ventricle.

The cause of this blockage is the mitral valve hitting the thickened septum. When this happens, the mitral valve does not close properly and blood can leak back into the left atrium.

Cellular Changes

If you look closely under a microscope, the heart muscle cells of a person with HCM are not arranged in a straight line, but rather in a somewhat chaotic pattern. This disorder can cause changes in the electrical signals that travel through the lower chambers of the heart, causing an abnormal heart rhythm called ventricular arrhythmia. This can be dangerous.

How common is this condition called HCM?

It is estimated that about 1.5 million people in the United States alone, or about one in every 500 people, have HCM. This is more common than a disease like ``(Multiple Sclerosis)''. So, it is not that rare.

At what age does HCM usually develop?

Most of the time, this heart disease appears in young people, around the age of eighteen or twenty. However, it can start at any age.

If I have HCM, is there a risk of getting pregnant?

You may need special medical care, such as regular echocardiography. However, you will most likely have a healthy pregnancy and a normal delivery. However, it is important to discuss this with your doctor before you become pregnant and discuss the risks. Your doctor will also tell you which medications for HCM you can continue to take during pregnancy. You may also need to have a pacemaker or implantable cardioverter defibrillator (ICD) fitted if necessary during pregnancy.

What causes HCM?

There are several factors that can cause HCM:

• Genetics: This is the main cause. HCM is a disease that can be inherited from parents to children. This means that there is a defect in the genes that control the characteristics of the heart muscle. There are several types of genes that cause HCM. Even if there is a genetic defect, not everyone in the family will develop the disease in the same way. Some people may have the HCM gene but never develop the disease.
• High blood pressure: High blood pressure that has been present for a long time can also be a cause of this.
• Aging: There is a possibility of this condition occurring as you age.
• Unexplained cases: Sometimes, no specific cause can be found for HCM.

What symptoms might someone with HCM experience?

Some people with HCM may experience a variety of symptoms, while others may have no symptoms at all. The most common symptoms are:

• Chest pain: This usually occurs during exercise or exertion, but can sometimes occur while just standing or after eating.
• Shortness of breath and fatigue: These symptoms, especially when you are tired, are common in older adults. This is due to increased pressure in the left atrium and lungs.
• Fainting or fainting (syncope): This can be caused by abnormal heart rhythms (arrhythmias) or abnormal responses of blood vessels during exercise. Sometimes, no cause can be found.
• Palpitations: This can be caused by an abnormal heart rhythm, such as atrial fibrillation (atrial flutter) or ventricular tachycardia (ventricular tachycardia). About 25% of people with HCM have atrial fibrillation. This increases the risk of blood clots and heart failure.
• Swelling of the veins in the lower body or neck.

What other health problems can be associated with HCM?

Many people with HCM live normal lives and don't have any major health problems. Some don't even need to take medication. However, even if you don't have symptoms, your doctor may advise you not to do strenuous exercise.

However, some people with HCM may develop heart conditions that shorten their lifespan or reduce their quality of life. The main ones are:

Sudden Cardiac Arrest and Sudden Cardiac Death

Sudden cardiac arrest is a sudden loss of heart function due to a dangerously fast heart rate (e.g., Ventricular Tachycardia). In this case, emergency treatment, such as CPR and defibrillation, is required as soon as symptoms begin. If this treatment is not given properly, sudden cardiac death can occur.

Most people with HCM have a low risk of sudden cardiac death. However, HCM is the leading cause of sudden cardiac death in people under the age of 35. You may have heard that HCM has also been linked to the sudden deaths of some young professional athletes.

Heart Failure

Just because you have `(Heart Failure)` doesn't mean your heart has "failed" and stopped working. It just means your heart is not pumping blood properly and efficiently. Symptoms of `(Heart Failure)` include:

• Shortness of breath.
• Feeling tired when active (fatigue).
• Swelling of the ankles, legs, and abdomen.
• Needing to urinate at night while resting.
• Dizziness, confusion, difficulty concentrating, fainting.
• Feeling like your heart is beating fast (palpitations).
• A dry, hacking cough.
• Feeling full, bloating, loss of appetite, or stomach pain.

How to diagnose HCM? (Diagnosis)

HCM is diagnosed based on the following:

• Medical history: Your doctor will ask you about your symptoms and whether anyone in your family has had similar illnesses.
• Physical exam: The doctor will listen to your heart and lungs. People with HOCM may hear an abnormal heart sound (heart murmur).
• Tests: An echocardiogram (echo) is the main test used to diagnose HCM. This can help to see if the walls of the heart are thickened.

There are other tests:

• Blood tests.
• Electrocardiogram (ECG).
• Chest X-ray.
• Exercise stress echo test.
• Cardiac catheterization.
• Magnetic resonance imaging (MRI).

What are the treatments for HCM?

Your doctor will decide what treatment is right for you based on the following factors:

• Is there any blockage in the way blood leaves the heart (outflow tract narrowing)?
• How does your heart work?
• Your symptoms.
• Your age and how active you are.
• Do you have abnormal heart rhythms (arrhythmias)?

The main goal of treatment is to reduce or prevent symptoms and reduce the risk of complications such as heart failure and sudden cardiac death.

The following can be done as treatment:

• Identify risks and continue to see a doctor.
• Lifestyle changes.
• Medicines.
• Specialized medical procedures.

How to screen for HCM?

Since HCM is a condition that can be passed down from generation to generation, if one of your parents, siblings, or children (i.e., a first-degree relative) has the disease, it is important to get tested.

The first step is to have your heart examined by an Electrocardiogram (ECG) and an Echocardiogram (echo). If these tests show signs of HCM, you will need to see a doctor who specializes in this condition (cardiologist).

Sometimes, even if you have a family history of HCM, your test results may be normal. If so, doctors recommend that you have an echo and ECG every three years until you are 30, and then every five years.

Who is at higher risk of sudden cardiac death due to HCM?

Most people with HCM have a low risk of sudden cardiac death. However, a small number of people with HCM have an increased risk. It's important to know if you have this condition. Your doctor can tell you what you can do to reduce your risk.

People at higher risk of sudden cardiac death due to HCM are:

• People with a family history of sudden cardiac death.
• People who have had syncope several times in their youth.
• People who have an abnormal blood pressure response to exercise.
• People with a previous history of abnormally fast heartbeats (arrhythmia).
• Those with severe symptoms and poor heart function.

Talk to your doctor about your personal risk. If you have two or more risk factors for sudden death, your doctor may recommend antiarrhythmic medications (medicines that control abnormal heart rhythms) or an implantable cardioverter defibrillator (ICD) to reduce your risk.

What medications are used to manage HCM?

Doctors prescribe various medications to control your symptoms and prevent further complications. Medications like ``Beta blockers`` and ``calcium channel blockers`` relax the heart muscle, helping it fill with blood and pump blood more efficiently. Other medications can control your heart rate or reduce the risk of abnormal heart rhythms (arrhythmias).

You may be told not to take certain medications, for example nitrates (which lower blood pressure) or digoxin (which increases the force of heart contractions).

Sometimes you may need to take antibiotics and take other precautions to reduce the risk of a life-threatening infection called bacterial endocarditis.

What specific medical procedures are used to treat HCM?

There are several methods to treat Hypertrophic Obstructive Cardiomyopathy (HOCM), a condition that causes obstruction of blood flow:

Septal Myectomy

This is a surgical procedure. In a septal myectomy, the surgeon removes a small section of your thickened septal wall. This widens the outflow tract from the left ventricle to the aorta. Doctors only consider this surgery if medication doesn't help. This often resolves the mitral valve problem.

Ethanol Ablation

This is also called `(septal ablation)`. This method is used for people who cannot have the `(septal myectomy)` surgery mentioned earlier. In this, the doctor finds a small coronary artery that supplies blood to the upper part of your septum. Then he inserts a balloon catheter into that artery and inflates it. Next, he injects a dye `(contrast agent)` to find the exact location of the thickened septal wall that is blocking the blood flow.

Once that spot is found, the doctor injects a very small amount of pure alcohol through the catheter. This alcohol kills the cells, and over the next few months the septum shrinks back to its normal size. This widens the path for blood to flow.

Implantable Cardioverter Defibrillator (ICD)

An implantable cardioverter defibrillator (ICD) can help people with life-threatening arrhythmias or sudden cardiac death. An ICD is a small device that is implanted under your skin. It is connected to your heart by wires. The ICD continuously monitors your heart rhythm. When it detects a very fast, abnormal heartbeat, it delivers a small but powerful electrical shock to the heart, causing it to beat normally again. Your doctor will tell you if an ICD is right for you.

Heart Failure Management

There are several ways to manage heart failure, from lifestyle changes to medications that reduce symptoms and help the heart muscle work properly.

How can I improve my quality of life with HCM?

Whether you have symptoms or not, whether you have been diagnosed with HCM, or if someone in your family has it, there are several things you can do to keep your heart health at its best:

• Limit fluids and salt (sodium): If you have symptoms of heart failure, you may need to limit your fluid intake and salt intake. Ask your doctor for specific guidelines about your diet, including alcohol and caffeine.
• Exercise with caution: Your doctor will talk to you about what exercise is right for you. Many people with HCM can do noncompetitive aerobic activities. Doctors do not recommend heavy lifting or high-intensity sports.
• See your doctor regularly: If you have HCM, you should see your cardiologist regularly to monitor your condition.
• Reduce your risk of infection: If you have HCM, your doctor will advise you to take steps to reduce your risk of developing an infection called ``infective endocarditis''.

How can you reduce the risk of developing Infective Endocarditis?

People with HCM are more likely to develop a bacterial infection called ``bacterial endocarditis'' or ``infective endocarditis''.

Bacterial endocarditis is an infection of your heart valves or the lining of your heart (endocardium). It happens when germs (especially bacteria, but sometimes fungi and other microorganisms) enter your bloodstream and attack the lining of your heart or the valves. This can cause the valves to become thickened, perforated, or scarred. This often results in a leaky heart valve. If not treated properly, it can be fatal.

You can take these steps to reduce this risk:

• Take good care of your teeth and gums: See a dentist every six months, brush your teeth daily, floss, and check that your toothpaste fits properly.
• See a doctor if you have symptoms of an infection:
• A fever over 100 degrees Fahrenheit, sweating, or chills.
• Skin rash.
• Pain, tenderness, redness, or swelling.
• A wound or cut that does not heal, or a wound that is red, hot, and oozing pus.
• Sore throat, throat tightness, or pain when swallowing.
• Sinus discharge, nasal congestion, headache, or pain along the upper cheekbones.
• A dry or phlegmy cough that lasts more than two days.
• White spots in the mouth or tongue.
• Nausea, vomiting, or diarrhea.

Don't delay seeking treatment. Common colds and flu don't cause endocarditis. However, other infections with similar symptoms can cause endocarditis. So, be safe and see your doctor.

• Take preventive antibiotics before certain medical and dental procedures. Talk to your doctor about the type of antibiotics you should take, the amount, and for what types of procedures you should take them.
• Keep a bacterial endocarditis identification card available from the American Heart Association.

Finally, this is what you have to say (Take-Home Message)

Most people with HCM live normal, happy lives. There are several treatments available for those who have symptoms or who are at risk of developing serious problems. Your prognosis depends on how well your heart muscle is working, what your symptoms are, and how well you respond to and follow your treatment plan.

Ask your doctor about your risks and what you can do to improve your quality of life and prevent infections. If you are diagnosed with HCM and develop symptoms or suspect an infection, see your doctor right away. Remember, you can live a good life with this condition!

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