Is there a problem with your heart's electrical system? Let's talk about Long QT Syndrome (LQTS).

Have you ever heard of the electrical system of the heart? Our heart works like a small machine. It has an electrical system, which controls the heartbeat. Sometimes, small changes can occur in this electrical system. One such change is called Long QT Syndrome, or `(Long QT Syndrome - LQTS)`. Don't worry, let's talk about this simply.

What is Long QT Syndrome? Simply put...

Imagine that after your heart beats once, it takes a little "rest" before it can beat again, like a battery recharging. The time it takes to "rest" or recharge is a little longer than normal, which is what we call long QT syndrome.

If you've had an ECG, which is a test that looks at the electrical activity of the heart, there's a part of it called the QT interval. This shows how long it takes for the lower chambers of your heart, called the ventricles, to contract and then relax, or "recharge" as I said.

The electrical activity of our heart is aided by small electrically charged particles called `ions`. Ions like sodium, calcium, potassium, and chloride contribute to this. These ions move in and out of the heart cells through special pathways called `ion channels`. Now, in this `(Long QT Syndrome)`, either there is a problem with these ion channels, or there is a lack of them. Then the heart is delayed in "recharging". When the QT interval is prolonged, there is an increased risk of a dangerously fast heart rhythm disorder called `Torsades de Pointes`, which can be life-threatening.

Are there types of Long QT Syndrome?

Yes, there are two main types. One is inherited , which means it is passed down through genes. The other is acquired , which means it is caused by other factors, such as certain medications.

Congenital types:

These are mainly caused by genetic defects in the `ion channels` I mentioned.

• Ion channel abnormalities: This is the most common type. There are several types such as `LQT1, LQT2, LQT3, LQT4, LQT5`. These are classified according to the type of ion channel involved. The risk of future heart disease may vary depending on the type.
• Jervell and Lange-Nielsen Syndrome: In this condition, both parents can carry the abnormal gene, but they may not have symptoms. This is a very rare condition. Because it is unlikely that both parents will carry the gene, each child has a 25% chance of inheriting it. People with this syndrome may be born deaf.
• Romano-Ward Syndrome:In this case, one parent usually has the ``Long QT Syndrome'' and the other does not. Each child has a 50% chance of inheriting this abnormal gene. In this case, hearing is normal.
• Timothy Syndrome: This is also a very rare type. It can affect not only the heart, but also other parts of the body.

How common is this condition?

Long QT Syndrome is actually a rare condition . For example, in the United States, it is estimated that approximately one in 2,000 people have this condition.

What are the symptoms of Long QT Syndrome?

These are the symptoms that are often seen:

• Syncope: Sudden loss of consciousness.
• Seizures: Some people may experience seizures.
• Cardiac arrest: This is very dangerous.
• Sudden death:

These symptoms occur when the dangerous heart rhythm disorder called `Torsades de Pointes` that I mentioned earlier occurs. At this time, the heart is unable to pump blood properly. If the brain does not receive enough blood supply, things like fainting and seizures occur. If this disorder continues, sudden death can occur. However, if the heart rate returns to normal, the symptoms stop.

Symptoms are most likely to appear when:

• While exercising (or a few minutes after exercising).
• During strong emotional stimulation , especially when suddenly scared or startled.
• During sleep or when suddenly waking up .

Importantly, up to 50% of people with Long QT Syndrome may never develop symptoms . And, for about one in ten people, cardiac arrest is the first sign of the condition. This can be fatal if not treated quickly.

Symptoms usually begin in early adulthood . If you have symptoms, they may recur. Symptoms are most likely to appear before the age of 30. However, some people may develop symptoms in their 50s and beyond.

What causes Long QT Syndrome?

As I mentioned before, this can be inherited from your parents (congenital) or acquired due to certain medications . In Long QT Syndrome, there is an abnormality in the gene code for your ion channels. These abnormal ion channels cause a delay in the recovery phase of your heart rate. This can lead to a life-threatening arrhythmia.

Some medications can cause ``Long QT Syndrome'' in people who have a certain predisposition to their ion channels. They may not even know it, but it is when they take those medications that ``Long QT'' occurs.

Drugs that can cause acquired long QT syndrome:

• Some `Antiarrhythmics` (medicines for heart rhythm disorders)
• Some antidepressants
• Some diuretics (medicines that make you urinate more)
• Some antibiotics
• Serotonin receptor inhibitors or antagonists
• Histamine receptor antagonists

Other causes of acquired long QT syndrome:

• Some types of poisons
• Anorexia nervosa (mental illness related to anorexia)
• Some `bradyarrhythmias` (abnormally slow heart rate)
• Low levels of calcium, magnesium, or potassium in the body
• Injury to your nervous system

Who is at higher risk of developing this condition?

The following people are at higher risk of developing ``Long QT Syndrome``:

• For children who are deaf from birth.
• Children and young people with a family history of unexplained sudden death or fainting (syncope).
• For blood relatives with ``Long QT Syndrome``.
• For those with `Cardiomyopathy` (heart muscle disease) or congenital heart disease.
• For those who have low levels of certain electrolytes in the body due to another medical condition.
• For those taking medications that affect ion channels in the heart.

Should I also be tested for Long QT Syndrome?

Long QT Syndrome is a genetic condition that can be passed down from generation to generation. If one of your first-degree relatives (parents, siblings, children) has Long QT Syndrome, it is a good idea for you to undergo genetic screening .

All first-degree relatives (siblings, parents, and children) should have an ECG. It is also important for anyone with a family history of seizures or fainting to have this test.

The first step is to let your doctor know that your family has this condition. He or she will likely do the necessary tests to check your heart. If those tests confirm that you have this condition, you should see a cardiologist who is well-versed in Long QT Syndrome.

What are the complications of Long QT Syndrome?

``Long QT Syndrome`` can cause the life-threatening arrhythmia ``Torsades de Pointes`` that I mentioned. This can even lead to sudden death.

How is Long QT Syndrome diagnosed?

Doctors can diagnose Long QT Syndrome with a simple ECG test. To diagnose the condition, they measure the QT interval on the ECG. If your QT interval is longer than 450 milliseconds, you may have Long QT Syndrome.

Your doctor will ask you about these things:

• Is there a family history of ``Long QT Syndrome''?
• Is there a family history of unexplained fainting, seizures, or cardiac arrest?
• Have you ever had fainting, seizures, or cardiac arrest, especially during exercise?

What tests are used to diagnose Long QT Syndrome?

In addition to the ECG, these tests may also be performed:

• Blood tests.
• Genetic testing.
• Exercise stress test.
• Ambulatory monitor: This monitors your heart function for several days.

How is Long QT Syndrome treated?

There are medications, devices, or surgeries that can help control symptoms and prevent sudden death for Long QT Syndrome. While these treatments cannot completely cure the condition, they can protect you from abnormal heart rhythms.

Medications

Many people with Long QT Syndrome (even those without symptoms) take a beta-blocker, such as nadolol. Other medications can shorten your QT interval or improve your electrolyte levels. Your doctor will tell you which medications are best for you.

Devices

Some patients with ``Long QT Syndrome'' may also need devices like these:

• Implantable Cardioverter Defibrillator (ICD): An implantable cardioverter defibrillator (ICD) may be implanted in people who have a history of cardiac arrest or who are still symptomatic despite beta-blocker treatment. This device detects life-threatening arrhythmias and automatically delivers a shock to the heart to prevent sudden death.
• Pacemaker: This device helps people with abnormally slow heart rates.

Surgery

Some patients with Long QT Syndrome may need to undergo Left Cardiac Sympathetic Denervation (LCSD) or sympathectomy. This is a minimally invasive procedure in which certain nerves in your sympathetic nervous system are removed.

What are the complications/side effects of treatment?

Possible side effects or complications depend on the type of treatment you receive.

• Beta-blocker:
• Low blood pressure.
• Dizziness.
• Headache .
• Fatigue.
• ICD (Implantable Cardioverter Defibrillator):
• Device errors.
• Collapsed lung.
• Bleeding.
• Infections.
• Pacemaker:
• Collapsed lung.
• Bleeding.
• Infections.
• Device errors.
• LCSD (Left Cardiac Sympathetic Nerve Decompression Surgery):
• Horner syndrome.
• Collapsed lung.
• Facial flushing or sweating.

Can Long QT Syndrome be prevented?

You can't prevent it if you inherit it from your parents. However, for those who don't inherit it, you can prevent the conditions that cause acquired (long QT syndrome) later on. For example, be careful with certain medications.

What happens if I have Long QT Syndrome?

With proper treatment , the mortality rate for people with ``Long QT Syndrome`` is about 1%. Without treatment, the situation is dire. About 21% of people with symptoms who do not receive treatment die within a year of the onset of syncope.

But remember, people with ``Long QT Syndrome'' can live full lives . They can conceive and give birth to children.

How long does Long QT Syndrome last?

Inherited Long QT Syndrome is a lifelong condition. However, symptoms and the risk of complications may decrease over time.

How do I take care of myself?

Long QT Syndrome can affect many aspects of your daily life. You should consult your doctor before doing certain things.

Noise and stress

Some people with Long QT Syndrome may be affected by sudden loud noises or emotional stress. While you can't control all the noise and stress around you, you can try to limit them.

• Tell your friends and family that it's not safe to suddenly wake you up.
• Instead of a loud alarm that goes off all at once, use an alarm that has a pleasant sound.
• Turn down the volume of loud things that might startle you.

Exercise

Some types of Long QT Syndrome are more likely to cause problems with exercise. Your doctor will tell you what activities are appropriate for you, depending on the type of abnormal gene you have. Because exercise can cause fatal arrhythmias, you should talk to your doctor before you decide to play competitive sports. If you decide to play sports, you may need to take some safety precautions.

Swimming can be dangerous for some people with ``Long QT Syndrome''. If you faint while swimming, you could drown.

Medicines

Many medications can prolong the QT interval. These medications may affect people with ``Long QT Syndrome`` more than those without it. If you have ``Long QT Syndrome``, you should do the following:

• Tell all your doctors that you have ``Long QT Syndrome''.
• Talk to your doctor before taking any medication prescribed for other medical conditions.
• Avoid taking over-the-counter (OTC) medications (except plain aspirin or paracetamol) without talking to your doctor.

If you have ``Long QT Syndrome'', these types of drugs may affect you:

• Antihistamines (medicines for allergies)
• Antidepressants (medications for depression)
• Medication for mental health problems
• Medicines for heart disease
• `Antibiotics`
• Antifungals
• `Antivirals`
• Medicines for intestinal diseases
• Anticonvulsants
• Diuretics (medicines that cause increased urination)
• Medications for high blood pressure
• Medications for migraines
• Cholesterol-lowering drugs

When should I see my doctor?

You should see a doctor when you start having symptoms and at least once a year thereafter. An electrophysiologist is the best option.

If your child has ``Long QT Syndrome'', you should see your doctor more than once a year to make sure they are receiving the correct dose of medication for their current weight.

If you have a device implanted, you should see your doctor once a year to make sure it is working properly.

When should I go to the Emergency Department (ETU) ?

A person in cardiac arrest needs immediate medical attention . Someone nearby should begin CPR (cardiac resuscitation) and call 911 (or your local emergency number).

What questions should I ask my doctor?

Some questions you can ask your doctor:

• What type of ``Long QT Syndrome'' do I have?
• What specific treatment do you recommend for me?
• How often do I need to see you?
• What medications and activities should I avoid with my specific type of ``Long QT Syndrome''?

Finally, a Take-Home Message

It's normal to feel anxious when you find out you have heart disease. But asking your doctor questions and, if necessary, talking to a counselor can help you feel more comfortable. It's important to follow your doctor's instructions, take your medications on time, and keep your appointments.

It can also be a relief to let your family and friends know that you have Long QT Syndrome. If you develop symptoms while they are with you, they can call for emergency help. Even better, if they know how to perform CPR, it could save your life. Don't worry, with proper management, you too can live a healthy life.

` Long QT Syndrome, LQTS, heart disease, QT interval, heart rate, arrhythmia, genetic diseases