Have you ever seen someone in your family or someone you know suddenly faint and collapse while exercising or when they are very scared? Sometimes, behind such things lies a heart condition that we have not heard much about, but which can be very serious. One such condition is Long QT Syndrome (LQTS). Although the name may sound a bit complicated, let's understand it simply.
What is Long QT Syndrome (LQTS)?
Simply put, Long QT Syndrome (LQTS) is a problem with your heart's electrical system.
Think of your heart as a flash on a camera. After it beats once, it needs to be charged a little before it can beat again. That's how the heart's electrical system needs to recharge between beats. The time it takes for this to happen is what we measure on an ECG (Electrocardiogram) as the QT interval .
This recharge time is longer for someone with LQTS than for someone who is normal. This means that the heart's electrical system takes longer to prepare for the next beat.
How does this happen?
The electrical activity of our heart is controlled by tiny electrically charged particles called ions (for example, sodium, potassium, calcium). These ions move in and out of heart muscle cells through tiny gates called ion channels .
In Long QT Syndrome, these ion channels either don't work properly or they are missing enough. This causes the heart to recharge too late.
When the QT interval is prolonged in this way, a very dangerous, life-threatening, rapid, irregular heartbeat (arrhythmia) can occur. In medical terms, we call this Torsades de Pointes . This is a form of a dangerous condition called Ventricular Tachycardia .
What are the main types of LQTS?
LQTS can be divided into two main types: inherited and acquired.
1. Inherited/Congenital LQTS
This is caused by a genetic defect. That means it is inherited from parents to children. Here too, there are several main types.
| Congenital type of LQTS | A simple explanation |
|---|---|
| Romano-Ward Syndrome | This is the most common type. In this case, one of the parents may have LQTS. There is a 50% chance that a child will inherit the condition. Hearing (hearing) in these people is normal. |
| Jervell and Lange-Nielsen Syndrome | This is very rare. In this case, both parents are carriers of the LQTS gene (they may not have symptoms). There is a 25% chance that a child will inherit the condition. Children with this condition are often born deaf. |
| Timothy Syndrome | This is also a very rare type. It can affect not only the heart, but also other parts of the body. |
2. Acquired LQTS
This is not caused by genetic factors. Some people may be born with a slight weakness in their ion channels. But that is not a problem. However, this condition can arise due to some external factor.
The main reasons are:
- Certain medications: Some antibiotics, antidepressants, antiarrhythmics, and diuretics.
- Decreased body salt levels: Decreased levels of potassium, magnesium, or calcium in the blood.
- Other causes: Eating disorders like anorexia nervosa, damage to the nervous system, and certain toxins.
What could these symptoms be?
The most dangerous thing about LQTS is that about 50% of people do not experience any symptoms . However, when symptoms do occur, they are often serious. These symptoms occur when the previously mentioned dangerous heart rhythm pattern called Torsades de Pointes occurs.
| Main symptom | What happens to it? |
|---|---|
| Syncope | Due to the irregular heartbeat, the brain does not receive enough blood supply. This can cause sudden loss of consciousness and collapse. |
| Seizures | A condition called a seizure can sometimes occur due to decreased blood flow to the brain. |
| Cardiac Arrest | If that dangerous heartbeat pattern continues, the heart may stop beating completely. |
| Sudden Death | Cardiac arrest can be fatal if not treated immediately. Sometimes, this is the first symptom of someone with LQTS. |
Situations where symptoms are more likely to appear:
- While exercising or after you finish.
- During a sudden, intense mental stimulation (e.g., when very scared, startled).
- During sleep or when waking up suddenly from sleep.
Who is at higher risk of developing this condition?
The following people are at higher risk of developing LQTS:
- Close blood relatives (parents, siblings, children) of someone with LQTS.
- People with a family history of unexplained memory loss, seizures, or sudden death at a young age.
- Children who are born deaf (due to the risk of Jervell and Lange-Nielsen Syndrome).
- People with other heart diseases like cardiomyopathy.
- People who use medications that prolong the QT interval.
If someone in your family has been diagnosed with LQTS, it's a good idea to get tested for it. First, tell your doctor about it. He or she will refer you for an ECG test.
How do you know if you have LQTS?
The main way to diagnose this is through an ECG (Electrocardiogram) . The doctor measures the QT interval on the ECG. Usually, if this interval is longer than 450 milliseconds, LQTS can be suspected.
In addition, the doctor may recommend several other tests:
- Blood tests: Check the levels of salts in the body, such as potassium and magnesium.
- Exercise Stress Test: See how your heart's function changes during exercise.
- Ambulatory Monitor (Holter Monitor): A small device is placed on your chest to continuously record your heart rhythm for 24 hours or more.
- Genetic testing: Check for genetic mutations associated with LQTS.
What are the treatments for this?
Although LQTS cannot be completely cured, there are treatments that can help control symptoms, prevent the risk of sudden death, and lead a normal life.
| Treatment method | Description |
|---|---|
| Medications | Many people (even those without symptoms) are given beta-blocker medications. These help control the heart rate a bit and reduce the heart's response to sudden stimuli. |
| Devices |
|
| Surgery | For high-risk patients who are difficult to control with medications and devices, a surgery called Left Cardiac Sympathetic Denervation (LCSD) is performed. This involves removing some of the nerves that supply the heart, reducing the heart's response to sudden stimuli. |
Things to consider when living with LQTS
It is possible to live a normal, full life with LQTS. However, there are a few things you need to be aware of in your daily life.
1. Sudden noises and stress
For people with some types of LQTS, sudden, loud noises (e.g., an alarm, a phone ringing) or sudden shock or fear can be dangerous.
- Tell family and friends that it's not safe to tease you.
- Instead of loud alarm clocks, use ones that play gentle music.
2. Exercise and sports
For people with some types of LQTS, competitive sports and strenuous exercise can be particularly risky.
- Be sure to ask your doctor what exercises are appropriate and inappropriate for you.
- Swimming is especially dangerous for some people. There is a risk of drowning if you lose consciousness in the water. Do not go swimming alone.
3. Be especially careful with medications!
This is the most important thing. Even many common medications can make LQTS worse.
- Tell every doctor who treats you that you have LQTS.
- Before prescribing medication for any other condition, talk to your cardiologist.
- Do not buy any over-the-counter medicine from a pharmacy or anywhere else without consulting a doctor (except for things like plain paracetamol and aspirin).
When should I see a doctor?
Once you've been diagnosed with LQTS, you should see your doctor at least once a year for a check-up. It's best to see an electrophysiologist, a doctor who specializes in heart rhythm disorders.
If your child has this condition, you will need to see the doctor frequently as the dosage of medication needs to be adjusted according to their weight.
When should I go to the ETU (Emergency Treatment Unit)?
If someone is unconscious and falls, call an ambulance immediately. In the meantime, if there is someone trained to perform CPR (cardiopulmonary resuscitation), give it. In a situation like this, every second counts.
Take-Home Message
- Long QT Syndrome (LQTS) is a genetic or acquired condition in which the heart's electrical system takes too long to recharge.
- Sudden loss of consciousness, seizures, and unexplained heart failure are the main symptoms. However, many people may not experience any symptoms at all.
- If anyone in your family has this condition or has a history of sudden death at a young age, it is very important that you also have an ECG test.
- Before taking any medication (even for other conditions), talk to your doctor to make sure it is safe for you.
- With proper treatment (medication, ICD device) and lifestyle changes, someone with LQTS can live a full, healthy life. There is no need to be afraid, but it is essential to be careful.
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