Description

Long QT syndrome (LQTS) is a heart rhythm disorder that cause fast, chaotic heartbeats. The irregular beats of the heart can be deadly. The LQTS affects the electrical signals that travel through the heart and cause it to beat.

Some people are born with the DNA changes that cause the long-QT syndrome. This is known as congenital long QT syndrome. LQTS may also occur later in life due to some health conditions, certain medications, or changes in the levels of minerals in the body. This is called acquired long QT syndrome.

The long QT syndrome can cause fainting and seizures. Young people with LQTS syndrome have an increased risk of sudden cardiac death.

Treatment for long QT syndrome includes lifestyle changes and medication to avoid the danger of heart beats. Sometimes, a medical device or surgery is necessary.

Symptoms

The most common symptom of long QT syndrome are fainting, also called syncope. A fainting episode of LQTS can happen with little or no warning.

Fainting occurs when the heart beats erratically for a short time. If you are going to faint when you are excited, angry, or scared, or during exercise. If you have LQTS , things that startle you can cause you to pass out, such as a loud ringtone or an alarm clock.

Before fainting happens, some people with long QT syndrome may have symptoms such as:

  • Blurred vision.
  • The dizziness.
  • Pounding heartbeats called palpitations.
  • The weakness.

The long QT syndrome can also cause seizures in some people.

Babies who are born with LQTS may have symptoms during the first weeks or months of life. Sometimes the symptoms start later in infancy. Most of the people who are born with LQTS have symptoms by age 40. The symptoms of long QT syndrome, sometimes happen during sleep.

Some people with long QT syndrome (LQTS) do not notice any symptoms. The disorder may be found during a heart test called an electrocardiogram. Or may be discovered when genetic testing is performed for other reasons.

When to see a doctor

Make an appointment for a health checkup if you faint, or if you feel like you have a pounding or fast heartbeat.

Tell your healthcare team if you have a parent, brother, sister or child with long QT syndrome. The long QT syndrome can run in families, which means that it can be hereditary.

Causes

Long QT syndrome (LQTS) is caused by changes in the heart's electrical signaling system. Does not affect the form or shape of the heart.

To understand the causes of LQTS , it can help to know how the heart beats in general.

In a typical heart, the heart sends blood to the entire body during each beat of the heart. The chambers of the heart to tighten and relax to pump blood. The heart's electrical system controls this coordinated action. The electrical signals called impulses move from the top to the bottom of the heart. They say that the heart when to squeeze and beat. After each beat of the heart, the charging system to prepare for the next beat.

But in the long-QT syndrome, the electrical system of the heart takes longer than usual to recover in between beats. This delay is called a prolonged QT interval.

The long QT syndrome, usually falls into two groups.

  • Congenital long QT syndrome. You are born with this type of LQTS . It is caused by changes in the DNA that is passed through families. That means that it is hereditary.
  • Acquired long QT syndrome. This type of LQTS is caused by another health condition or medication. Usually can be reversed when the specific cause is found and treated.

Causes of congenital long QT syndrome

Many of the genes and their changes have been linked to long QT syndrome (LQTS).

There are two types of congenital long QT syndrome:

  • Romano-Ward syndrome. This most common type that occurs in people who get only a single change in the gene of one of the parents. The reception of a mutation of one of the parents is known as an autosomal dominant pattern of inheritance.
  • The syndrome of Jervell and Lange-Nielsen syndrome. This rare form of LQTS usually occurs very early in life, and that is serious. Children with this type of LQTS are also deaf. In this syndrome, the children receive the change in the gene from both parents. This is called a pattern of inheritance is autosomal recessive.

Causes of the acquired long QT syndrome

A medicine or other health condition can cause acquired long QT syndrome.

If a drug that causes acquired long QT syndrome, the disorder can be called as a drug-induced long QT syndrome. More than 100 drugs can cause prolonged QT intervals in healthy people. Medications that can cause LQTS include:

  • Some antibiotics such as erythromycin (Eryc, Erythrocin, others), azithromycin (Zithromax), and others.
  • Some antifungal medications used to treat yeast infections.
  • Water pills, also called diuretics, which cause the body to eliminate the excess of potassium or other minerals.
  • Heart rhythm medicines called anti-arrhythmics, you can do that, the QT interval is longer.
  • Some medicines used to treat mental health conditions such as anxiety and depression.
  • Some medicines used to treat an upset stomach.

Always tell your healthcare provider about all the medicines you are taking, including those bought without a prescription.

Health conditions that can cause acquired long QT syndrome include:

  • The body temperature below 95 degrees Fahrenheit (35 degrees Celsius), a condition that is called hypothermia.
  • Low level of calcium, also called hypocalcemia.
  • Low magnesium, also called hypomagnesemia.
  • Low potassium, also called hypokalemia.
  • A tumor of the adrenal gland that is usually not cancer, called pheochromocytoma.
  • Stroke or brain bleed.
  • Underactive thyroid, also called hypothyroidism.

Risk factors

Things that may increase the risk of long QT syndrome (LQTS), which include:

  • A history of a cardiac arrest.
  • Having a parent, brother, sister or child with long QT syndrome.
  • The use of drugs that are known to cause prolonged QT intervals.
  • The people who are assigned female at birth who take certain medicines for the heart.
  • A lot of vomiting or diarrhea, which can cause changes in the body, and minerals such as potassium.
  • Eating disorders such as anorexia nervosa, which can also cause changes in the levels of minerals in the body.

If you have long QT syndrome and wish to become pregnant, tell your health care professional. Your care team carefully controls during pregnancy to help prevent the things that can trigger LQTS symptoms.

Complications

Usually after an episode of long QT syndrome (LQTS), the heart returns to its normal rhythm. But the sudden cardiac death can occur if the heart rhythm is not corrected quickly. The heart rhythm can be reset by your account. Sometimes, it is necessary for a treatment to restore the heart rhythm.

The complications of long QT syndrome may include:

  • Torsades de pointes ("twisting of the points").This is a life-threatening rapid heartbeat. The two lower chambers of the heart beating fast and out of rhythm. The heart pumps less blood. The lack of blood in the brain cause sudden fainting, often without notice. If a long QT interval is prolonged for a long time, fainting may be followed by a full body seizure. If the pace dangerous is not corrected herself, then put in danger the life of arrhythmia called ventricular fibrillation in the following way.
  • Ventricular fibrillation. This type of irregular heart beat causes the lower chambers of the heart to beat so fast that the heart trembles and stops the pumping of the blood. Unless a device called a defibrillator is quickly used to correct heart rhythm, brain damage and death can occur.
  • The sudden cardiac death. This is the swift and it is not expected that the elimination of all heart activity. The long QT syndrome has been linked to sudden cardiac death in young people who otherwise appear healthy. LQTS may be responsible for some of the unexplained events in children and young adults, such as unexplained fainting, drowning, or seizures.

Torsades de pointes ("twisting of the points"). This is a life-threatening rapid heartbeat. The two lower chambers of the heart beating fast and out of rhythm. The heart pumps less blood. The lack of blood in the brain cause sudden fainting, often without notice.

If a long QT interval is prolonged for a long time, fainting may be followed by a full body seizure. If the pace dangerous is not corrected herself, then put in danger the life of arrhythmia called ventricular fibrillation in the following way.

Appropriate medical treatment and lifestyle changes can help to prevent the complications of long QT syndrome.

Prevention

There is No known way to prevent congenital long QT syndrome (LQTS). If someone in your family has LQTS , ask a health care professional if genetic screening is right for you. With proper treatment, you can monitor and prevent the dangerous beating of the heart, which can lead to LQTS complications.

Regular medical checkups and good communication with your health care professional may also help prevent the causes of some types of acquired long QT syndrome. It is especially important not to take medicines that may affect the heart rhythm and cause a prolonged QT interval.

Diagnosis

To diagnose long QT syndrome (LQTS), a professional of the health examination. Normally you have questions about your symptoms and medical and family history. The healthcare provider listens to your heart with a device called a stethoscope that is placed against his chest. If your healthcare provider thinks that you have an irregular heartbeat, you may perform tests to check the heart.

Tests

Tests are done to check the health of the heart and to confirm the long-QT syndrome (LQTS).

Electrocardiogram (ECG or EKG)

An ECG is the most common test used to diagnose long QT syndrome. It records the electrical signals in the heart, and shows how fast or how slow the heart is beating. Sticky patches called electrodes attached to the chest and sometimes the arms and legs. The wires connect the electrodes to a computer, that prints or displays the results of the test. The heart of the signals are shown as the waves on the results of the test.

  • In an ECG , there are five waves. They use the letters P, Q, R, S and T.
  • Q-waves through T show the heart of signaling in the lower chambers of the heart.
  • The time between the start of the Q wave and the end of the T wave is called the QT interval. This is the time that it takes for the heart to contract and refill with blood before beating again. If the range takes longer than usual to spend, what is called a prolonged QT interval.

Ideal QT interval depends on your age, your sex and your personal heart rate. In people who have LQTS complication known as torsade de pointes, the waves of the ECG results look braided.

If long QT syndrome symptoms do not occur often, they can not be seen in a normal ECG . If so, your health care professional may ask you to wear a heart rate monitor in your home. There are several different types.

  • Holter Monitor. This small portable ECG device that records the heart's activity. It is used for a day or two while you do your regular activities.
  • Event recorder. This device is as a Holter monitor, but the records only at certain times for a few minutes at a time. It is typically used for about 30 days. Normally push of a button when you feel symptoms. Some devices automatically record when an irregular heart rhythm is detected.

Some personal devices, such as smartwatches, have sensors that can take an ECG . Ask your health care professional if this is an option for you.

Testing effort

These tests often involve walking on a treadmill or pedaling a stationary bike. A member of your care team, check the activity of your heart while you exercise. Tests of effort to show how the heart reacts to physical activity. If you can't do the exercise, you can receive a drug that increases the heart rate as you exercise. Sometimes an echocardiogram is done during a stress test.

Genetic testing

A genetic test is available to confirm the long-QT syndrome (LQTS). The test checks for changes in genes that can cause the disorder. Check with your insurance company to see if it is covered.

If you have long QT syndrome, your health care team may suggest that other members of the family also get genetic testing to check for the disorder.

Genetic testing for long QT syndrome can not find all the legacy of the cases of long QT syndrome. Families are encouraged to talk with a genetic counselor before and after the test.

Treatment

Treatment for long QT syndrome (LQTS) may include:

  • Life-style changes.
  • Drugs.
  • A medical device.
  • Surgery.

The objectives of LQTS treatment are:

  • Avoid irregular heart beats.
  • Prevent sudden cardiac death.

Your healthcare provider will talk to you about your treatment options. The treatment depends on the symptoms and the type of long QT syndrome. You may need treatment, even if they have no symptoms.

Therapies

Some people with acquired long QT syndrome may receive fluids or minerals, such as magnesium, through a needle into a vein.

Drugs

If the medicine causes of long QT syndrome (LQTS), stopping the drug may be all that is needed to treat the disorder. Your health care professional can tell you how to do it safely. Do not change or stop taking any medicine without talking to your health care team.

Some people with LQTS you need drugs to treat the symptoms and prevent life-threatening changes in heart rhythm.

Drugs used to treat long QT syndrome may include:

  • Beta-blockers. These drugs slow the heart rate. To reduce the chances of having a long QT episode. Beta-blockers are used to treat long QT syndrome include nadolol (Corgard), and propranolol (Inderal LA, InnoPran XL).
  • Mexiletine. Taking this medicine for the heart rate with beta-blocker may help to shorten the QT interval. You can help reduce the risk of fainting, seizures, or sudden cardiac death.

Surgery or other procedures

Some people with long QT syndrome require surgery or a device to control the heartbeat. Surgery or other procedures that are used for LQTS, the treatment may include:

  • To the left sympathetic denervation heart (LCSD) of the surgery. This surgery can be done if you have long QT syndrome, and continuous changes in the heart rhythm, but the beta-blockers does not work for you. It doesn't cure the long QT syndrome. In contrast, the surgery helps to reduce the risk of sudden cardiac death. In this treatment, the surgeons remove specific nerves along the left side of the spine. These nerves are part of the body of the sympathetic nervous system, which helps to control the rhythm of the heart.
  • An Implantable cardioverter defibrillator (ICD).This device, which is placed under the skin near the collarbone. Constantly checks the heart's rhythm. If the device finds an irregular heartbeat, sends low-or high-energy shock to reset the heart's rhythm. Most people with long QT syndrome do not need to anICD. But the device can be suggested by some athletes to help them to return to competitive sports. The decision to place anICD, especially in children, should be carefully considered. The placement of anICDrequires of the surgery. Sometimes, the device may send disturbances that are not needed. Talk with your health care team about the benefits and risks of anICD.

An Implantable cardioverter defibrillator (ICD). This device, which is placed under the skin near the collarbone. Constantly checks the heart's rhythm. If the device finds an irregular heartbeat, sends low-or high-energy shock to reset the heart's rhythm.

Most people with long QT syndrome do not need an ICD . But the device can be suggested by some athletes to help them to return to competitive sports. The decision to place an ICD , especially in children, should be carefully considered. The placement of an ICD requires surgery. Sometimes, the device may send disturbances that are not needed. Talk with your health care team about the benefits and risks of ICD .

Lifestyle and home remedies

Your health care professional may suggest lifestyle changes to help you manage the long-QT syndrome (LQTS). These changes can reduce the risk of an episode of fainting or sudden cardiac death associated with LQTS .

  • Know what sports are safe. You may be able to remain fully active in sports, including competitive sports. But talk with your health care team first. In general, people with long QT syndrome should never swim alone. Physical activities are likely to be good if you take someone who can help in case of fainting.
  • Check amazing sounds. Turn down the volume of the ringtones and other devices, such as phones that may startle you, especially during sleep. Things that startle you can cause you to faint.
  • Control of the emotions. Very excited, angry, or surprise can cause a fast heartbeat, changes in some people with long QT syndrome. Do more exercise, the practice of mindfulness and connecting with others in support groups are some of the ways to reduce stress.
  • Review your medicines. Do not take medicines that may cause prolonged QT intervals. If you are not sure if you take these medicines, talk to your health professional. It is a good idea to let your healthcare team know about all of the medications and supplements you are taking, even those that you buy without a prescription.
  • Get health checkups. If you have changes in your symptoms or health in general, your health care team may upgrade your plan of treatment or suggest treatments.

Coping and support

Worry about the possible dangerous heart rhythms associated with long-QT syndrome (LQTS) can cause stress for you and your loved ones. Here are some things that can help you manage your condition.

  • Tell other people haveLQTS. Make family, friends, teachers, neighbors, and anyone else who has regular contact with you conscious of your heart rhythm disorder and its symptoms. Wear a medical alert id to show others that you have LQTS .
  • Have an emergency plan. The members of the family may want to learn cardiopulmonary resuscitation (CPR) so that they can help if you ever need it. It may be appropriate to have or be able to quickly get an automated external defibrillator (AED).
  • Seek support or advice. It might be helpful to join a support group, where you can share your experiences and feelings with other people who are familiar with the long-QT syndrome. Families who have inherited long QT syndrome can also be helpful to talk with a genetic counselor.

Preparing for your appointment

If you palpitations, fast or irregular heartbeat, make an appointment for a health checkup. You may be sent to a doctor trained in heart disease. This type of health care professional is called a cardiologist.

You can also see a doctor trained in heart rhythm disorders, called an electrophysiologist.

Here's some information to help you prepare for your appointment and know what to expect from your health care team.

What you can do

  • Write down any symptoms you've had, and for how long. Include those that do not appear to be associated with the long QT syndrome.
  • List important medical information, including any other health condition you have and the names and doses of the medications that you take. It is also important to share all of the family history of irregular heartbeat or sudden death with your health care professional.
  • Write down the questions that you want to be sure to ask your health care professional.

Questions for the health care professional at your first appointment are:

  • What is likely causing my symptoms?
  • There are other possible causes of these symptoms?
  • What tests do I need?
  • You should see a specialist?

Questions to ask if you are sent to a cardiologist or electrophysiologist include:

  • I have the long QT syndrome? If so, what kind?
  • What is my risk of complications?
  • What treatment do you recommend it?
  • If you are recommending medications, what are the possible side effects?

Questions to ask if your healthcare provider recommends the surgery include:

  • How does this type of surgery help me?
  • Where should I have my surgery?
  • What should I expect from my recovery and rehabilitation after surgery?

Other questions are the following:

  • I need frequent check-ups of health and long-term treatment?
  • The emergence of the symptoms of long QT syndrome should be taken into account?
  • What activity restrictions I need to follow?
  • What kind of lifestyle changes are recommended?
  • What medicines should I avoid?
  • What is my long-term prognosis with treatment?
  • It is safe for me to become pregnant in the future?
  • What is the risk of my future children have long QT syndrome?
  • How can genetic counseling help my family?

Do not hesitate to ask other questions.

What to expect from your doctor

Your health care team may ask:

  • What are your symptoms?
  • When did the symptoms begin?
  • The symptoms worsened over time?
  • Do strong emotions, such as anger, excitement, or surprise, trigger your symptoms?
  • Does exercise cause symptoms?
  • Do you shivered as, for example, the doorbell or the telephone ringing — trigger your symptoms?
  • Do you ever feel dizzy or light-headed?
  • Have you ever fainted?
  • Have you ever had a seizure?
  • What other medical conditions do you have?
  • Does anyone in your family have heart disease or a heart rhythm disorder?
  • He has a father, mother, brother, sister or son has died by drowning or an unexpected cause?
  • What medications are you taking?
  • Have you ever used illegal drugs? If so, what?
  • Do you use caffeine? How much?

Know as much as possible about your health and the health of your family health history helps your health care team to learn of your diagnosis and treatment plan.

What you can do in the meantime

While you wait for your appointment, ask your family members if someone is related to family history of long QT syndrome or unexplained death.

Symptoms and treatment of the Long QT syndrome