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19 / 04 / 2018
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Long QT syndrome (LQTS)


Cardiovascular System


Long QT syndrome (LQTS) is a congenital disorder characterized
by a prolongation of the QT interval on electrocardiogram (ECG). Long QT syndrome (LQTS) is a disorder of the heart's electrical system. The condition leaves you vulnerable to fast, chaotic heartbeats that may lead to fainting - and in some cases, cardiac arrest and possibly sudden death.

You can be born with a genetic predisposition for long QT syndrome. In addition, more than 50 medications, many of them common, as well as electrolyte abnormalities and various medical conditions, may cause the condition.

Treatment for long QT syndrome may involve limiting your physical activity, avoiding certain medications or taking medications to prevent the development of a chaotic heart rhythm. Some people with long QT syndrome also need an implantable cardioverter-defibrillator (ICD) to prevent against sudden death.

Signs and symptoms

Not all people with long QT syndrome experience symptoms. A third or more never develop signs and symptoms and may be aware of their condition only from results of an electrocardiogram (ECG) performed because they have a family history of long QT syndrome or for an unrelated reason.

For people who do experience signs and symptoms of long QT syndrome, the most common signs include:

  • Syncope. This is another word for fainting. In people with long QT syndrome, fainting spells are caused by the heart transiently beating erratically.
  • Seizures. If the heart remains in electrical chaos, the brain becomes increasingly deprived of oxygen. This can cause seizures. In fact, some people with long QT syndrome have been misdiagnosed as having the seizure disorder epilepsy and have been placed on anti-seizure medication.
  • Sudden death. Normally, the heart returns to its normal rhythm. If this doesn't happen spontaneously and paramedics don't arrive in time to convert the rhythm back to normal with an external defibrillator, sudden death can occur.

The main symptom — fainting spells — often occurs during exercise or emotional excitement, such as when you're startled, angry or scared. A person with long QT syndrome may lose consciousness, for example, while playing basketball or after hearing a startling noise, such as an alarm clock going off or a phone ringing.

Signs and symptoms of inherited long QT syndrome may start in the first months of life, or as late as middle age. Most people who experience symptoms from long QT syndrome have their first symptom during their first three decades of life. About half have their first cardiac symptoms by the age of 12.

Rarely, signs and symptoms of long QT syndrome may occur during sleep or arousal from sleep. Some doctors believe that inherited long QT syndrome may explain some cases of sudden infant death syndrome (SIDS).


Your heart beats 100,000 times a day to circulate blood throughout your body. To pump blood, your heart's chambers must contract and relax in a coordinated manner. Contraction and relaxation are controlled by electrical impulses that travel through your heart muscle — like electricity through wires.

Your heart consists of four chambers — two upper chambers (atria) that receive blood and two lower chambers (ventricles) that pump blood. Within the upper-right chamber of your heart is a group of cells called the sinus node. The sinus node produces the electrical impulse that starts each heartbeat.

Your heart is composed of two upper and two lower chambers. The upper chambers, the right atrium and left atrium, receive incoming blood. The lower chambers, the more muscular right and left ...A prolonged QT interval refers to an abnormality seen on an electrocardiogram that reflects a disturbance in how your heart conducts electricity.

Each impulse first travels through your heart's atria. The atria contract, squeezing blood into the two ventricles below. The atria relax. The impulse then travels through your ventricles, and the ventricles contract, pumping blood out to your lungs and the rest of your body. The ventricles then relax. This pattern of alternating contraction and relaxation of your heart's upper and lower chambers makes up your heartbeat.

After each heartbeat, your heart's electrical system recharges itself in preparation for the next heartbeat. In long QT syndrome, however, your heart muscle takes longer than normal to recharge between beats. This electrical disturbance can be seen on an electrocardiogram (ECG). Another abbreviation for this test is EKG.

Prolonged QT interval
An ECG test measures electrical impulses as they travel through your heart. Patches with wires attached to your skin measure these impulses, which are displayed on a monitor or printed on paper.

An ECG measures electrical impulses as five distinct waves. Doctors label these five waves using the letters P, Q, R, S and T. The P wave shows electrical activity in your heart's upper chambers. The other waves, Q through T, reflect electrical activity in your heart's lower chambers.

The interval between the start of the Q wave and the end of the T wave (QT interval) corresponds to the time it takes for your heart's ventricles to contract and then rest before beginning the next contraction. In other words, the QT interval refers to the length of time it takes cells in your heart's lower chambers to electrically recharge between beats.

Doctors can measure a QT interval and can tell whether it occurs in a normal amount of time. If it takes longer than normal, it's called a prolonged QT interval.

Long QT syndrome results from abnormalities in the heart's electrical recharging system. However, the heart's structure is normal. Abnormalities in your heart's electrical system may be inherited or acquired.

Inherited long QT syndrome
Genetic variants in at least six genes cause approximately two-thirds of inherited long QT syndrome. Although sporadic or spontaneous mutations can occur, you typically inherit this variant from one of your parents. Rarely, you can receive genetic variants from both parents, which can result in a very severe form of long QT syndrome that's also accompanied by deafness.

Progress has been made in recent years in understanding the genetics of inherited long QT syndrome. Before these genetic breakthroughs, doctors initially described two forms of inherited long QT syndrome:

  • Romano-Ward syndrome. This more common form occurs in children who inherit only a single genetic variant from one parent.

  • Jervell and Lange-Nielsen syndrome. Symptoms of this rare form — named after the researchers who first identified it — usually occur earlier and are more severe than in Romano-Ward syndrome. It's seen in children who are born both deaf and who have long QT syndrome because they inherited genetic variants from each parent.

Scientists are investigating a possible link between SIDS and long QT syndrome. Researchers suspect that a small percentage of babies with SIDS had a genetic defect or mutation for this syndrome.

Acquired long QT syndrome
More than 50 medications, many of them common, can prolong the QT interval in otherwise healthy people and cause a form of acquired long QT syndrome known as drug-induced long QT syndrome.

Medications capable of prolonging the QT interval and upsetting heart rhythm include certain antibiotics, antidepressants, antihistamines, diuretics, heart medications, cholesterol-lowering drugs, diabetes medications, as well as some antifungal and antipsychotic drugs.

People who develop drug-induced long QT syndrome also may have some subtle genetic defects in their hearts, making them more susceptible to arrhythmias from taking drugs that can cause prolonged QT intervals.

Sometimes strokes or other neurologic disorders can cause acquired long QT syndrome.

Risk factors

People at risk of long QT syndrome include:

  • Children, teenagers and young adults with unexplained fainting, unexplained near drownings or other accidents, unexplained seizures or a history of cardiac arrest
  • Family members of children, teenagers and young adults with unexplained fainting, unexplained near drownings or other accidents, unexplained seizures or a history of cardiac arrest
  • Relatives of persons with known long QT syndrome
  • People taking medications known to cause prolonged QT intervals

Long QT syndrome is rare and often goes undiagnosed or misdiagnosed as a seizure disorder, such as epilepsy. However, researchers believe that long QT syndrome may be responsible for some otherwise unexplained deaths in children and young adults. For example, an unexplained drowning of a young person may be the first clue to inherited long QT syndrome in a family.

People with low potassium, magnesium or calcium in their blood — such as those with the eating disorder anorexia nervosa — may be susceptible to prolonged QT intervals. Potassium, magnesium and calcium are all important minerals for the health of the heart's electrical system.

When to seek medical advice

Sudden loss of consciousness during physical exertion or emotional excitement or after use of a new medication is a reason to see your doctor.

Because long QT syndrome can occur in families, consider seeing your doctor to be tested for long QT syndrome if a close relative (your parent, sibling or child) receives a diagnosis of or dies of suspected long QT syndrome.

Screening and diagnosis

If your doctor suspects that you have long QT syndrome, you may need to undergo several evaluations using an electrocardiogram to confirm the diagnosis and gauge the severity of the problem. In addition, your medical history and a list of your medications are important in diagnosing long QT syndrome.

Most people with suspected long QT syndrome have a clearly prolonged QT interval on an ECG. However, some don't, making the condition more difficult to diagnose. Other testing may then be necessary.

You may be referred to a cardiologist — a doctor who specializes in the study of the heart and its function — or a long QT syndrome specialist for additional tests such as:

  • An exercise stress test. This ECG test is performed while you're walking on a treadmill or pedaling a stationary bike. By monitoring your heart while it's working harder, your doctor can better assess its function and limits.
  • A nonexercise (medication) stress test. This ECG test is performed while you're given a medication that stimulates your heart in a way similar to exercise. The medication is given through a vein in your arm and may include epinephrine (adrenaline). Adrenaline is a substance that your body releases in response to stress. In some people with long QT syndrome, fainting spells are triggered by sudden bursts of adrenaline in the body, such as are experienced during intense exercise or emotional upset.
  • Ambulatory ECG monitoring (Holter monitoring). This test is used to monitor your heart for rhythm irregularities during normal activity for an uninterrupted 24-hour period. During the test, electrodes attached to your chest are connected to a portable recorder that attaches to your belt or is carried by a shoulder strap. The recorded information can then be analyzed to check for heart rhythm irregularities, such as prolonged QT intervals.
  • Event ECG recording. This is similar to the ambulatory ECG except that you may need to wear a portable ECG recorder for days or weeks as it records your heart rhythms.
  • An electroencephalogram (EEG). This test rules out neurologic causes of fainting, such as a seizure disorder. The procedure measures the waves of electrical activity the brain produces. Small electrodes attached to your head pick up the electrical impulses from your brain and send them to the EEG machine, which records brain waves.
  • Genetic testing. While genetic testing for long QT syndrome has been available on a limited basis for a number of years, a more commercially available genetic screening test for long QT syndrome was expected to be introduced in the summer of 2004.


Prolonged QT intervals may never cause any problems. However, physical or emotional stress may "trip up" a heart susceptible to prolonged QT intervals and cause the heart's rhythm to spin out of control.

A prolonged QT interval may trigger a particular irregular heart rhythm (arrhythmia) called torsades de pointes, in which your heart's ventricles beat fast. When this arrhythmia occurs, less blood is pumped out from your heart. Less blood then reaches your brain, causing you to faint.

If torsades de pointes is short lasting — lasting less than one minute — your heart can correct itself and beat normally again seconds later, and you regain consciousness on your own. However, if torsades de pointes persists, it can lead to a life-threatening arrhythmia called ventricular fibrillation.

In ventricular fibrillation, the ventricles beat so fast that your heart quivers and ceases pumping blood. Unless your heart is shocked back into a normal rhythm by a device called a defibrillator, ventricular fibrillation can lead to brain damage and death.

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This information is provided for general medical education purposes only and is not meant to substitute for the independent medical judgment of a physician relative to diagnostic and treatment options of a specific patient's medical condition.

In no event will the be liable for any decision made or action taken in reliance upon the information provided through this web site.
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