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What is LQTS?
Normal QT interval
Long QT interval
- LQTS is a disorder of the heart’s electrical system.
- The electrical activity of the heart is produced by the flow of ions (electrically charged particles of sodium, calcium, potassium, and chloride) in and out of the cells of the heart. Tiny ion channels control this flow.
- The QT interval is the section on the electrocardiogram (ECG) that represents the time it takes for the electrical system to fire an impulse through the ventricles and then recharge. It is translated to the time it takes for the heart muscle to contract and then recover.
- LQTS is a defect in the ion channels, causing a delay in the time it takes for the electrical system to recharge after each heartbeat. When the QT interval is longer than normal, it increases the risk for torsade de pointes, a life-threatening form of ventricular tachycardia.
- LQTS is rare. The prevalence is about 1 in 5,000 persons in the Untied States.
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What causes LQTS?
Long QT syndrome can be acquired or congenital:
Acquired LQTS is caused by many medications. Sensitivity to these medications may be related to genetic causes. See medications to avoid.
Congenital LQTS is usually inherited. It is caused by an abnormality in the gene that forms the ion channels, slowing the recovery phase of the heartbeat. Forms of inherited LQTS include:
- Recent Classifications – LQT1, LQT2, LQT3, LQT4, LQT5; these are classified by the type of channel which causes the LQTS. The type of LQTS classification is related to the risk of future cardiac events, those with LQT3 having the highest risk of life-threatening arrhythmias.
- Jervell, Lange-Nielsen Syndrome – both parents are carriers of the abnormal gene, but they do not have LQTS. Each child has a 25-percent chance of inheriting LQTS. This syndrome is associated with deafness at birth and is extremely rare, as there is a small chance that both parents would carry the LQTS gene.
- Romano-Ward Syndrome – one parent has LQTS and the other parent does not. Each child has a 50-percent chance of inheriting the abnormal gene. In this syndrome, hearing is normal; however the likelihood that children in this family would have LQTS is greater. The gene may be present in all the couple’s children, some of them or none at all.
Those at risk for LQTS include:
- children who are deaf at birth
- children and young adults who have unexplained sudden death or syncope in family members
- blood relatives of family members with LQTS
- those taking certain medications known to increase the risk for LQTS. See medications to avoid
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What are the symptoms?
The most common symptoms include:
- syncope
- seizures
- sudden death
The symptoms of LQTS are related to torsade de pointes. During this arrhythmia, the ventricle beats very fast and irregularly. The heart is unable to pump blood effectively to the body. If the brain does not receive an adequate blood supply, syncope (fainting) and seizure-like activity will occur. If the arrhythmia continues, sudden death will occur. If the heart rhythm returns to normal, symptoms will stop.
Symptoms are most common during:
- exercise (or within a few minutes after)
- emotional excitement, especially being startled
- during sleep or upon waking suddenly
Some people with congenital LQTS never have symptoms. The diagnosis is made during a routine ECG or during an evaluation because a family member has it. Symptoms usually first appear during the early teen years.
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How is LQTS diagnosed?
LQTS is usually diagnosed by measuring the QT interval on the ECG. Other testing may include:
Your doctor will also ask you if you have a:
- family history of LQTS
- family history of unexplained fainting, seizures, or cardiac arrest
- history of fainting, seizures or cardiac arrest, especially with exercise
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How is it treated?
Treatment is aimed at preventing sudden death and controlling symptoms. Treatment includes:
Medications
Most patients (even those without symptoms) are treated with a beta-blocker. Other medications may be used to shorten the QT interval. Your doctor will discuss what medications are best for you. It is important to know:
- the names of your medications
- what they are for
- how often and at what times to take them
Medications to avoid
There are many medications that can prolong the QT interval. Those with LQTS may be more prone to the effects of these medications. If you have LQTS, you should:
- Avoid all over-the-counter medications (except for plain aspirin or acetaminophen)
- Tell all your health care professionals you have LQTS, and there are many drugs you cannot take
- Talk to your doctor before taking any medications prescribed for any other medical condition. The following types of medications may affect you if you have LQTS:
- antihistamines
- antidepressants, mental illness medications
- heart medications
- antibiotics, anti-fungals, antivirals
- intestinal medications
- anticonvulsants
- diuretics
- antihypertensives
- migraine medications
- cholesterol lowering medications
For a complete, updated list of medications, contact the SADS Foundation at www.sads.org*
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Devices
- Patients who have a history of cardiac arrest or symptoms, in spite of beta-blocker therapy, may receive an implantable cardioverter defibrillator (ICD). This device detects life-threatening arrhythmias and automatically shocks the heart to prevent sudden death.
- Patients who have an abnormally slow heart rate may receive a pacemaker.
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Lifestyle changes
- Family testing - All first-line relatives (brothers, sisters, parents and children) should have EKG testing. Any other family members who have a history of seizures or fainting should also undergo testing.
- Exercise - If you have LQTS, sometimes, fatal arrhythmias occur with exercise. So, ask your cardiologist if you are able to exercise. Competitive sports are NOT recommended.
- Buddy system - Your family and friends should be told you have LQTS. They should be told to call for emergency help (911) if you begin to have symptoms or faint.
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Future treatments will be geared toward more gene specific therapies. For example, certain types of LQTS are more likely to initiate events during exercise, while others are more related to startling or emotional distress. Your doctor will be able to give you activity guidelines based on the specific type of LQTS gene you carry. Therapies may be directed to treat the specific gene, rather than prevention of future complications.
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How to find a doctor if you have Long QT Syndrome (LQTS)
Adult LQTS:
Find a Cleveland Clinic Heart Center Cardiologist who treats Abnormal Heart Rhythms
Pediatrics:
Find a Cleveland Clinic pediatrician who treats Abnormal Heart Rhythms
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Resources:
The Sudden Arrhythmia Death Syndromes Foundation (SADS),
540 Arapeen Drive, Suite 207, Salt Lake City, UT 84108
Phone: 800.786.7723
E-mail: sads@mail.aros.net
www.sads.org.*
American Heart Association: Long QT Syndrome*
*a new browser window will open with this link. The inclusion of links to other web sites does not imply any endorsement of the material on the web sites or any association with their operators
