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Long QT syndrome (LQTS) is an arrhythmogenic disorder characterized by repolarization abnormalities with a tendency to cause life threatening cardiac events. The first manifestation of the syndrome may be sudden death, therefore, early diagnosis and therapy is of great importance. LQTS can be congen …
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Long QT syndrome. For people with LQTS who survive cardiac arrest and remain untreated, the risk of death within 15 years is greater than 50%. With proper treatment this decreases to less than 1% over 20 years.
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“Generally speaking, adults with LQTS who have not had clinical symptoms yet, such as passing-out spells, or have survived sudden cardiac death, have continued but diminishing risk of sudden death from LQTS as they age.” In summary, there is no data on how long a person can live with undiagnosed — and therefore untreated — long QT syndrome.
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What is long QT syndrome and can it be treated?
What is the life expectancy of someone with long QT syndrome?
Is there any natural treatment for long QT syndrome?
How do I live with long QT syndrome?
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Who is the first degree relative of QT syndrome?
First-degree relatives are your parents, siblings and children. The first step is to tell your doctor that you have a family history of this condition.
What are the symptoms of LQTS?
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 can occur.
Why is LQTS rare?
LQTS occurs as the result of 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 Q-T interval is longer than normal, it increases the risk for torsade de pointes, a life-threatening form of ventricular tachycardia. LQTS is rare.
What are the most common forms of inherited LQTS?
Forms of inherited LQTS include: Recent Classifications – Multiple ion channel abnormalities have been discovered. The most common ones include LQT1, LQT2, LQT3, LQT4, LQT5; these are classified by the type of channel which causes the LQTS.
When do congenital LQTS symptoms appear?
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.
What is the Q-T interval?
The Q-T 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.
What is Lange Nielsen syndrome?
Jervell, Lange-Nielsen Syndrome (autosomal recessive inheritance pattern) – Both parents are carriers of the abnormal gene, but they may not manifest LQTS. Each child has a 25-percent chance of inheriting LQTS.
What is long QT syndrome?
Acquired long QT syndrome is the result of conditions, medications or events that prolong the QT interval. It is more common in women than men. Stopping the medication and/or correcting the condition causing LQTS often resolves the issue. Sometimes the symptoms are caused by not one but several triggers — for example, a genetic mutation combined with a medication that prolongs the QT interval.
When are children diagnosed with LQTS?
Children may be diagnosed in infancy or later, either because they go through an episode of seizures or cardiac arrest, or because of family members known to have the mutation. Babies have even been diagnosed in utero. Boys are more likely than girls to have a dangerous cardiac episode from LQTS by age 15.
What are the symptoms of LQTS?
If symptoms appear, they may include: Palpitations (a fluttering in the chest) Seizure-like activity caused by lack of blood flow to the brain. Syncope (dizziness or fainting) Sudden cardiac arrest.
Can a drug lengthen QT?
A wide variety of drugs may lengthen the QT interval . If you have been diagnosed with LQTS, consult your physician about which drugs put you at risk. The most common types of drugs are:
Can genetic testing confirm LQTS?
Genetic Testing. If congenital LQTS is suspected our genetic counselors can assist you with genetic testing to confirm the underlying cause. Once the genetic cause is identified then at risk family members can consider genetic testing to determine if they are at risk as well.
What is the medical term for a long QT?
Long QT syndrome (LQTS) is a medical condition that affects the normal electrical activity of the heart. The term QT refers to the portion of the tracing on an electrocardiogram (EKG) that reflects the change in heart rhythm. Doctors may also call this condition Jervell and Lange-Nielsen syndrome or Romano-Ward syndrome.
How to prevent LQTS?
Reducing stress and anxiety whenever possible. Consider giving yoga or meditation a chance. Avoiding strenuous exercise and some types of sports, such as swimming. Swimming, especially in cold water, is a known trigger for LQTS complications.
What to do if you have LQTS 3?
taking sodium channel blockers if you have LQTS 3. If you experiencing fainting or other signs of an abnormal heart rhythm, your doctor may recommend more invasive treatment, such as implanting a pacemaker or implantable cardioverter defibrillator. These devices recognize and correct abnormal heart rhythms.
How many types of LQTS are there?
LQTS can be either inherited or acquired, meaning that something beyond genetics causes it. Seven types of inherited LQTS exist. They are numbered LQTS 1, LQTS 2, and so on. Researchers have identified more than 15 different types of genetic mutations that can lead to LQTS.
Why is LQTS important?
LQTS is concerning because the heart relies on an even, steady rhythm and electrical activity to beat correctly. LQTS makes it easier for the heart to beat out of time. When that occurs, oxygen-rich blood doesn’t pump to the brain and body. Not everyone with LQTS has symptoms, but those who do might notice:
Can you take LQTS with a long period of time?
If you take any of these medications for a long period of time, your doctor might regularly monitor your heart rhythm on an EKG to check for anything unusual. Several other things can cause LQTS, especially those that cause a loss of potassium or sodium from your bloodstream, such as: severe diarrhea or vomiting.
Is there a cure for LQTS?
There’s no cure for LQTS. Instead, treatment usually involves reducing your risk of developing a heart arrhythmia by: taking medications called beta blockers to reduce too fast heart rhythms. avoiding medications known to prolong QT interval. taking sodium channel blockers if you have LQTS 3.
When does long QT syndrome occur?
However, congenital long QT syndrome signs and symptoms can occur in babies during the first weeks to months after birth, or in childhood or later. Some people with congenital long QT syndrome never have signs or symptoms. Signs and symptoms of long QT syndrome might occur during sleep.
What is prolonged QT?
This delay, which often can be seen on an electrocardiogram (ECG), is called a prolonged QT interval. Long QT syndrome is often grouped into two main categories, depending on the cause. If you are born with the condition, it's called congenital long QT syndrome. Some forms of long QT syndrome result from a genetic mutation ...
What to do if you suddenly faint?
Call your doctor if you suddenly faint during physical activity or emotional excitement or after taking any new medication. If you have a first-degree relative (parent, sibling or child) with long QT syndrome, it's important to let your doctor know. Long QT syndrome can run in families.
How many genes are linked to long QT?
More than 17 genes have been linked to long QT syndrome so far, and researchers have identified hundreds of mutations within these genes. Doctors have described two forms of congenital long QT syndrome: Romano-Ward syndrome (autosomal dominant form).
What causes QT syndrome?
Long QT syndrome may be caused by certain medications, mineral imbalances or medical conditions (acquired long QT syndrome). Long QT syndrome is treatable. You might need to avoid or take certain medications to prevent dangerous heartbeat ...
Can you prevent QT syndrome?
You may be able to prevent the health conditions that lead to some types of acquired long QT syndrome with regular health checkups and good communication with your doctor . It is especially important to avoid medications that can affect your heart rhythm and cause prolongation of the QT interval. By Mayo Clinic Staff.
Can long QT be prevented?
Congenital long QT syndrome is caused by a genetic mutation. There is no known prevention. However, with proper treatment, you can prevent the dangerous heartbeats that can lead to long QT syndrome complications.
What is the best treatment for LQTS?
Beta-blockers are drugs of choice for patients with LQTS. [ 7] . The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. They may also reduce the QT interval in some patients.
What antihistamines should be avoided for long QT syndrome?
Epinephrine ( adrenaline) for local anesthesia or as an asthma medication should be avoided in patients with long QT syndrome (LQTS). Antihistamines. Antihistamines to be avoided include the following: Terfenadine (Seldane [recalled from US market]): For allergies.
What is gene specific therapy?
Gene-specific therapy is an area under investigation in the treatment of long QT syndrome (LQTS). For example, because LQT3 is associated with gain-of-function mutations in sodium channels, antiarrhythmic agents with sodium channel blocking properties have been suggested as gene-specific therapy for patients with LQTS3.
Do asymptomatic patients with LQTS need hospitalization?
Asymptomatic individuals with LQTS usually do not require hospitalization. However, carefully evaluate them and provide follow-up care in an ambulatory setting. A cardiologist or a cardiac electrophysiologist should examine patients with LQTS on a regular basis.
Can you swim with LQT1?
Syncope and sudden cardiac death during swimming or diving are strongly related to LQT1. Therefore, patients with LQT1 should avoid swimming with no supervision.
Who is consulted when evaluating LQTS?
A cardiologist and a cardiac electrophysiologist are typically consulted when patients with LQTS are evaluated. In families of patients with genotypically confirmed LQTS, genetic counseling of patients and family members should be considered.
Can QT prolonging drugs reduce potassium?
All patients with long QT syndrome (LQTS) should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium levels. In a study of 133,359 electrocardiograms from 40,037 patients, the use of a single QT-prolonging agent increased the corrected QT (QTc) interval by 11.08 ms; when two such drugs were used, there was another 3.04 ms increase in the QTc interval relative to when a single drug was used. [ 4]
What Is Long QT Syndrome?
Long QT syndrome is a condition that increases risk for dangerous arrhythmias. It can lead to fainting, cardiac arrest and sudden cardiac death.
QT Prolongation Symptoms
The symptoms of long QT syndrome often occur when a person is active or experiences emotional or physical distress. Symptoms typically begin in childhood and may include:
Long QT Syndrome Diagnosis
If you have symptoms or a family history that indicates long QT syndrome, you will have tests including:
Long QT Syndrome Treatment at Penn
The Penn Cardiac Arrhythmia Program is a nationally recognized leader in treatments for many types of arrhythmias. Every year, we implant more than a thousand devices and counsel patients on effective ways to prevent complications.
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