Long QT syndrome
![Long QT syndrome](https://mdtodate.com/wp-content/uploads/2022/01/LongQT-ECG--300x150.png)
Long QT syndrome (LQTS) is a heart rhythm disorder that could cause rapid irregular heartbeats. These heartbeats can cause the patient to faint suddenly. Certain people suffering from the condition experience seizures. In certain instances, LQTS can cause sudden death.
Certain people have an inherited mutation that can cause Long QT Syndrome (congenital Long QT Syndrome). Long QT syndrome could result from certain drugs minerals, imbalances in mineral levels, or medical illnesses (acquired Long QT Syndrome).
Long QT syndrome can be treated. It is possible to limit or take certain medicines to reduce the risk of heartbeat events. Sometimes the treatment of long QT syndrome requires surgical intervention or the use of implantable devices♥.
Symptoms
Shedding light on dangerous faints
A lot of people with long QT syndrome do not show any symptoms or signs. You may be aware of the condition due to:
- The results are from electrocardiograms (ECG) taken in connection with an unrelated cause
- An ancestral history is associated with QT syndrome
- Genetic test results
The most frequent manifestation of the long QT syndrome.
Fainting (syncope) is when the heart beats in an irregular manner. It is possible to faint when stressed, angry, or scared or when you exercise. Things that frighten you like an alarm clock, could cause you to fall asleep. A fainting or shaky feeling due to prolonged QT syndrome could be experienced with no warning. Some individuals may have an early warning sign that is the feeling of lightheadedness, heart palpitations blurred vision or weakness.
Seizures can also occur in those who suffer from LQTS.
In general, the heart will return back to a normal beat. If this doesn’t happen itself or if an external defibrillator doesn’t work at the time needed to bring your heart’s beat in a normal way, then sudden death could take place.
Many people suffering from indications of symptoms of QT syndrome experience their first episode before the age of 40. However congenital long QT syndrome symptoms and symptoms may be seen in newborns in the first weeks or months following birth, or even in early childhood or later. A few people suffering from congenital QT syndrome don’t show any symptoms or signs.
Signs and symptoms of QT syndrome can manifest in sleep.
When is the best time to seek medical help?
Contact your physician if you suddenly experience a fainting sensation during exercise in the midst of emotional turmoil or following the use of any new medication. If you are a family member in the first degree (parent or sibling) who suffers from prolonged QT syndrome, it’s vital to inform your doctor. Long QT syndrome is a common occurrence in families.
Long QT syndrome is a heart rhythm disorder triggered by heart-related issues with the electrical recharge system. The heart’s structure is normal.
The heart normally sends blood to the body with each heartbeat. The chambers in the heart expand and relax to circulate blood. It is controlled through the cardiac electrical circuit. The electrical signals (impulses) flow between the upper and bottom of your heart to cause it to beat and contract. Following each heartbeat, the heart system recharges itself to prepare for the next heartbeat.
In the case of long QT syndrome, your heart’s electrical system is taking longer than usual to recharge between heart beats. This is the delay that is visible in the electrocardiogram (ECG) is known as an extended QT interval.
Long QT syndrome is usually divided into two types, based on the reason.
- If you’re born with this condition, it’s known as congenital Long QT syndrome. Certain types that are a result of QT syndrome are caused by genetic mutations that are transmitted via the families (inherited).
- If an unrelated medical issue or medication causes it, it’s known as an acquired Long QT Syndrome. This kind of long QT syndrome can be reversed once the root cause has been discovered.
The causes of congenital long QT syndrome
Over 17 genes are associated with the long QT syndrome as of now Researchers have discovered hundreds of mutations in these genes.
Doctors have identified two types of congenital long QT syndrome.
- Romano Ward syndrome (autosomal dominant variant). This more frequent form is found in those who only inherit a single mutation in the gene in one of their parents.
- Jervell as well as Lange-Nielsen Syndrome (autosomal recessive variant). This rare type usually manifests later and can be more serious. In this form, children are born with defective genes that are passed down from their parents. Children have been given the long QT syndrome as well as deafness.
What causes acquired QT syndrome
![Normal heart chambers](https://mdtodate.com/wp-content/uploads/2022/01/heart-physiology-300x236.png)
More than 100 drugs some of which are commonly used may prolong the QT interval in healthy individuals and lead to the development of long QT syndrome.
The medications that may cause long QT syndrome in the acquired form can cause it. These include:
- Certain commonly used antibiotics, like erythromycin (Eryc, Erythrocin, other) azithromycin (Zithromax, Zmax), and others.
- Certain antifungal medications that are administered by mouth can be used in treating yeast infections
- Diuretics that can cause an unbalanced electrolyte (low potassium levels, commonly)
- Heart rhythm medications (especially anti-arrhythmic medicines that increase your QT time)
- Certain antidepressants and antipsychotic drugs
- A few anti-nausea medicines
If your problem is due to medication, it can be referred to as drug-induced long QT syndrome. Be sure to inform your doctor of any medication you are taking and even those you purchase without a prescription.
Other causes of the acquired QT syndrome can be attributed to:
- Low potassium levels (hypokalemia)
- Low calcium levels (hypocalcemia)
- Low magnesium levels (hypomagnesemia)
- COVID-19 infection
Risk factors
Certain factors can increase the risk of developing acquired or congenital long QT syndrome, or its manifestations:
- A previous history of cardiac arrest
- Being a first-degree relative (parent sibling) with the long QT syndrome
- Utilizing medicines that have been proven to prolong QT intervals
- Being a woman and taking heart medication
- Excessive vomiting or diarrhea
- Disorders of eating, like anorexia, can result in electrolyte imbalances
Sexual relations don’t seem to carry a high risk for people suffering from the syndrome of long QT. Pregnancy and birth aren’t associated with a risk increase of developing symptoms in women who have been diagnosed with the syndrome. If you do have the disorder and are pregnant the doctor will be sure to closely monitor you both during and after the pregnancy.
Complications
The right medical treatment and lifestyle modifications can reduce the risk of complications due to long QT syndrome.
Complications of the long QT syndrome can include:
- Torsades de Pointes (‘twisting between the points’). This is a serious form of arrhythmias of the ventricular type. The heart’s lower chambers (ventricles) beat quickly and in a chaotic manner, creating waves in an ECG monitor that look twisted. The heart pumps less blood. The absence of blood supply to the brain can cause your body to collapse suddenly and, in many cases, without warning. If the episode continues for an extended period, the episode of fainting could lead to an entire body seizure. If the risky rhythm is not corrected a life-threatening arrhythmia known as ventricular fibrillation can occur. Other arrhythmias have also been reported.
- Ventricular fibrillation. This condition causes the ventricles to beat at such a rate that the heart quivers and ceases pumping blood. Unless your heart is stimulated back to a normal rhythm using a defibrillator ventricular fibrillation could cause brain injury and death in a flash.
- The sudden death. It’s now known that long QT syndrome may cause sudden death among young people who appear otherwise healthy.
- Unexplained seizures, drownings, faintings or other accidents. Long QT syndrome may be responsible for a number of unsolved deaths of children and young adults.
Prevention
Congenital Long QT syndrome is caused by a genetic mutation. There is no cure. But, with the right care, it is possible to reduce heartbeats that are dangerous and could cause prolonged QT syndrome-related problems.
It is possible to avoid health issues that can cause some forms of long QT syndrome acquired by having regular health checks and regular communication with your physician. It is particularly important to stay clear of medications that could alter your heart rate and lead to a prolongation of that QT-QT interval.
Diagnosis
To identify the long QT syndrome, your physician will conduct a physical examination and will ask questions regarding the symptoms of your child or you and the family and medical history. Your doctor will utilize a stethoscope for listening to your heart.
Electrocardiograms are the most frequently used test to determine the presence of long QT syndrome.
Electrocardiogram (ECG or EKG)
An electrocardiogram is a fast and painless test that captures the electrical signals inside your heart. You could be suffering from an ECG when you are at rest, or at rest or while exercising on a treadmill, while at rest or on a stationary bicycle. If you are unable to safely exercise or rest, you might be offered an epinephrine-based medication (Adrenalin) which stimulates your heart’s activity in a manner like an exercise.
During an ECG the sticky patch (electrodes) can be placed over your chest to capture the electrical signals generated by your heart that trigger your heartbeat. The signals are displayed in waves on a printer or monitor for a computer.
An ECG It is possible to determine if you’re experiencing a prolonged QT. The test categorizes your heart’s electrical signals as five waves with the letters P Q, Q, and S. You can also tell if you are experiencing a prolonged QT interval. The waves that are labelled Q-T indicate the electrical activity that occurs in the lower chambers of your heart (ventricles).
The time between the start of the Q wave to the conclusion of the T-wave is called the QT interval. It takes time for the heart’s muscles to relax and fill with blood prior to beating again.
What’s normal for you is contingent on your age of you, gender, and heart rate. Your doctor will look into this information. If the interval is longer than usual to happen this is known as an extended QT interval.
Remote ECG monitoring
If your lengthy QT symptoms are prone to appear or go away, it might not be able to be spotted during the course of a typical ECG recording. In this instance, your doctor might recommend either continuous or remote ECG monitoring. There are many different kinds of monitoring.
- Holter monitor. A Holter monitor is a tiny, wearable device that captures an ongoing ECG generally for between 24 and 48 hours.
- Event monitor. This portable device is like a Holter monitor, however, it only records at specific dates for a short period at one time. It is possible to wear it for longer than the Holter monitor, but typically for 30 days. It is typically a matter of pressing the button whenever you experience symptoms. Certain devices automatically record the moment an abnormal rhythm is observed.
Genetic testing
A genetic test to determine the cause of long QT syndrome is available and is recommended by numerous physicians to establish the presence of the condition. Contact your insurance company to determine if the test is covered.
It is important to know that tests for genetic causes of long QT syndrome aren’t able to detect all cases of inherited lengthy QT syndrome. It is recommended that families talk to a genetic counsellor prior to and after the test.
If you’ve been able to confirm a DNA test to detect the long QT syndrome, your physician might recommend that any of the other members of your family also test to find out if they have this gene.
Treatment
The treatment for the long QT syndrome includes lifestyle changes as well as medications. It could also involve surgical procedures or other treatments.
The purpose of treatment is to stop irregular heartbeats and sudden deaths. The doctor will go over with you the best treatment options based on the symptoms you are experiencing and the type of QT syndrome. Your doctor may suggest treatments even though you don’t notice any signs or symptoms.
If you are suffering from drug-induced QT syndrome Stopping the medication that causes the symptoms could be the only thing needed to manage the problem. Your physician will be able to tell you how to do this.
In other instances of acquired long QT disorder, treatment of the cause is required. Treatment is different but can include magnesium and other fluids prescribed by IV to balance electrolytes.
Medications
Medicines won’t fix long QT syndrome, However, they can safeguard against potentially life-threatening changes in the heart rhythm.
Treatment options for the long QT syndrome can include:
- Beta blockers. These heart drugs are the most common treatment option for patients suffering from Long QT Syndrome. They reduce heart rate and make lengthy QT incidents less common. Beta-blockers are prescribed in treating QT conditions comprise the nadolol (Corgard) as well as propranolol (Inderal LA, InnoPran XL).
- Mexiletine. Taking this heart rhythm drug in conjunction with a beta-blocker may reduce the QT interval and lower your chance of fainting, seizures, and sudden demise.
Always take the medication prescribed by your physician as directed.
Other procedures or surgeries
Based on your specific symptoms, your physician may look into other QT syndrome treatments that may be appropriate for you, such as:
- left cardiac sympathetic denervation (LCSD) procedure. In this procedure surgeons cut off certain nerves that lie on to the spine’s left inside your chest. These nerves form part of the sympathetic nervous system of your body that helps regulate your heart rate. It is usually reserved for those with a prolonged QT or persistent heart rhythm issues with a high risk of developing symptoms and who are unable to use or tolerate beta blockers. It isn’t a cure for long QT syndrome however it can help lower the risk of sudden death.
- Implantable cardioverter-defibrillator (ICD). An ICS is Implanted under the chest’s skin and constantly detects the heartbeat. It could stop a life-threatening arrhythmia. When it detects an irregular heart rhythm when it detects an irregular heartbeat, it releases electrical shocks that reset the heart to normal. Many people with long QT syndrome don’t require an ICD. However, it can be carried out in athletes in order to allow them to athletes to return to competitive sports. The choice to implant an ICD in particular, in children, should be considered with care. Implanting an ICD is a significant procedure that can cause unintentional shocks or other issues.
Lifestyle and home solutions for home and lifestyle
Alongside recommending medication or surgical procedures, your physician may suggest changes in your lifestyle to decrease your risk of suffering a prolonged QT syndrome-related fainting spell, or sudden heart attack.
- Find out what sports you can play safely. It may be feasible to be fully engaged in sport, including competition, after studying the risks and benefits with your physician. It is generally accepted that those with long QT syndrome shouldn’t swim by themselves. It is likely that recreational activities will be fine if you have a companion in the event of an episode of fainting.
- Conduct a sound test. Turn down the volume on doorbells, as well as others (such as phones) which could wake you, particularly during sleeping.
- Keep your emotions in check. Being very excited or in awe of someone else’s reaction could trigger changes in heart rate in people suffering from long QT syndrome.
- Be sure to check your medications. Avoid drugs that may cause prolonged QT intervals. If you’re not certain, ask your physician. It’s recommended to inform your doctor about every medication and supplement you use, even the ones you purchase without a prescription.
- Check-ups are scheduled regularly. During your appointment make sure to inform your doctor of the modifications in the symptoms you experience or your condition. Your doctor might modify treatments or offer other therapies for you.
Helping to cope and providing support
Being concerned about potentially dangerous heart rhythms that are associated with the long QT syndrome can create stress for both you and your family members. Do your best to aid your loved ones to know how they can support you and help your needs in case of emergency.
- Let other people know. Make family, teachers, friends, and your neighbors, as well as any other person who has frequent contact with you, aware of your heart condition as well as the symptoms. You should wear a health alert ID to inform others about your heart condition.
- Prepare for emergencies. Family members may be interested in learning about cardiopulmonary resuscitation to be able to offer immediate resuscitation in the event that you require it. In certain circumstances, it is appropriate to own or be able to quickly access an automated external defibrillator.
- Find support or counsel. Some people find it beneficial by joining a group in which they can discuss their experiences and emotions with others and their families who are aware of the disorder. Families who have genetically inherited long QT syndrome may consider it beneficial to speak with an expert in genetics.
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