Congenital Long QT Syndrome (abbreviated LQTS) is a congenital heart condition caused by a genetic mutation.

Sometimes, people can develop LQTS later in life as a side effect of various medications, including  certain antiarrhythmic drugs, antibiotics, antihistamines, and antidepressants.

LQTS can be managed, but it may have potentially fatal complications. This article will help you understand what Long QT Syndrome is and how to deal with it.

What is Long QT Syndrome?

The term “Long QT” is a reference to a specific abnormality in the patient’s electrocardiogram or ECG. During the normal heart rhythm the heart will contract to pump blood and then relax to fill up again. All of this is controlled by the electrical impulses that control the heart’s rhythm. When the heart’s electrical system “fires,” the electrocardiogram (which is an recording of the heart’s electrical activity) exhibits a sharp squiggle known as a “QRS Complex.”  After each heartbeat, the electrical system of the bottom chambers (ventricles) must reset itself to get ready for the next beat.  The signal produced by this electrical resetting is a broad wave known as the T-wave. We can measure how long it takes for the heart to reset itself by measuring the time from the beginning of the QRS to the end of the T-wave, which is known as the QT Interval.

For people with Long QT Syndrome, the QT interval is longer than normal. This usually occurs because of an electrical imbalance of the heart and abnormal movement of sodium and potassium in and out of the heart muscle cells.

If the QT interval is too long, the heart may become unstable electrically. This can lead to dangerously rapid electrical disturbances in the bottom chambers. Those electrical disturbances result in very rapid, dangerous beating of the heart, to the point where the heart stops pumping effectively. At its worst, Long QT Syndrome can cause sudden death, even in young people. It can also cause fainting and seizures from very rapid heart arrhythmias.

Doctors will use a variety of methods to diagnose LQTS, but the most important test is the ECG. There are actually many different types of LQTS, but the three most common are referred to as LQT1, LQT2, and LQT3. Additional information can be obtained from other testing such as a stress test, holter monitor, event monitor, or an echocardiogram. Genetic testing is available to determine which gene mutation is responsible for the problem, as there are dozens of mutation that can cause LQTS. Knowing which variety of LQTS you have is important to help determine the risk of serious problems and the best treatment.

What Treatments for Long QT Syndrome are Available?

Much of the treatment for LQTS depends whether it is congenital (genetic) or secondary to drugs or other electrical problems (acquired LQTS).  Obviously, if a patient has “Drug-Induced Long QT Syndrome” or DILQTS, the main treatment is to eliminate the drug or drugs responsible for the problem. Most of the time the patient’s condition will improve, but once you identify a patient who is prone to DILQTS, they should avoid any other drugs that are known to prolong the QT Interval.

Above all, patients with LQTS should be under the care of an experienced cardiologist or heart rhythm specialist (Cardiac Electrophysiologist). Given the risk of sudden death, you need to seek the expertise of a specialist for guidance in matters related to this condition.

Here are some treatments typically prescribed for Congenital Long QT Syndrome:

1. Medicine

Most patients with Congenital LQTS benefit from beta blockers, which block adrenaline. Beta blockers can help stabilize patients with the more common types of LQTS, and are essential in anyone with LQTS who has fainted in the past.

2. Surgical Procedures

Patients with LQTS who are felt to be at risk of life-threatening heart rhythm problems may receive an implantable cardioverter-defibrillator (ICD). This battery-operated device is surgically implanted under the skin at the left upper chest region. It monitors the heart continuously, and if the patient develops a lethal arrhythmia like ventricular tachycardia or ventricular fibrillation it can deliver a strong electrical jolt to save the patient’s life (defibrillation). ICDs are reserved for patients who already have experienced a life-threatening arrhythmia or patients who continue to have symptoms such as fainting despite the use of beta blockers. Certain rare types of LQTS (like LQT3) have such a high risk of sudden death that an ICD may be recommended as a preventative measure even in the absence of symptoms.

Rarely, surgical intervention for LQTS may be recommended in patients with severe symptoms who are refractory to medical therapy. The procedure involves removing certain nerves along the left side of the spine that are involved with the sympathetic (fight or flight) nervous system. It may be successful in reducing or eliminating the arrhythmia in some patients with LQTS.

How Can I Treat LQTS Without Medications?

With LQTS, there are lifestyle changes that may help. There are no holistic remedies that are proven to be beneficial, but some are acceptable to take. For example, potassium supplementation is often recommended by doctors, provided that the patient does not have an excessively high potassium level or have underlying kidney disease.  Other lifestyle strategies include:

1. Exercise Limitation

The most common type of congenital LQTS (LQT1) can result in serious, even life-threatening rapid heart arrhythmias, but most often these arrhythmias are triggered by high adrenaline levels released during strenuous exercise. Patients with LQTS, even those with no prior symptoms, should avoid heavy exercise like competitive sports.  Light exercise like walking or low-impact aerobics may be fine, but you need to check with your cardiologist or electrophysiologist for specific recommendations.

2. Stress Reduction

One of the recommended “treatments” for Long QT Syndrome is stress reduction. This also includes making sure that the activities you engage in are not going to stress the heart out too much.  Certain types of LQTS (specifically LQT2) can result in sudden rapid ventricular arrhythmias in response to loud noises like a car horn, so doctors recommend lowering the volume of cellphone ringers and avoiding jarring noises like alarm clocks and loud doorbells.

Additional mind/body techniques such as meditation can be very helpful for someone with Long QT Syndrome. Yoga can also help, but be aware that today’s yoga can be a lot more stressful that the yoga of yesteryear. Opt for gentle yoga classes or restorative, rather than high-powered yoga classes.

3. Electrolyte Supplementation

Potassium is often recommended for people with LQTS. Magnesium supplementation might also be helpful, but this is not clinically proven.

4. Fish Oil

If you have inherited long QT syndrome, fish oil may be a reasonable dietary supplement to take in addition to other therapies. However, there is no proven benefit to this and other “holistic” remedies.

5. Healthy Diet

A healthy diet is sure to improve any medical condition you have, so of course, it is recommended for Long QT Syndrome as well. A heart-healthy diet can only improve the ability of your heart to function. Reduce the consumption of overly fatty foods, excess sugar, and, of course, caffeine.

Talk to Your Doctor About Long Q-T Syndrome

LQTS can potentially cause serious, even fatal heart rhythm problems.  Although lifestyle changes for some people with the condition may be helpful, anyone with this condition should be under the care of an experienced medical specialist. Your doctor can help guide you to the best way to manage Long QT Syndrome.

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