Treatment

Treatment for inherited long QT syndrome involves some simple preventive measures. It can also involve medications, as well as left cardiac sympathetic denervation surgery or implanting medical devices such as a defibrillator (ICD), or both.

The goal of treatment is either to prevent the long QT heart from ever beating out of control or to prevent sudden death. Your doctor will discuss with you the most appropriate treatment options for your condition based on your symptoms, your type of long QT syndrome, and your risk of fainting or sudden cardiac arrest.

For acquired long QT syndrome, treating the cause of the condition may eliminate it. Doctors will also treat heart rhythm disorders (arrhythmias) as needed.

If you are diagnosed with acquired long QT syndrome due to certain medications, your doctor may recommend that you stop taking the medication causing the condition and switching medications. Some people might need additional treatment.

Medications

Medications used to treat long QT syndrome may include:

  • Beta blockers. These heart drugs include nadolol (Corgard) and propranolol (Inderal LA, InnoPran XL). They slow the heart rate and make the rhythm associated with long QT syndrome less likely. Beta blockers work by blunting the way your heart reacts to adrenaline.
  • Mexiletine. Taking this anti-arrhythmic drug in combination with beta blockers might help shorten the QT interval and decrease the likelihood of a long QT syndrome-triggered faint, seizure or sudden death.

    This medication is often used for the third-most common subtype of inherited long QT syndrome, LQT3. However, it may also be used for the two most common subtypes, LQT1 and LQT2.

  • Spironolactone and potassium. For certain forms of long QT syndrome, spironolactone (Aldactone), a medication used to help the body hold on to potassium, or potassium supplements, or both, might improve the heart's recharging system.
  • Fish oil. In general, current evidence does not support supplementation with heart-healthy fish oil (omega-3 fatty acid) or eating fish high in omega-3 fatty acids to decrease the risk of cardiac events or death from abnormal heart rhythms. However, if you have inherited long QT syndrome, fish oil may be a reasonable complementary alternative medication to take in addition to other therapies. Talk to your doctor before starting fish oil or any other supplements or medications.

Your doctor might suggest treatment for long QT syndrome even if you don't often experience signs or symptoms.

If you do need treatment, take the medications your doctor prescribes as directed. While medications won't cure the condition, they provide some protection against possibly fatal disruptions of your heart rhythm. You might need to take a medication such as a beta blocker indefinitely.

Surgical procedures

Depending on your condition, your doctor might consider other treatments for you, including:

  • Left cardiac sympathetic denervation surgery. In this procedure, surgeons remove specific nerves along the left side of your spine in your chest. These nerves are part of the body's sympathetic nervous system, which helps regulate heart rhythm. The surgery significantly reduces the risk of sudden death.

    This surgery is generally reserved for people who are considered at high risk of sudden death and are experiencing appropriate ICD shocks, people who are continuing to experience fainting or seizures while taking their medications, or people unable to tolerate their medications because of side effects.

  • Implantable cardioverter-defibrillator (ICD). This device, which is implanted under the skin of your chest, can stop a potentially fatal arrhythmia. An ICD continuously monitors your heartbeat. If it detects an abnormal heart rhythm, it delivers electrical shocks to reset the heart to a normal rhythm.

    Implanting an ICD is a major procedure and can result in inappropriate shocks and other complications. Therefore, the decision to implant an ICD, especially in children, needs to be carefully considered.

    Most people with inherited long QT syndrome do not need an ICD. Importantly, an ICD should not be implanted solely because of the tragic occurrence of a long QT syndrome-triggered sudden death in a relative.

Potential future treatments

A combination of advances in the field of genetics, computerized clinical decision support systems that alert doctors to drug interactions, and handheld or wearable monitors may help improve treatment for people with both inherited and acquired long QT syndrome.

Lifestyle changes

In addition to medications or surgery, your doctor might recommend lifestyle changes to reduce your chances of a long QT syndrome-related fainting spell or sudden cardiac arrest. These could include:

  • Avoiding medications that could cause prolonged QT intervals
  • Getting plenty of liquids during illnesses that are causing vomiting or diarrhea
  • Lowering your temperature if a fever occurs
  • Reducing your exposure to loud or startling noises
  • Staying away from situations that could make you excited or angry
  • Avoiding strenuous exercise or contact sports, or have a safety plan in place if you continue in competitive sports

Work with your doctor to balance these lifestyle recommendations against the clear, heart-healthy benefits of an active lifestyle. It may be possible to stay fully active in sports, including competitive sports, after carefully reviewing the risks and benefits with your doctor.

If your symptoms are mild or don't occur very often, your doctor might recommend only simple preventive measures or lifestyle changes, and he or she might not prescribe any daily medications for you.

July 22, 2017
References
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