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Long QT Syndrome

Long QT syndrome is a heart condition that affects the heart’s electrical system and can lead to sudden cardiac arrest. Treatment options vary based on severity and may include medication, implanted devices, or surgery to help regulate heart rhythm and reduce complications.

What is long QT syndrome?

Long QT syndrome occurs when the heart takes too long to recharge between beats, creating a longer-than-normal QT interval, or a prolonged QT interval.

A healthy heart beats at a regular rhythm. Steady electrical signals tell the heart when to contract and when to recharge. On an electrocardiogram (EKG or ECG), these signals appear as waves. Doctors label each part of the wave with a letter, typically P through U.

  • The Q phase marks the start of the ventricular contraction.
  • The T wave indicates when the ventricles finish relaxing.
  • The time between the Q and T phases is called the QT interval, which should normally range from 0.35 to 0.46 seconds in adults (corrected for heart rate).

If you have long QT syndrome, your ventricles may get a new excitation signal when they should be relaxing. This can lead to a dangerous heart arrhythmia called Torsades de Pointes that can cause your heart to stop.
 

Long QT syndrome symptoms

Long QT syndrome symptoms often don't appear until a serious arrhythmia occurs. When it does, you may experience:

  • Fainting or dizziness
  • A racing or pounding heartbeat (palpitations)
  • Seizures
  • Sudden cardiac arrest

Symptoms typically begin in adolescence, with the highest risk occurring before age 30.

Types of long QT syndrome

Long QT syndrome can be either congenital (present at birth due to genetic factors) or acquired, (developed later due to external factors, such as medication or medical conditions).There are four main types of ASD. The type of ASD depends largely on where the actual hole or opening is located along the septum.

This inherited form of long QT syndrome is caused by genetic changes, often affecting potassium and sodium channels in the heart. Some sub-types are also associated with congenital deafness. Since these genetic changes can be passed down, long QT syndrome can run in families.

Acquired long QT syndrome may result from external factors like electrolyte imbalances, hypothyroidism, hypothermia, and previous cardiovascular problems like stroke and heart failure. Certain medications — including anti-arrhythmics, antibiotics, anti-nausea drugs, antidepressants, and antifungal — can also cause long QT.

Risk factors for long QT syndrome

Your risk of developing long QT syndrome is higher if you have:

  • A family history of long QT
  • Other heart conditions
  • Eating disorders
  • Thyroid disease
  • Fluid loss that leads to electrolyte deficiency
  • Medicines that affect ion channels and heart rhythms

Diagnosing long QT syndrome

The primary test for diagnosing long QT syndrome is an EKG. This painless, non-invasive test uses small sensors placed on your chest to measure your heart’s electrical activity. Your doctor measures the time between phases of the heartbeat to determine whether you have a normal QT interval.

If your EKG results suggest long QT syndrome or if you have a family history of the condition, additional tests — such as blood tests, exercise stress tests, drug challenge tests, or genetic tests — may be needed to confirm the long QT syndrome diagnosis or rule out other conditions.

Long QT syndrome treatment

Treatment for acquired long QT syndrome focuses on addressing the underlying cause or stopping the offending agent.

For congenital long QT syndrome, the common treatment is a beta-blocker, which helps shorten the QT interval and reduce the risk of dangerous arrhythmias like Torsades de Pointes.

In cases where medication isn’t enough, you may need surgery to either implant a pacemaker or defibrillator to regulate the heart rhythm or remove specific nerves that trigger arrhythmias.

Is long QT syndrome curable?

It depends. Acquired long QT syndrome can often be cured by treating the underlying issue, such as correcting electrolyte imbalances or stopping medications that prolong the QT interval.

Inherited long QT syndrome, however, can't be cured, but treatment can effectively reduce the risk of life-threatening arrhythmias.

Understanding arrhythmia

What is a cardiac arrhythmia? William Sauer, MD, cardiologist and cardiac electrophysiologist, explains how we check for serious or harmless arrhythmias and the different treatment options available at Mass General Brigham.

Long QT syndrome self-care

People with long QT syndrome should make lifestyle changes to lower the risk of dangerous arrhythmias:

  • Exercise cautiously: Ask your doctor about a safe exercise routine for your condition.
  • Minimize sudden shocks: Being startled or experiencing strong emotions can raise your heart rate and trigger a dangerous arrhythmia. Avoid sudden noises or other stimuli that may startle you.
  • Avoid QT-prolonging substances: Avoid drugs, foods, and supplements that may further prolong the QT interval.

Things to avoid with long QT syndrome

Certain substances and medications can further prolong the QT interval and increase arrhythmia risk. Check a long QT syndrome "drugs to avoid" list to determine if any of the medications or therapies you take may be putting you at risk.

FAQs

Long QT syndrome occurs when the heart’s electrical system malfunctions due to ion channel dysfunction. Ion channels govern the electrical impulses that control heart contractions. When these ion channels don’t function properly, the heart takes longer to recharge between beats.

Ion channel dysfunction can be hereditary (genetic) or acquired (caused by medications or other medical conditions). The exact cause depends on the type of long QT syndrome a person has.

Some people with long QT syndrome may qualify for disability benefits, depending on the type and the impact it has on their life. Jervell and Lange-Nielsen Syndrome, a severe form of long QT syndrome, is included on the Social Security Administration's Compassionate Allowances List, meaning it qualifies as a disability under federal guidelines, likely due to its association with congenital deafness.

Long QT syndrome does not go away on its own. Acquired long QT may resolve if the underlying issue (such as medication side effects or electrolyte imbalances) is corrected. Inherited long QT syndrome cannot be cured but can be managed with treatment.

Long QT syndrome can cause sudden fainting or seizure, which may make driving unsafe. Driving restrictions vary by state, so it’s best to check with your local DMV for specific guidelines.

Check a long QT syndrome "drugs to avoid" list to determine if any of the medications or therapies you take may be putting you at risk.

Some people with long QT syndrome may experience heart palpitations and chest pain.

It depends on what type of long QT syndrome you have and how well it is managed. The highest risk of sudden cardiac events is within the first two years after the initial symptoms. With proper treatment and lifestyle adjustments, many people with long QT syndrome live full, healthy lives.

There's no cure for genetic long QT syndrome. However, treatments can effectively manage symptoms and significantly reduce the risk of serious complications.

With the right treatment and lifestyle adjustments, most people with LQTS can live a mostly normal life. It’s important to follow a doctor-approved exercises routine, avoid certain medications and supplements that prolong the QT interval, and minimize startling situations that can trigger a cardiac event.

Regular medical follow-ups and proactive management can help you stay safe while leading an active life.