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Long QT Syndrome

Long QT syndrome (LQTS) is a type of arrhythmia that can lead to an abnormally fast or chaotic heartbeat. This fast heartbeat can cause heart palpitations, fainting, seizures, and rarely, sudden cardiac arrest. Getting treatment for LQTS from the experts at UPMC can prevent serious complications.

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What Is LQTS? 

LQTS is a problem with the heart’s electrical system that causes the heart to beat in a fast, chaotic way. The condition gets its name from electrocardiogram (ECG) graphs that show a prolonged QT interval — meaning that your heart is taking longer than normal to “recharge” after each beat.

How common is LQTS?

LQTS is a rare disorder that can occur at any age.

What are the types of LQTS?

There are two types of LQTS:

  • Inherited LQTS – Caused by an altered gene that may have come from a parent.
  • Acquired LQTS – Caused by medication, mineral deficiencies, or other health conditions.

Sometimes, a combination of genetics and other factors can lead to LQTS.

What causes LQTS?

Inherited LQTS is caused by a genetic mutation that affects the levels of minerals that generate electricity inside the heart. Acquired LQTS can be caused by health conditions, medication, and mineral imbalances in the body.

What triggers long QT syndrome?

Acquired LQTS can be triggered by several conditions, including:

  • Electrolyte imbalances that affect potassium, magnesium, or blood calcium levels.
  • History of heart disease or stroke.
  • Malnutrition.
  • Medications.
  • Problems with the thyroid gland.

What are LQTS risk factors and complications?

LQTS risk factors

Some people are more likely to get LQTS than others. This includes people who:

  • Have a family history of LQTS.
  • Have a disorder that leads to malnutrition.
  • Have a history of unexplained sudden cardiac arrest.
  • Take drugs known to cause LQTS, such as some antibiotics, antifungals, or water pills.

Complications of LQTS

LQTS can lead to fainting spells, seizures, or, rarely, sudden cardiac arrest.

The heart rhythm changes from LQTS often correct on their own — but can be deadly when they don't. Young people with LQTS are at higher risk of sudden cardiac arrest.

Treatment for LQTS helps prevent complications.

How can I prevent LQTS?

Because LQTS is an inherited disorder or is triggered by other medical conditions, there is nothing you can do to prevent LQTS.

Do I need genetic testing for LQTS?

If you have a family member with LQTS, your doctor may also suggest genetic testing.

Before the test, a genetic counselor will talk to you about the risks and benefits of DNA testing. They'll also tell you about any limits in translating the results.

If you carry a gene change for LQTS, there's a risk that you may pass it along to your children. Doctors may suggest that your parents, siblings, and children also have the test.

Your care team can talk with family members about their LQTS risks and DNA testing.

What Are the Signs and Symptoms of LQTS?

Not everyone with LQTS has symptoms. Sometimes LQTS is found on an ECG or genetic testing that is done for other reasons.

When people have symptoms, the most common are:

Fainting spells often come on suddenly, but sometimes people have warning symptoms such as blurred vision, lightheadedness, or weakness. People with LQTS may experience fainting in response to emotions such as excitement, anger, or fear.

When should I see a doctor about my LQTS symptoms?

If you experience unexplained fainting or a seizure, you should go to the Emergency Department or dial 911. Make an appointment with your doctor if you have heart palpitations.

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How Do You Diagnose LQTS?

If your doctor suspects you have LQTS, they'll ask you about your symptoms, medical history, and family history.

What to expect during your visit

Your doctor will do a physical exam and may order tests to confirm a LQTS diagnosis. Your doctor or nurse will tell you when to expect your test results and will call you when they're available.

Tests to diagnose LQTS

We offer a full range of tests for LQTS, including:

  • Echocardiogram – This noninvasive test uses sound waves to create images of your heart.
  • Electrocardiogram (ECG) – This noninvasive test measures the electrical activity in your heart.
  • Electrophysiology (EP) study – This test uses a special catheter that is threaded through your blood vessels and into your heart to map electrical impulses.
  • Event recorder – This portable device is similar to a Holter monitor. However, it only records when you are having symptoms.
  • Exercise stress test – This test will find a long QT interval that occurs only during exercise.
  • Holter monitor – This wearable ECG device records your heart’s activity throughout the day.

What are the criteria for a LQTS diagnosis?

Your doctor will review your test results to see if your heart’s electrical system is taking longer than average to “recharge” after each beat. This recharging time is known as the QT interval. If you are diagnosed with LQTS, your ECG results will show a prolonged QT interval.

LQTS prognosis

Getting early treatment can help you manage your LQTS and may prevent dangerous complications. Your doctor will discuss your condition and prognosis with you.

What is the life expectancy of long QT syndrome?

People who get proper treatment for LQTS have a low death rate and can live a full life. However, if LQTS is untreated, there is a higher risk of death — especially within the first two years after the first cardiac event.

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How Do You Treat LQTS?

The goal of treatment for LQTS is to avoid sudden cardiac arrest, fainting spells, and fatal heart rhythms.

Your doctor will plan your treatment based on how severe your LQTS symptoms are and your risk for life-threatening problems.

Lifestyle changes

Your doctor may ask you to make changes to your habits and routines to reduce the risk of LQTS episodes.

What should I avoid if I have LQTS?

  • Intense emotions and stress.
  • Medications and supplements that may trigger LQTS.
  • Loud noises and situations where you may be startled.
  • Risky activities, such as swimming alone.

Medicine to treat LQTS

Your doctor may prescribe beta-blockers to slow your heart rate, or a combination of a beta blocker and heart rhythm medication to shorten your QT interval.

Surgery for LQTS

Surgery may help you manage LQTS, especially if symptoms are severe and other treatments have not worked. Surgical procedures include:

  • Implantable cardioverter defibrillator (ICD) – An ICD continuously tracks your heart rate. It can send a shock if needed to keep your heart pumping normally.
  • Left cardiac sympathetic denervation (LCSD) – LCSD involves removing the nerves on the left side of the spine in the chest to improve heart rhythm control. This procedure is only recommended for people who cannot take LQTS medications.

How effective is treatment?

The goal of LQTS treatment is to reduce your risk of sudden cardiac death. Some causes of LQTS can be treated by addressing the underlying problem. Medications and ICDs are effective LQTS treatments. However, even with treatment, you may not have a normal QT result on an ECG.

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Why Choose UPMC for LQTS Care?

UPMC doctors have special expertise in treating LQTS. Our team:

  • Helps you get answers about your genetic heart disease.
  • Provides a team of heart doctors for both children and adults, genetic counselors, heart surgeons, and other experts trained in LQTS care.
  • Takes a thorough, family-based approach to caring for people with LQTS.

Last reviewed by a UPMC medical professional on 2024-10-01.