Long QT syndrome factsheet

Introduction 

Long QT syndrome (LQTS) is a heart rhythm disorder that can cause abnormally fast or erratic heartbeats, known as arrhythmias. 

A QT interval is the time it takes the heart muscle to contract and then recover during a heartbeat. If another electrical impulse arrives during a QT interval that is longer than normal, it can cause arrhythmia. 

When arrhythmia happens, less blood is pumped from the heart, which can cause oxygen supply to the brain and body to drop. This can cause fainting or loss of consciousness. LQTS can be life-threatening if not treated quickly.

 Signs and symptoms

LQTS is a condition that people are born with, but it is usually only picked up in older children and young adults.

The most common symptoms of LQTS are:

  • fast or irregular heartbeats 
  • fainting or collapsing 
  • seizures

These symptoms, or episodes, can happen when your child is doing activities that increase their heart rate. 

This can include:

  • exercise like swimming
  • emotional situations
  • responding to sudden loud noises. 

Diagnosis

Diagnosing LQTS can be challenging as many children don't show symptoms early on. A heart specialist or cardiologist can use tests like an echocardiogram (ECG) to check for abnormal heart rhythms. 

Treatment

Treatment focuses on reducing the risk of arrhythmias and includes:

Medications

Beta-blocker medications can help control irregular heartbeats and slow the heart rate.

Lifestyle modifications

Most children will need to avoid activities and sports that put a lot of stress on the body, like swimming. Some children will also need to avoid loud noises and environments where they might be startled or woken up suddenly.

Implantable cardioverter defibrillator

In severe cases, your child may need an implantable cardioverter defibrillator (ICD) inserted. 

An ICD is a device that delivers an electrical shock if it senses that the heart is beating in a potentially dangerous rhythm. This shock can help return the heart to normal rhythm and get it pumping again. 

Automatic External Defibrillator

An Automatic External Defibrillator (AED) is a portable device that gives an electric shock to get the heart beating normally again. They are programmed to automatically analyse your child’s heartbeat and give an appropriate shock.

AEDs are commonly found in public places like:

  • shopping centres
  • airports
  • workplaces
  • parks
  • community centres.

Speak to your child’s cardiologist about whether this device is recommended.

 Management

Medication to avoid

Some medications can make LQTS worse or increase the risk of fast heart rhythms. Check with your child’s doctor or cardiologist before starting new medications.

Genetic testing

If your child is diagnosed with LQTS, immediate family members, like parents and siblings, should be tested to see if they are also at risk. 

Genetic testing is done to see if family members carry a similar or changed gene.

LQTS can affect family members differently. Some family members with LQTS may never have any symptoms, while others might be more severe.

Resources and more information

The Children's Hospital at Westmead

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The Heart Centre for Children at Westmead

Biography
The Heart Centre for Children incorporates cardiology, cardiac surgery, cardiac research and the Edgar Stephens Ward at The Children's Hospital at Westmead. They offer comprehensive information about cardiac (heart) services provided to children.
Related Links
Australian Genetic Heart Disease Registry

title
Australian Genetic Heart Disease Registry

Biography
The Registry was established in 2007 to collect valuable information on Australian families with genetic heart disease to provide a better understanding of these conditions in Australia. They offer detailed information on conditions for patients.
Related Links
CredibleMeds®​​​​​​​

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CredibleMeds®​​​​​​​

Biography
CredibleMeds®​​​​​​​ is an online resource, published in the United States, that promotes the safe use of medicines, including the QTdrugs list of drugs that should be avoided by those with long QT syndrome.
Related Links

Disclaimer

This factsheet is provided for general information only. It does not constitute health advice and should not be used to diagnose or treat any health condition.

Please consult with your doctor or other health professional to make sure this information is right for you and/or your child.

The Sydney Children’s Hospitals Network does not accept responsibility for inaccuracies or omissions, the interpretation of the information, or for success or appropriateness of any treatment described in the factsheet.

© Sydney Children’s Hospitals Network 2024


This factsheet was produced with support from John Hunter Children's Hospital.