Long QT Syndrome LQTS in Children

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Contact the Inherited Cardiac Arrhythmia Program

Long QT Syndrome (LQTS) is caused by an abnormal electrical signal in the lower parts of your heart. It can lead to developing fast, erratic heartbeats (arrhythmias). People with LQTS may have symptoms such as fainting (syncope) or seizures during exercise or periods of stress/emotional upset.  This is typically caused by abnormally rapid beating of the heart's pumping chambers (ventricular fibrillation). Left untreated, this can be a life-threatening condition that could lead to sudden cardiac death.

LQTS is typically an inherited condition that is passed down through a family.  It may also be caused by particular mediations.  It is very difficult to diagnose this condition without a cardiology team that has expertise in the electrical disorders of the heart as well as a genetics team that can properly evaluate the potential for gene mutations that may lead to this condition. LQTS is fairly rare, it is estimated that about 1 in every 2,500 children born have LQTS.

How Boston Children’s Hospital approaches Long QT Syndrome and other inherited conditions

The Inherited Cardiac Arrhythmia Program at Boston Children’s specializes in family-based services for children with inherited arrhythmias like Long QT syndrome. The program meets with and assesses entire families all at once, usually in one day. Our specialists use genetic testing to identify all family members at risk of developing the condition. Cardiologist Dominic Abrams, MD, director of the Inherited Cardiac Arrhythmia Program, says of this approach:

 “At Boston Children’s, our family focused approach enables one-stop shop where we can diagnose problems and come up with a plan for each member of the family. We know that some family members, especially children, may show no or only very subtle evidence of the disease, making accurate clinical diagnosis challenging. Genetic testing is an increasingly useful mechanism to identify all family members at risk of developing the condition, although the results require detailed evaluation and correlation with clinical findings. Complete medical information is gathered, and genetic testing results, if available, are used to reveal an inherited heart condition."

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