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Prolonged QT syndrome is an electrical abnormality of the heart.  It occurs when the heart fails to repolarize correctly after each heartbeat.  Typically the syndrome is inherited.  Sudden unexpected death often occurs in children and teenagers due to a very fast abnormal heart rhythm known as torsade de pointes.  It often goes undiagnosed in families until one or more members dies suddenly or has frequent fainting episodes.  It is often mistaken for epilepsy.  Other causes of prolonged QT include medications and electrolyte abnormalities.  In the case of medication, the interval should shorten once the patient has been taken off of it and there should be no permanent damage.  Electrolyte abnormalities create a problem for people without a history of long QT if the deficiency is severe.  They more often create a problem for people with a history of long QT.

Sudden death often occurs during strenuous activity, sleep, and after being startled by a loud noise.  Although these are the most common, it can occur at any time.  Symptoms are not often present, so it is important for anyone who has a history of a sudden death in the family to be tested.  

Prolonged QT is diagnosed through an EKG.  Often the QT interval is borderline, and may be misdiagnosed.  For this reason it is important to have several done in order to rule long QT out.  A borderline QT interval has also been known to cause problems.  

Prolonged QT is typically treated with a beta-blocker.  If it cannot be controlled despite medication or if medication cannot be tolerated, an implantable defibrillator and/or pacemaker must be inserted. 

I have established a long QT eGroup, similar to LISTSERV, for people who would like to meet others with this condition.  Please feel free to join by going to www.egroups.com/group/longqt 

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