Long QT Syndrome - The Physicians Guide to LQTS

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Long QT Syndrome: General Information for Physicians

___ Long QT syndrome is a cardiac ion channel disease that predisposes its carriers to lethal ventricular arrhythmias. In individuals who have long QT syndrome, the time required for ventricular repolarization is prolonged. This creates a problem because it makes the heartbeat electrically unstable and especially vulnerable to the arrhythmias Torsades de Pointes and ventricular fibrillation. The exact mechanism causing this instability is still not fully understood.

___ The symptoms of long QT syndrome are a direct effect of the arrhythmias it causes. Both Torsades de Pointes and ventricular fibrillation can result in syncope, seizures, shortness of breath, palpitations, and even death. Any one of these events may be an indicator of an individual who has long QT syndrome. Generally these symptoms occur within seconds of a long QT triggering event. A long QT triggering event is any scenario that stimulates the release of norepinephrine into the blood. Norepinephrine release is associated with the "fright, fight, flight" response, but even simple things such as a phone or alarm clock ringing during the night can be enough to cause problems for long QT sufferers.

___ Current research indicates that long QT syndrome is exhibited in about 1 in every 4,000 individuals in the United States. Although this seems like a relatively low percentage of the population, it is in fact more prevalent than many other more well known diseases such as cystic fibrosis and phenylketonuria. Long QT syndrome owes its relatively high prevalence in the population to its order of genetic inheritance. It is passed on between generations as an autosomal ___ dominant.

___Untreated long QT syndrome can spell out major problems. Fortunately, with proper medical treatment, the 10 year mortality rate for long QT syndrome carriers can be lowered from 10% to less than 1%. This statistic highlights the importance of properly identifying and treating people with long QT syndrome. For more information about identifying potential carriers of long QT syndrome, please see the next section of our website entitled "Recognizing Long QT Syndrome" by clicking here.

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