Syndrome, long QT

An inherited defect in the heart's rhythm. In the U.S. it is estimated that 4,000 American children and young adults die yearly of the long QT syndrome. It is a common cause of sudden death among school athletes, as well as the cause of many unexplained drownings. The first sign may unfortunately be sudden cardiac death. The heart muscle abruptly goes into fibrillation, beating too fast and so ineffectively that the blood stops circulating. The heart has to be defibrillated (shocked back into a normal rhythm) within a few minutes if the person is to survive. The name of the syndrome comes from the QT segment in the tracing on the electrocardiogram (ECG). This segment lasts slightly longer in the syndrome than normal. The heart takes longer to recharge itself between beats. Certain conditions can trigger an abnormal cardiac rhythm. The abnormal rhythm results in syncope (fainting spells), dizziness, palpitations, seizures or sudden death. Among the known triggers are intense physical exercise, swimming, being suddenly startled or badly frightened. The cause of the long QT syndrome is a mutation in a gene that regulates the heart's electrical system. The mutation causes a defect in heart muscle structures called ion channels that predisposes the heart to lapse into a very rapid, ineffectual rhythm called fibrillation. The mutation responsible for the long QT syndrome is inherited as an autosomal dominant, which means that the mutant gene is on a non-sex chromosome and that each child of an affected parent has a 1 in 2 (50%) chance of inheriting it. The diagnosis of the long QT syndrome can be made by electrocardiogram (ECG) in about 50% of cases. However, in about 10% of cases, the QT interval on the initial ECG looks normal and in another 40% the QT interval is not sufficiently prolonged to permit a clear-cut diagnosis. The long QT syndrome can also be detected after a person dies by a molecular genetic analysis of their tissue. Early diagnosis is essential. Drugs called beta-blockers can help to maintain a normal heart rhythm in 90% of cases. In the remaining cases, a pacemaker can be implanted to set the heart's rhythm or an automatic defibrillator can be put in that can detect and correct an abnormal heart rhythm. Children with long QT syndrome should not engage in competitive sports. Noncompetitive activities are generally all right. However, they should stop whatever they are doing if they experience any possible symptoms of the long QT syndrome. The long QT syndrome is one of the sudden arrhythmia death syndromes (SADS). The SADS Foundation is at 508 East South Temple, Suite 20, Salt Lake City, Utah 84102; telephone numbers (800) STOP-SAD (786-7723) and (801) 531-0937.

Medical dictionary. 2011.

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