Long QT syndrome occurs due to a disturbance in the electrical system of the heart. Normally, ions move in and out of cardiac cells to keep the heart beating regularly. When there is a defect in either the ion channels or the muscle fibers, an irregular heartbeat can develop, which can be life-threatening if left untreated. This syndrome specifically affects the lower chambers of the heart and gets its name from the way it presents on an electrocardiogram. Long QT syndrome is rare -- it affects only about one in every 5,000 people in the United States.
Long QT syndrome has electrical and muscular components. Normally, electricity flows through the nodes of the heart and triggers the muscle fibers to contract by using ion channels. These ion channels maintain the proper electrical flow by carrying calcium, sodium, and potassium through cardiac cells. When there is a problem with these channels, the flow of electricity is interrupted, which can lead to dangerous ventricular arrhythmias.
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