The Long-QT Syndrome

Abstract

The heart is a pump that is stimulated by electrical signals to beat between about 50 to 180 times per minute, depending upon the individual’s level of activity and emotional state. The heart itself is made up of millions of cells that generate repetitive sequences of electrical discharges (referred to in medicine as depolarization) and recharging (repolarization) that produce each heart beat. Electrical activity of the heart cells is produced by the synchronized movement of sodium, potassium, and calcium ions into and out of the cells. ### Long-QT Syndrome Long-QT Syndrome (LQTS) is a disorder of the electrical activity of the heart that can result in blackouts (fainting, also known as syncope) or sudden cardiac death. It occurs infrequently and is caused either by an inherited genetic condition (inherited form of LQTS) or by some prescription medications (acquired form of LQTS). QT refers to the interval on ECG between the beginning of the heart’s electrical discharge through its electrical recovery (see Figure 1). Figure 1 illustrates how the QT interval is measured on the ECG. Schematic diagram of the electrical activity of one heart beat recorded by an ECG. The electrical discharge (depolarization) of the ventricle is represented by the QRS complex, with the T wave reflecting electrical recovery (repolarization). The QT is the time interval from the beginning of the QRS complex to the end of the T wave, and is generally less than 0.44 seconds in normal individuals. In subjects with the long-QT syndrome, the QT is usually longer than 0.44 seconds. Delayed electrical recovery within the heart can be associated with very dangerous, rapid, and chaotic heart rhythms (medical terms for these abnormal heart rhythms are …