Long Q-T Syndrome (LQTS) is a hereditary disorder of the heart’s electrical rhythm that normally affects children to young adults. The disorder is found in the electrical signal emitted from the heart. The rhythm can be measured on an electrocardiogram, or ECG. The ECG shows a picture of a waveform. There are five different parts of the waveform, distinguished by the letters P, Q, R, S, T. “The Q-T interval represents the time for electrical activation and inactivation of the ventricles, the heart's lower chambers.” The interval between the Q-T can be measured in fractions of a second to see if the time difference is irregular. When the interval is too long, it is called a prolonged Q-T intermission.
Over fifty thousand people suffer from LQTS and about 3,000 die per year. Most of the deaths are children and teenagers.
There are four different mutations of the gene that have been discovered. Type 1 LQTS is the result of a mutation in the protein that ensures potassium properly reaches the heart cells after the heart muscles contract. Type 2 LQTS is the direct result of a mutation in the gene known as the HERG gene. The HERG gene has imprinted in the gene the potassium channel. The potassium channel in the HERG gene tells the potassium where to flow.



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