Short QT Syndrome


Arrhythmias

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An arrhythmia is an abnormal heart rhythm.  Some arrhythmias cause no symptoms, while others can cause death.  In general arrhythmias are not normal, but they are not all dangerous.  The short QT-interval found in SQTS increases the risk of an arrhythmia originating in either the small chambers of the heart (atria) or in the big chambers of the heart (ventricles). The two arrhythmias of concern in patients with Short QT Syndrome are atrial fibrillation and ventricular fibrillation.

Atrial fibrillation is the arrhythmia that originates in the atria and is characterized by a fast and very irregular pulse. The patient will often be able to feel the irregular heart beats, and may develop other symptoms in terms of dizziness, shortness of breath, chest pain and anxiety. The arrhythmia may terminate spontaneously within minutes or hours, but can become a chronic condition. Many people learn to live with it and it rarely becomes a major problem.  This type of arrhythmia is rarely fatal, and is generally not considered dangerous if the proper medications are taken

Ventricular fibrillation (V-fib) is a life-threatening event. The diagnosis of ventricular fibrillation is difficult to make because the unmonitored patient typically dies before the diagnosis can be made. When patients with Short QT Syndrome gets an implantable defibrillator (ICD) their heart rhythm is constantly monitored, and in a couple of such patients, ventricular fibrillation was registered when they passed out and before they received a shock by the ICD that saved them. In controlled environment in a laboratory electrophysiological studies have also shown that patients with Short QT Syndrome develop ventricular fibrillation much more easily than normal people. These observations suggest that V-fib is the cause of death in patients with Short QT Syndrome that die suddenly.

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