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Short QT syndrome (SQT) is a very rare genetic disease of the electrical system of the heart, and is associated with an increased risk of abnormal heart rhythms and sudden cardiac death. [1] The syndrome gets its name from a characteristic feature seen on an electrocardiogram (ECG) – a shortening of the QT interval .
Sudden arrhythmic death syndrome (SADS) is a sudden unexpected death of adolescents and adults caused by a cardiac arrest. However, the exact cause of the cardiac arrest, and thus the exact cause of death, is unknown. These deaths occur mainly during sleep or at rest. [7] One type of conduction defect known as Brugada syndrome can be ...
An abnormally prolonged QT interval could be due to long QT syndrome, whereas an abnormally shortened QT interval could be due to short QT syndrome. The QTc length is associated with variations in the NOS1AP gene. [21] The autosomal recessive syndrome of Jervell and Lange-Nielsen is characterized by a prolonged QTc interval in conjunction with ...
Long QT syndrome, the most common form of cardiac channelopathy, is characterized by prolonged ventricular repolarization, predisposing to a high risk of ventricular tachyarrhythmias (e.g., torsade de pointes), syncope, and sudden cardiac death.
Jervell and Lange-Nielsen syndrome causes severe sensorineural hearing loss from birth, affecting both ears. Those affected have a prolonged QT interval on an electrocardiogram and are at risk of abnormal heart rhythms (arrhythmias), which can cause dizziness, blackouts, or seizures. [2]
Lown–Ganong–Levine syndrome (LGL) is a pre-excitation syndrome of the heart. Those with LGL syndrome have episodes of abnormal heart racing with a short PR interval and normal QRS complexes seen on their electrocardiogram when in a normal sinus rhythm .
Research in the late 2000s has linked this finding to ventricular fibrillation, particularly in those who have fainted or have a family history of sudden cardiac death. [5] [6] [7] Although there is a significant relationship between ventricular fibrillation and some early repolarization's patterns, the overall lifetime occurrence of idiopathic ventricular fibrillation is exceptionally rare. [8]
A "short-coupled variant of torsade de pointes", which presents without long QT syndrome, was also described in 1994 as having the following characteristics: [20] Drastic rotation of the heart's electrical axis; Prolonged QT interval - may not be present in the short-coupled variant of torsade de pointes