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The treatment for short QT syndrome is aimed at preventing abnormal heart rhythms and reducing the risk of sudden cardiac death. It has been difficult to experimentally test potential treatments as the condition is very rare, so the evidence for treatment effectiveness comes largely from consensus opinion. [ 1 ]
The risk of arrhythmias is higher for those with Jervell and Lange-Nielsen syndrome than other forms of long QT syndrome. [10] Although this risk is dependent on the underlying genetic defect and degree of QT prolongation, without treatment more than 50% of those affected will die before the age of 15. [11]
Shell nail syndrome; Shone's syndrome; Short anagen syndrome; Short bowel syndrome; Short man syndrome; Short QT syndrome; Short rib – polydactyly syndrome; SHORT syndrome; Shwachman–Diamond syndrome; Shy-Drager syndrome; Sick building syndrome; Sick sinus syndrome; Silver–Russell syndrome; Simpson–Golabi–Behmel syndrome; Singleton ...
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 ...
Torsades de pointes is associated with long QT syndrome, a condition whereby prolonged QT intervals are visible on an ECG. Long QT intervals predispose the patient to an R-on-T phenomenon , wherein the R-wave, representing ventricular depolarization, occurs during the relative refractory period at the end of repolarization (represented by the ...
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.
Examples of these inherited arrhythmia syndromes include long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and short QT syndrome (SQTS). Many are also associated with environmental or neurogenic triggers such as response to loud sounds that can initiate lethal arrhythmias.
Romano–Ward syndrome is a descriptive term for a group of subtypes of long QT syndrome, specifically subtypes LQT1-6 and LQT9-16. [8] Several subtypes of Romano–Ward syndrome have been described based on the underlying genetic variant. [5] These subtypes differ in clinical presentation and their response to treatment.