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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 ]
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 ...
Timothy syndrome, which may or may not occur with syndactyly; Short QT syndrome or Brugada syndrome; Long QT syndrome or other arrhythmia without additional symptoms. [2] CACNA1C-related disorders are inherited in an autosomal dominant manner. [2]
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]
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.
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 ...
Those affected by arrhythmogenic cardiomyopathy may not have any symptoms at all despite having significant abnormalities in the structure of their hearts. [6] If symptoms do occur, the initial presentation is often due to abnormal heart rhythms (arrhythmias) which in arrhythmogenic cardiomyopathy may take the form of palpitations, or blackouts. [7]
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.