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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 ]
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]
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 ...
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 ...
Treatment depends on the type of cardiomyopathy and condition of disease, but may include medication (conservative treatment) or iatrogenic/implanted pacemakers for slow heart rates, defibrillators for those prone to fatal heart rhythms, ventricular assist devices (VADs) for severe heart failure, or catheter ablation for recurring dysrhythmias ...
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.
Cardiac arrhythmias are another possible cause. Arrhythmias such as asystole or bradycardia are more likely in children, in contrast to ventricular fibrillation or tachycardia as seen in adults. [30] Additional causes of sudden unexplained cardiac arrest in children include hypertrophic cardiomyopathy and coronary artery abnormalities. [160]