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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 .
In 2016, the WHO recorded 56.7 million deaths [3] with the leading cause of death as cardiovascular disease causing more than 17 million deaths (about 31% of the total) as shown in the chart to the side. In 2021, there were approx. 68 million deaths worldwide, as per WHO report.
In 20% of cases, no cause of death can be found, even after extensive examination. [13] Sudden arrhythmic death syndrome in alcohol abuse is a significant cause of death among heavy drinkers, characterized by older age and severe liver damage, highlighting the need for family screening for heritable channelopathies. [4]
Abnormalities in this system occur in relatively rare genetic diseases such as Long QT syndrome, Brugada syndrome, and Catecholaminergic polymorphic ventricular tachycardia, all associated with sudden death. Consequently, autopsy-negative sudden cardiac deaths (no physical abnormalities identified) may comprise a larger part of the ...
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.
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 ...
Jervell and Lange-Nielsen syndrome (JLNS) is a rare type of long QT syndrome associated with severe, bilateral sensorineural hearing loss. [2] Those with JLNS are at risk of abnormal heart rhythms called arrhythmias , which can lead to fainting , seizures , or sudden death.
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 ...