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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 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]
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.
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]
SUDC is rare, with a reported incidence in the United States of 1.2 deaths per 100,000 children, compared to 54 deaths per 100,000 live births for SIDs. There are approximately 400 deaths per year of SUDC in the U.S, with over 200 of these cases being the children aged 1–4 years. [3] SUDC deaths have occurred at the following sites: [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 ...
This could help elucidate the cause of death in the patient. Other physical signs or symptoms can help determine the potential cause of the cardiac arrest. [30] Below is a chart of the clinical findings and signs/symptoms a person may have and potential causes associated with them.
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.