Short QT syndrome

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Revision as of 01:08, 20 February 2025 by Prab (talk | contribs) (CSV import)

Short QT syndrome (SQTS) is a rare genetic disorder that affects the heart's electrical system. It is characterized by a shortened QT interval, which can be detected on an electrocardiogram (ECG). This condition can lead to life-threatening arrhythmias and sudden death.

Symptoms

The primary symptom of Short QT syndrome is a high risk of sudden, life-threatening arrhythmias. These can lead to syncope, or fainting, and sudden death. Some individuals with SQTS may not exhibit any symptoms until an arrhythmia occurs.

Causes

Short QT syndrome is a genetic disorder, caused by mutations in certain genes that regulate the heart's electrical activity. These include the KCNH2, KCNQ1, and KCNJ2 genes. The condition is inherited in an autosomal dominant pattern, meaning an affected individual has a 50% chance of passing the disorder onto their children.

Diagnosis

Diagnosis of Short QT syndrome is based on the measurement of the QT interval on an ECG. A QT interval of less than 360 milliseconds is generally considered indicative of SQTS. Genetic testing can also be used to confirm a diagnosis.

Treatment

Treatment for Short QT syndrome primarily involves managing the risk of arrhythmias. This can include the use of beta blockers, implantable cardioverter defibrillators (ICDs), and in some cases, surgical procedures.

Prognosis

With appropriate management, individuals with Short QT syndrome can lead normal lives. However, the risk of sudden death remains, making regular monitoring and treatment essential.

See also



Cardiovascular disease A-Z

Most common cardiac diseases

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Genetic disorders relating to deficiencies of transcription factor or coregulators
(1) Basic domains
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  • Sinus Rhythm Labels
    Sinus Rhythm Labels
  • QT Interval
    QT Interval
  • Short QT Syndrome
    Short QT Syndrome
  • Labelled DDD ICD
    Labelled DDD ICD
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