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Potentially life-threatening emergency
Short QT syndrome
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Other Resources UpToDate PubMed
Potentially life-threatening emergency

Short QT syndrome

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Contributors: Benjamin L. Mazer MD, MBA, Ryan Hoefen MD, PhD
Other Resources UpToDate PubMed

Synopsis

A rare cause of syncope, atrial fibrillation, and arrhythmias. A shortened QT interval is defined as less than 340-360 milliseconds. Shortened QT intervals may be secondary to many causes, such as sepsis or electrolyte disturbances; however, the short QT syndrome typically refers to a short QT interval that is genetic in etiology. Both sporadic and familial cases have been described. Patients typically present with atrial fibrillation or syncope at an unusually young age. Others are diagnosed due to a family history of sudden cardiac death.

Multiple genes have been implicated in the syndrome including KCNH2, KCNQ1, and KCNJ2. These genes code for cardiac ion channels.

Many patients with short QT syndrome receive cardioverter-defibrillator implantation. Antiarrhythmic drugs are sometimes used, but there is incomplete evidence for their effectiveness.

For more information, see OMIM.

Codes

ICD10CM:
I45.9 – Conduction disorder, unspecified

SNOMEDCT:
698272007 – Short QT syndrome

Differential Diagnosis & Pitfalls

Best Tests

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References

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Last Updated: 03/29/2017
Copyright © 2019 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Short QT syndrome
Print 1 Images
Short QT syndrome : AV conduction abnormality, Heart palpitations, Syncope, Cardiac dysrhythmia, Presyncope
Copyright © 2019 VisualDx®. All rights reserved.