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Updated: Jan 11, 2026

Determining the Likelihood of Variant Pathogenicity Using Amino Acid-level Signal-to-Noise Analysis of Genetic Variation
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Cardiac Channelopathies in the Pediatric Patient: Short QT Syndrome

Reina Bianca Tan1, Maully J Shah2

  • 1Division of Cardiology, Department of Pediatrics, NYU Langone Health and Hassenfeld Children's Hospital, 403 East 34th Street, Level 3, New York, NY 10016, USA.

Cardiac Electrophysiology Clinics
|November 8, 2025
PubMed

Insights

Short QT Syndrome is a rare genetic disorder causing dangerous heart arrhythmias. Management focuses on preventing sudden cardiac arrest through ICDs and antiarrhythmic drugs like quinidine.

Area of Science:

  • Cardiology
  • Genetics
  • Electrophysiology

Background:

  • Short QT Syndrome (SQTS) is a rare inherited arrhythmia disorder.
  • Characterized by accelerated cardiac repolarization and a short QT interval on ECG.
  • Associated with a high risk of life-threatening atrial and ventricular arrhythmias, including sudden cardiac arrest.

Keywords:
Cardiac implantable electronic deviceChannelopathyImplantable cardioverter defibrillatorShort QT

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