Abstract

BackgroundShort QT syndrome is a rare, inherited channelopathy associated with sudden cardiac arrest (SCA) but the characteristics and prognosis of short QT interval (SQTI) in Korean patients remain unclear. This study aimed to determine the clinical characteristics and outcomes of SQTI in a Korean population.MethodsConsecutive patients with SQTI from January 1999 to March 2019 in three university hospitals in South Korea were recruited. SQTI was defined as a Bazett’s formula-corrected QT interval (QTc) ≤ 340 ms in serial electrocardiograms. Age- and sex-matched patients with a normal QTc and without overt cardiovascular disease were included in a 1:4 ratio. Clinical and ECG features and outcomes were compared between patients with and without SQTI.Results34 patients with SQTI [age, 23.5 (21–30.5) years; 31 male] were followed up for 4.8 (2.0–7.8) years. Early repolarization, tall T wave, and U wave were significantly more frequent in patients with SQTI than in those without SQTI. QT dispersion [44.0 (28.0–73.0) vs. 20.0 (12.0–35.0) ms, P < 0.001] was significantly wider and heart rate [52.0 (47.0–58.0) vs. 70.0 (62.3–84.0)/min, P < 0.001] was significantly slower in patients with SQTI than in those without. Atrial fibrillation (AF, 11.8% vs. 2.2%, P = 0.030) and ventricular arrhythmia (VA)/SCA (8.7% vs. 0%, P = 0.007) were significantly more frequent in patients with SQTI than in those without. SQTI was significantly associated with AF [odds ratio, 5.911; 95% confidence interval, 1.257–27.808; P = 0.025] and VA/SCA.ConclusionsIn this subset of Korean population, SQTI was associated with AF and VA/SCA.

Highlights

  • Short QT syndrome is a rare, inherited channelopathy associated with sudden cardiac arrest (SCA) but the characteristics and prognosis of short QT interval (SQTI) in Korean patients remain unclear

  • The medications in the SQTI and normal QT groups were not significantly different except for digoxin (5.9% vs. 0.7%, P = 0.041); drugs that were known to shorten QT interval were not administered to patients in the SQTI and normal QT groups

  • Main findings The main findings of the present study are as follows: (1) the prevalence of SQTI was 0.001%; (2) QT dispersion was significantly wider and heart rate was significantly slower in patients with SQTI than in those with a normal QT interval

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Summary

Introduction

Short QT syndrome is a rare, inherited channelopathy associated with sudden cardiac arrest (SCA) but the characteristics and prognosis of short QT interval (SQTI) in Korean patients remain unclear. This study aimed to determine the clinical characteristics and outcomes of SQTI in a Korean population. Short QT syndrome (SQTS) is a rare, life-threatening, inherited channelopathy associated with ventricular arrhythmia (VA) and sudden cardiac arrest (SCA) [1]. The first reported clinical case was of familial short QT interval < 300 ms accompanied by arrhythmia [2]. Kim et al BMC Cardiovasc Disord (2021) 21:17

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