The Short QT Syndrome: Proposed Diagnostic Criteria

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• 作者：Michael H. Gollob MD ; ^† ; ; ^{mgollob@ottawaheart.ca} ; Calum J. Redpath MBChB ; PhD^† ; ; Jason D. Roberts MD
• 关键词：arrhythmia ; genetics ; short QT syndrome
• 刊名：Journal of the American College of Cardiology
• 出版年：2011
• 出版时间：15 February 2011
• 年：2011
• 卷：57
• 期：7
• 页码：802-812
• 全文大小：814 K

文摘

Objectives

We aimed to develop diagnostic criteria for the short QT syndrome (SQTS) to facilitate clinical evaluation of suspected cases.

Background

The SQTS is a cardiac channelopathy associated with atrial fibrillation and sudden cardiac death. Ten years after its original description, a consensus regarding an appropriate QT interval cutoff and specific diagnostic criteria have yet to be established.

Methods

The MEDLINE database was searched for all reported cases of SQTS in the English language, and all relevant data were extracted. The distribution of QT intervals and electrocardiographic (ECG) features in affected cases were analyzed and compared to data derived from ECG analysis from general population studies.

Results

A total of 61 reported cases of SQTS were identified. Index events, including sudden cardiac death, aborted cardiac arrest, syncope, and/or atrial fibrillation occurred in 35 of 61 (57.4 % ) cases. The cohort was predominantly male (75.4 % ) and had a mean QT_c value of 306.7 ms with values ranging from 248 to 381 ms in symptomatic cases. In reference to the ECG characteristics of the general population, and in consideration of clinical presentation, family history, and genetic findings, a highly sensitive diagnostic scoring system was developed.

Conclusions

Based on a comprehensive review of 61 reported cases of the SQTS, formal diagnostic criteria have been proposed that will facilitate diagnostic evaluation in suspected cases of SQTS. Diagnostic criteria may lead to a greater recognition of this condition and provoke screening of at-risk family members.