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A new formal diagnostic approach to short QT syndrome (SQTS) will improve its clinical understanding, and the ability of clinicians to evaluate potentially at-risk family members, according to a study published recently in the Journal of the American College of Cardiology.1

Researchers led by Dr Michael Gollob, University of Ottawa Heart Institute, Ontario, conducted a search of the MEDLINE electronic database for relevant articles published before May 2010.  Published cases were considered eligible for inclusion in the study if the report provided details regarding the age, sex, clinical history, and QT interval of the affected patient.


Reproduced courtesy of J Am Coll Cardiol

The search strategy yielded 197 articles that were reviewed for reported cases of SQTS. A total of 15 articles described unique cases of SQTS with inclusion of relevant clinical details. From these articles, 61 separate SQTS cases were reviewed. A subset of patients were reported in multiple articles; however, care was taken to ensure that these subjects were only included once in the study.

The authors’ diagnostic criteria are divided into four different components including ECG, clinical history, family history, and genotype. In order to be eligible to receive points in the latter 3 sections, a minimum of 1 point must be received from the ECG criteria. An overall score of 4 points or greater indicates a high-probability diagnosis of SQTS, whereas 2 points or less makes a diagnosis of SQTS low probability. Patients with a score of 3 points are considered to have an intermediate probability of having SQTS.

Of the 61 SQTS cases studied, 58 (95%) would receive a diagnostic score indicating high-probability SQTS.  The authors encourage clinicians to remain wary of benign variants of QTc abbreviation, analogous to the challenges of evaluating prolonged QT intervals in consideration of LQTS. Treatment consideration should be reserved for those cases receiving a high-probability score, whereas medical surveillance or expert opinion considered for intermediate- or low-probability cases, they say.


1 Gollob MH, Redpath CJ, Roberts JD. The Short QT Syndrome: Proposed Diagnostic Criteria.  J Am Coll Cardiol 2011:57;802-12.

Published on: May 6, 2011

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