The Short QT Syndrome

Gollob, Michael H.; Redpath, Calum J.; Roberts, Jason D.

doi:10.1016/j.jacc.2010.09.048

Cited by 274 publications

(68 citation statements)

References 45 publications

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“…However, diagnosis of SQTS may be challenging when QTc are of borderline values, and some patients with true SQTS may have longer QTc duration 4, 10, 11, 12. Moreover, there is a large overlapping between healthy subjects and SQTS, which is illustrated by long‐term follow‐up population‐based studies showing no adverse prognosis among subjects with a QTc <300 to 360 ms 15, 16, 19.…”

Section: Discussionmentioning

confidence: 99%

“…It is a very exceptional syndrome with only some tens of cases reported in the literature so far 4, 5…”

Section: Introductionmentioning

confidence: 99%

“…A diagnostic value of 300 ms,1, 7 then of 320 ms8 for the QT interval and of 340 ms for the corrected QT (QTc)9 had been initially proposed, although some symptomatic patients with SQTS may present with longer QTc interval 4, 10, 11, 12. A diagnostic score was proposed in 2011, including QTc value, and clinical and family history, demonstrating excellent sensitivity,4 although this score has not gained wide acceptance because of some limitations 13, 14. SQTS should be currently diagnosed in the presence of a QTc ≤330 to 360 ms according to recent guidelines 2, 3…”

Section: Introductionmentioning

confidence: 99%

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Shortening of the Short Refractory Periods in Short QT Syndrome

Rollin

Gandjbakhch

Giustetto

et al. 2017

JAHA

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BackgroundDiagnosis of short QT syndrome (SQTS) remains difficult in case of borderline QT values as often found in normal populations. Whether some shortening of refractory periods (RP) may help in differentiating SQTS from normal subjects is unknown.Methods and ResultsAtrial and right ventricular RP at the apex and right ventricular outflow tract as determined during standard electrophysiological study were compared between 16 SQTS patients (QTc 324±24 ms) and 15 controls with similar clinical characteristics (QTc 417±32 ms). Atrial RP were significantly shorter in SQTS compared with controls at 600‐ and 500‐ms basic cycle lengths. Baseline ventricular RP were significantly shorter in SQTS patients than in controls, both at the apex and right ventricular outflow tract and for any cycle length. Differences remained significant for RP of any subsequent extrastimulus at any cycle length and any pacing site. A cut‐off value of baseline RP <200 ms at the right ventricular outflow tract either at 600‐ or 500‐ms cycle length had a sensitivity of 86% and a specificity of 100% for the diagnosis of SQTS.ConclusionsPatients with SQTS have shorter ventricular RP than controls, both at baseline during various cycle lengths and after premature extrastimuli. A cut‐off value of 200 ms at the right ventricular outflow tract during 600‐ and 500‐ms basic cycle length may help in detecting true SQTS from normal subjects with borderline QT values.

show abstract

Section: Discussionmentioning

confidence: 99%

“…It is a very exceptional syndrome with only some tens of cases reported in the literature so far 4, 5…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Shortening of the Short Refractory Periods in Short QT Syndrome

Rollin

Gandjbakhch

Giustetto

et al. 2017

JAHA

View full text Add to dashboard Cite

show abstract

“…Short QT syndrome is a very exceptional channelopathy carrying a significant risk of sudden cardiac death due to malignant ventricular arrhythmias 3. SQT is caused by an excessive abbreviation of repolarization duration, mainly due to some inherited gain of function in potassium channels 3.…”

Section: Discussionmentioning

confidence: 99%

“…SQT is caused by an excessive abbreviation of repolarization duration, mainly due to some inherited gain of function in potassium channels 3. SQT is diagnosed if corrected QT is ≤330 ms or 360 ms together with clinical events 4.…”

Section: Discussionmentioning

confidence: 99%