Short QT syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Short QT syndrome(SQTS) is associated with an increased risk of atrial fibrillation, syncope and sudden death due to ventricular fibrillation.

Natural History

The symptoms of SQTS usually develop between the second and fourth decade of life and start with symptoms such as syncope and palpitations. The primary manifestations of SQTS are palpitations, syncope, atrial fibrillation, and cardiac arrest. Without treatment, the patient will develop a worsening of arrhythmia, which may eventually lead to cardiac arrest. The circumstances in which symptoms arise in SQTS patients are highly variable. According to a few studies, SQTS patients can be asymptomatic in a significant proportion of the study population and get diagnosed only with a strong familial predisposition^[1].

complications

Complications can result due to SQTS.

atrial fibrillation
ventricular fibrillation
Sudden cardiac death

Prognosis

Long term prognosis has not been assessed due to the unavailability of data. Severe shortening of QT values in adults ≤300 msec and in young patients with median QTc of 285 msec have increased risk of SCD either at rest or in sleep^[2].

References

↑ Rudic B, Schimpf R, Borggrefe M (2014). "Short QT Syndrome - Review of Diagnosis and Treatment". Arrhythm Electrophysiol Rev. 3 (2): 76–9. doi:10.15420/aer.2014.3.2.76. PMC 4711567. PMID 26835070.
↑ Villafañe J, Atallah J, Gollob MH, Maury P, Wolpert C, Gebauer R; et al. (2013). "Long-term follow-up of a pediatric cohort with short QT syndrome". J Am Coll Cardiol. 61 (11): 1183–91. doi:10.1016/j.jacc.2012.12.025. PMID 23375927.

[pmid26835070-1] Rudic B, Schimpf R, Borggrefe M (2014). "Short QT Syndrome - Review of Diagnosis and Treatment". Arrhythm Electrophysiol Rev. 3 (2): 76–9. doi:10.15420/aer.2014.3.2.76. PMC 4711567. PMID 26835070.

[pmid23375927-2] Villafañe J, Atallah J, Gollob MH, Maury P, Wolpert C, Gebauer R; et al. (2013). "Long-term follow-up of a pediatric cohort with short QT syndrome". J Am Coll Cardiol. 61 (11): 1183–91. doi:10.1016/j.jacc.2012.12.025. PMID 23375927.

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