Short QT syndrome epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Sumanth Khadke, MD[2]
Overview
Since the syndrome was first described in 2000, < 30 cases have been identified. The median age of presentation in adults is 30 years. The overall prevalence of SQTS is is 0.02 to 0.1% in adults and 0.05% in the pediatric population. In Japanese cohort study of 114,334 patients, prevalence was found to be 0.37 %. In an American cohort of 46,129 prevalence was nearly 2 percent. The Swiss cohort of 1767 patients and Finnish cohort of 10,822 patients had a prevalence of 1 and 0.4 percent respectively.There is a higher male predominance with higher penetrance despite it being autosomal dominant. The mean age of presentation in 30 years.
Prevalence
According to a few studies prevalence of Short QT interval is 0.02 to 0.1% in adults and it is believed to be 0.05% in the pediatric population[1].
- Inability to identify the risks associated with short QT interval has led to failure in knowing precise prevalence in adults but it appears to be less than 2 percent if a cut off of 360 milliseconds is used. Studies with large cohorts have made attempts to identify how frequently could SQTS occur in the general population.
- In a Japanese cohort of 114,334 patients with ECGs stored in an electronic database, 0.37 percent were found to have short QTc intervals (≤357 milliseconds in males, ≤364 milliseconds in females) [2].
- In an American cohort of 46,129 healthy volunteers (53 percent female), nearly 2 percent had a QTc interval ≤360 milliseconds [3].
- In a Swiss cohort of 41,767 army conscripts (99.6 percent male, mean age 19 years), 1 percent had a short QTc interval (<347 milliseconds), and 0.02 percent had a very short QTc interval (<320 milliseconds) [4].
- In a Finnish cohort of 10,822 middle-aged (mean 44 years) patients, 0.4 percent had a short QTc interval (<340 milliseconds), and 0.1 percent had a very short QTc interval (<320 milliseconds) [5]
Age
The median age of presentation is 30 years but ranges from just weeks to the sixth decade of life.
Gender
SQTS is believed to affect both males and females equally due to the autosomal dominant nature of the disease. Although, A few studies believe it affects males more than females with high penetrance in males[6].
References
- ↑ Guerrier K, Kwiatkowski D, Czosek RJ, Spar DS, Anderson JB, Knilans TK (2015). "Short QT Interval Prevalence and Clinical Outcomes in a Pediatric Population". Circ Arrhythm Electrophysiol. 8 (6): 1460–4. doi:10.1161/CIRCEP.115.003256. PMID 26386018.
- ↑ Miyamoto A, Hayashi H, Yoshino T, Kawaguchi T, Taniguchi A, Itoh H; et al. (2012). "Clinical and electrocardiographic characteristics of patients with short QT interval in a large hospital-based population". Heart Rhythm. 9 (1): 66–74. doi:10.1016/j.hrthm.2011.08.016. PMID 21855519.
- ↑ Mason JW, Ramseth DJ, Chanter DO, Moon TE, Goodman DB, Mendzelevski B (2007). "Electrocardiographic reference ranges derived from 79,743 ambulatory subjects". J Electrocardiol. 40 (3): 228–34. doi:10.1016/j.jelectrocard.2006.09.003. PMID 17276451.
- ↑ Kobza R, Roos M, Niggli B, Abächerli R, Lupi GA, Frey F; et al. (2009). "Prevalence of long and short QT in a young population of 41,767 predominantly male Swiss conscripts". Heart Rhythm. 6 (5): 652–7. doi:10.1016/j.hrthm.2009.01.009. PMID 19303371.
- ↑ Anttonen O, Junttila MJ, Rissanen H, Reunanen A, Viitasalo M, Huikuri HV (2007). "Prevalence and prognostic significance of short QT interval in a middle-aged Finnish population". Circulation. 116 (7): 714–20. doi:10.1161/CIRCULATIONAHA.106.676551. PMID 17679619.
- ↑ El-Battrawy I, Schlentrich K, Besler J, Liebe V, Schimpf R, Lang S; et al. (2019). "Sex-differences in short QT syndrome: A systematic literature review and pooled analysis". Eur J Prev Cardiol: 2047487319850953. doi:10.1177/2047487319850953. PMID 31122038.