Cor Vasa 2023, 65(4):582-588 | DOI: 10.33678/cor.2023.036

(Arrhythmogenic potential in Turner syndrome patients)

Sabina Kaprálováa, Jan Pavlíčekb, c, Alžběta Palátováa, Radek Vrtěld, Hana Tomáškováe, Jiřina Zapletalováa, Eva Kláskováa
a Dětská klinika, Lékařská fakulta Univerzity Palackého v Olomouci a Fakultní nemocnice Olomouc, Olomouc
b Klinika dětského lékařství, Lékařská fakulta Ostravské univerzity a Fakultní nemocnice Ostrava, Ostrava
c Centrum biomedicínského výzkumu, Fakultní nemocnice Hradec Králové, Hradec Králové
d Ústav lékařské genetiky, Lékařská fakulta Univerzity Palackého v Olomouci a Fakultní nemocnice Olomouc, Olomouc
e Ústav epidemiologie a ochrany veřejného zdraví, Lékařská fakulta Ostravské univerzity, Ostrava

Introduction: Turner syndrome (TS) is associated with a range of distinct cardiovascular risks such as congenital heart disease and acquired cardiovascular disease. Data on arrhythmogenic potential in TS carriers are inconsistent, especially regarding the occurrence of malignant ventricular arrhythmias due to long QT interval. The aim of this study was to evaluate the prevalence of ECG abnormalities in patients with TS.

Methods: 61 girls and women with TS syndrome were included in the study and underwent a cardiac examination including ECG and 24-hour ECG Holter monitoring. The 12-lead ECG was used to assess the length of PR and QT interval, the mean heart rate (HR) including z-score, presence of atrial/ventricular premature beats and other arrhythmias. QT interval was corrected according to Bazett and Hodges formulas, and both values were statistically compared. The relationship of parameters to individual karyotypes was analyzed.

Results: Median PR interval was 120 ms (mean 118.4 ms), short PR interval was identified in 13% (8/61), none of the patients had delta wave as a manifestation of ventricular pre-excitation. Median of absolute QT values was 340 ms (mean 336 ms). QTc interval prolonged above 440 ms was observed in 5% (3/61) of

Keywords: ECG abnormalities, Long QT interval, Malignant arrhythmias, Turner syndrome

Received: April 15, 2023; Revised: April 15, 2023; Accepted: April 16, 2023; Published: September 1, 2023  Show citation

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Kaprálová S, Pavlíček J, Palátová A, Vrtěl R, Tomášková H, Zapletalová J, Klásková E. (Arrhythmogenic potential in Turner syndrome patients). Cor Vasa. 2023;65(4):582-588. doi: 10.33678/cor.2023.036.
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    References

    1. Elsheikh M, Dunger DB, Conway GS, Wass JA. Turner's syndrome in adults. Endocr Rev 2002;23:120-140. Go to original source...
    2. Stochholm K, Juul S, Juel K, et al. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 2006;91:3897-3902. Go to original source... Go to PubMed...
    3. Wolff DJ, Van Dyke DL, Powell CM; Working Group of the ACMG Laboratory Assurance Committee. Laboratory guideline for Turner syndrome. Genet Med 2010;12:52-55. Go to original source... Go to PubMed...
    4. Saenger P, Wikland KA, Conway GS, et al. Recommendations for the diagnosis and management of Turner syndrome. J Clin Endocrinol Metab 2001;86:3061-3069. Go to original source... Go to PubMed...
    5. Bondy CA. Turner Syndrome Consensus Study Group. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab 2007;92:10-25. Go to original source... Go to PubMed...
    6. Mortensen KH, Andersen NH, Gravholt CH. Cardiovascular phenotype in Turner syndrome - integrating cardiology, genetics, and endocrinology. Endocr Rev 2012;33:677-714. Go to original source... Go to PubMed...
    7. Schoemaker MJ, Swerdlow AJ, Higgins CD, et al. United Kingdom Clinical Cytogenetics Group. Mortality in women with Turner syndrome in Great Britain: a national cohort study. J Clin Endocrinol Metab 2008;93:4735-4742. Go to original source... Go to PubMed...
    8. Bondy CA, Ceniceros I, Phillip LV, et al. Prolonged rate-corrected QT interval and other electrocardiogram abnormalities in girls with Turner syndrome. Pediatrics 2006;118:e1220-e1225. Go to original source... Go to PubMed...
    9. Noordman ID, Fejzic Z, Bos M, et al. Cardiac abnormalities in girls with Turner syndrome: ECG abnormalities, myocardial strain imaging, and karyotype-phenotype associations. Am J Genet 2021;185A:2399-2408. Go to original source... Go to PubMed...
    10. Salameh A, Gebauer RA, Grollmuss O, et al. Normal limits for heart rate as established using 24-hour ambulatory electrocardiography in children and adolescents. Cardiol Young 2008;18:467-472. Go to original source... Go to PubMed...
    11. Mortensen KH, Andersen NH, Gravholt CH. Cardiovascular phenotype in Turner syndrome - integrating cardiology, genetics, and endocrinology. Endocr Rev 2012;33:677-714. Go to original source... Go to PubMed...
    12. Choi Y, Hoffman J, Alarcon L, et al. Neonatal arrhytmias in Turner syndrome: a case report and review of the literature. Eur Heart J Case Rep 2021;5:160. Go to original source... Go to PubMed...
    13. Harahill NJ, Yetman AT, Dandorf DA, et al. The QT interval in patients with the Turner syndrome. Am J Cardiol 2021;140:118-121. Go to original source... Go to PubMed...
    14. Conen D, Adam M, Roche F, et al. Premature atrial contractions in the general population: frequency and risk factors. Circulation 2012;126:2302-2308. Go to original source... Go to PubMed...
    15. Panizo JG, Barra S, Mellor G, et al. Premature ventricular complex-induced cardiomyopathy. Arrhythm Electrophysiol Rev 2018;7:128-134. Go to original source... Go to PubMed...
    16. Noordman ID, Duijnhouwer AL, Coert M, et al. No QTc prolongation in girls and women with Turner syndrome. J Clin Endocrinol Metab 2020;105:148-156. Go to original source... Go to PubMed...
    17. CH, Andersen NH, Conway GS, et al. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol 2017;177:G1-G70. Go to original source... Go to PubMed...
    18. Dalla Pozza R, Bechtold S, Urschel S, et al. QTc interval prolongation in children with Turner syndrome: the results od exercise testing adn 24-h ECG. Eur J Pediatr 2009;168:59-64. Go to original source... Go to PubMed...
    19. Trolle C, Mortensen KH, Pedersen LN, et al. Long QT interval in Turner syndrome - a high prevalence of LQTS gene mutations. PLoS One 2013;8:e69614. Go to original source... Go to PubMed...
    20. Grouthier V, Moey MY, Gandjbakhch E, et al. Sexual dimorphisms, anti-hormonal therapy and cardiac arrhytmias. Int J Mol Sci 2021;2:1464. Go to original source... Go to PubMed...
    21. Atici A, Panc C, Karaayvaz EB, et al. Evaluation of the Tp-Te interval, Tp-Te/QTc ratio, and QT dispersion in patients with Turner sydnrome. Anatol J Cardiol 2018;20:93-99. Go to original source... Go to PubMed...

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