No QTc Prolongation in Girls and Women with Turner Syndrome

Abstract

Context Turner syndrome (TS) is a genetic condition and reported to be associated with a prolonged rate-corrected QT interval (QTc). Objectives To evaluate the prevalence of QTc prolongation in patients with TS, to compare their QTc intervals with healthy controls, and to investigate whether QTc prolongation is associated with a monosomy 45,X karyotype. Methods Girls (n=101) and women (n=251) with TS visiting our expertise center from 2004-2018 were included in this cross-sectional study. QT intervals of 12-leaded electrocardiograms were measured manually, using Bazett’s and Hodges’ formulas to correct for heart rate. A QTc interval of >450 ms for girls and >460 ms for women was considered prolonged. QTc intervals of patients with TS were compared to the QTc intervals of healthy girls and women from the same age-groups derived from the literature. Results In total, 5% of the population with TS had a prolonged QTc interval using Bazett’s formula and 0% using Hodges’ formula. Mean QTc intervals of these patients were not prolonged compared to the QTc interval of healthy individuals from the literature. Girls showed shorter mean QTc intervals compared to women. We found no association between monosomy 45,X and prolongation of the QTc interval. Conclusions This study shows that the QTc interval in girls and women with TS is not prolonged compared to the general population derived from the literature, using both Bazett’s and Hodges’ formulas. Furthermore, girls show shorter QTc intervals compared to women, and a monosomy 45,X karyotype is not associated with QTc prolongation.

Heart and Turner syndrome

“…However, a recent study reports that the QTc interval in girls and women with TS is not prolonged compared to the general population. In this study, a 45,X monosomy is not associated with QTc prolongation [16].…”

Cardiac abnormalities in girls with Turner syndrome: ECG abnormalities, myocardial strain imaging, and karyotype–phenotype associations

“…Besides cardiovascular malformations, electrocardiogram (ECG) abnormalities, mainly QTc prolongation, have been reported (Atici et al, 2018;Bondy, Ceniceros, et al, 2006;Dalla Pozza et al, 2006;Dalla Pozza et al, 2009;Sozen et al, 2008;Trolle et al, 2013). We recently found no increased prevalence of QTc prolongation using Hodges formula in a large cohort of girls (n = 101) and women (n = 251) with TS, as was confirmed by Harrahill et al in 112 women with TS (Harrahill et al, 2020;Noordman et al, 2020).…”

“…This study was approved by the local ethics committee (CMO Arnhem-Nijmegen). The current study population is partially overlapping with the study population of two previous publications (Noordman et al, 2018;Noordman et al, 2020).…”

Sexual Dimorphisms, Anti-Hormonal Therapy and Cardiac Arrhythmias

“…Recently this year, a larger study of 359 women found no difference in QTc duration compared to the general population. These authors described a lower prevalence of QTc prolongation in patients with TS compared to previous studies, which may be a result of a different cutoff value used for the definition of QTc prolongation (450 for girls and 460 ms for women, in comparison to a cutoff of 440 ms in other studies) [ 62 ]. Another possible explanation could be linked to the karyotypic formula of the patients (monosomia or mosaic).…”

“…Another possible explanation could be linked to the karyotypic formula of the patients (monosomia or mosaic). Although the association of karyotype with Y chromosomal material and shortening of QTc was recently demonstrated by Harrahill et al [ 58 ], this association has been rarely studied and the outcomes remain contradicting [ 61 , 62 ]. Finally, a high prevalence of mutations in the major LQTS genes in women with TS and prolonged QTc have already been discussed in 2013 [ 63 ].…”