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Cited by 55Genetic and Clinical Aspects of Sex Determination and Chromosomal AbnormalitiesCardiac electrophysiology and arrhythmiasWilliams Syndrome Research

Prolongation of the cardiac QTc interval in Turner syndrome

Bondy, Carolyn A., Van, Phillip L., Bakalov, Vladimir K., Sachdev, Vandana, Malone, Carol A., Ho, Vincent B., Rosing, Douglas R.·2006·Medicine
DOI 10.1097/01.md.0000205629.16302.bc
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Abstract

Anatomic anomalies of the cardiovascular system occur in approximately 50% of individuals with Turner syndrome (TS), with the specific genetic cause(s) for the heart defects still unknown. Because congenital heart disease may be associated with conduction system abnormalities, we compared electrocardiograms (ECGs) in 100 women with TS and 100 age-matched female controls. Women with TS were significantly more likely to demonstrate left posterior fascicular block (p < 0.005), accelerated AV conduction (p < 0.006), and T wave abnormalities (p < 0.006). The PR interval was significantly shorter (137 +/- 17 vs. 158 +/- 18 ms, p < 0.0001) and the rate-corrected QT interval (QTc) significantly longer in women with TS than in controls (423 +/- 19 ms vs. 397 +/- 18 ms; p < 0.0001). Twenty-one women with TS but no controls had a QTc greater than 440 ms. We found no statistically significant relation between body habitus, cardiac dimensions, evidence of congenital heart disease, or metabolic parameters and the incidence of ECG abnormalities or QTc duration in TS. Cardiac conduction and repolarization abnormalities appear to be intrinsic features of TS, suggesting that deletion of the second sex chromosome has more profound effects on the cardiovascular system than previously recognized, and that ECG analysis should be included in evaluating and monitoring patients with Turner syndrome.

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Crossref · 10.1097/01.md.0000205629.16302.bc

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  • Other ECG abnormalities ("RSR" complexes, right axis deviation, right ventricular hypertrophy, accelerated atrioventricular conduction, and T-wave abnormalities) have also been reported at higher rates compared to the general population.

    “Other ECG abnormalities (“RSR” complexes, right axis deviation, right ventricular hypertrophy, accelerated atrioventricular conduction, and T-wave abnormalities) have also been reported at higher rates compared to the general population.”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [487]

  • These ECG findings are minor in many cases and the clinical implications are not clear.

    “However, these findings are minor in many cases and the clinical implications are not clear.487,490,491”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [487]

  • A higher resting heart rate has been well documented in TS cohorts and has been linked to increased resting sympathetic tone and other features of dysautonomia.

    “A higher resting heart rate has been well documented in TS cohorts and has been linked to increased resting sympathetic tone and other features of dysautonomia.487,488”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [487]

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Authors
Bondy, C. A., Van, P. L., Bakalov, V. K., Sachdev, V., Malone, C. A., Ho, V. B., & Rosing, D. R.
Affiliations
National Institutes of Health (US)
ORCID
Venue
Medicine
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2006-03
Volume
85
Issue
2
Pages
75-81
ISSN
0025-7974 (ISSN-L)
DOI
10.1097/01.md.0000205629.16302.bc
OpenAlex
W2028306497
Cited by
55
References
37
Citations by year
2026: 1 · 2025: 1 · 2024: 1 · 2023: 1 · 2022: 3 · 2021: 2 · 2020: 5 · 2019: 2 · 2018: 2 · 2017: 1 · 2015: 2 · 2014: 2 · 2013: 5 · 2012: 4
FWCI
3.03
Type
journal article
Language
en
Open access
closed
Locations
  • Medicine (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
  • Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
Keywords
Turner syndrome, QTc prolongation, electrocardiographic abnormalities, left posterior fascicular block, T wave changes, PR interval, cardiac conduction disorders, cardiac repolarization abnormalities, congenital heart disease, cardiovascular anomalies
MeSH terms
Adolescent, Adult, Aged, Blood Pressure, Body Height, Child, Electrocardiography, Female, Heart, Heart Conduction System, Heart Ventricles, Humans, Middle Aged, Turner Syndrome, Echocardiography, Doppler, Body Mass Index, Case-Control Studies, Cardiovascular Abnormalities

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