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QTc interval prolongation in children with Ulrich-Turner syndrome

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Abstract

Introduction

The Ullrich-Turner-Syndrome (UTS) is the most commonly occurring sex chromosome abnormality in females. Cardiac malformations are well known, but no data exist on specific electrocardiogram (ECG) patterns in children and adolescents. A prolongation of the QT interval on the ECG has been correlated to an increased risk for sudden cardiac death, and medical treatment is warranted in patients with long QT syndrome (LQTS). Moreover, several drugs of common use are contraindicated in LQTS because of their effects on myocardial repolarization.

Methods

The ECG tracings of 86 UTS patients [mean age (±SD): 11.0±3.6 years; mean height-SD: −2.6±1.0] were analyzed and compared to age-matched control groups of 75 boys (mean age: 11.3±3.5 years; mean height-SD: −1.9±1.0) and 62 girls (mean age: 10.1±3.2 years; mean height-SD: −2.5±0.9) with short stature.

Results

Eighteen UTS patients (20.9%) had an abnormally prolonged QTc >0.45 s in contrast to only one control subject (0.9%). The QTc interval of UTS patients was significantly prolonged compared to that of the controls (p<0.01) and normal values.

Conclusions

There is an increased prevalence of a long QT interval among UTS patients. This should be taken into account for the cardiovascular screening of such patients. Patients with UTS have an intrinsically prolonged QT interval and as such may be at higher risk for arrhythmias in the context of a QT-prolonging medication.

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Acknowledgement

This publication is dedicated to Prof. C. Döhlemann for his contribution and intellectual support.

Conflict of interest statement

All authors state that there is no conflict of interests.

Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, Ludwig-Maximilians-University, Marchioninistr. 15, 81377, Munich, Germany

    Robert Dalla Pozza, Simon Urschel & Heinrich Netz

  2. Pediatric Endocrinology, Ludwig-Maximilians-University, Lindwurmstr. 4, 80337, Munich, Germany

    Susanne Bechtold, Matthias Buckl & Hans-Peter Schwarz

  3. Department of Cardiology, Ludwig-Maximilians-University, Marchioninistr. 15, 81377, Munich, Germany

    Stefan Kääb

  4. Department of Pediatric Cardiology, University Children’s Hospital, Lindwurmstr. 4, 80336, Munich, Germany

    Robert Dalla Pozza

Authors
  1. Robert Dalla Pozza
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  2. Susanne Bechtold
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  3. Stefan Kääb
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  4. Matthias Buckl
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  5. Simon Urschel
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  6. Heinrich Netz
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  7. Hans-Peter Schwarz
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Corresponding author

Correspondence to Robert Dalla Pozza.

Additional information

R. Dalla Pozza and S. Bechtold contributed equally to this publication.

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Dalla Pozza, R., Bechtold, S., Kääb, S. et al. QTc interval prolongation in children with Ulrich-Turner syndrome. Eur J Pediatr 165, 831–837 (2006). https://doi.org/10.1007/s00431-006-0194-0

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  • DOI: https://doi.org/10.1007/s00431-006-0194-0

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