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01-12-2006 | Original Paper

QTc interval prolongation in children with Ulrich-Turner syndrome

Authors: Robert Dalla Pozza, Susanne Bechtold, Stefan Kääb, Matthias Buckl, Simon Urschel, Heinrich Netz, Hans-Peter Schwarz

Published in: European Journal of Pediatrics | Issue 12/2006

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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Title: QTc interval prolongation in children with Ulrich-Turner syndrome
Authors: Robert Dalla Pozza
Susanne Bechtold
Stefan Kääb
Matthias Buckl
Simon Urschel
Heinrich Netz
Hans-Peter Schwarz
Publication date: 01-12-2006
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 12/2006
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-006-0194-0

Keynote webinar | Spotlight on medication adherence

Springer Medicine

QTc interval prolongation in children with Ulrich-Turner syndrome

Abstract

Introduction

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusions

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