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Aortic dilatation in patients with Turner’s syndrome without structural cardiac anomaly

Published online by Cambridge University Press:  29 May 2015

Nassiba Alami Laroussi ,
Nagib Dahdah ,
Frédéric Dallaire ,
Johanne Thérien  and
Anne Fournier
Nassiba Alami Laroussi*
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
Nagib Dahdah
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
Frédéric Dallaire
Affiliation:
Department of Pediatrics, CHU Sherbrooke, Sherbrooke, Quebec, Canada
Johanne Thérien
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
Anne Fournier
Affiliation:
Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, Quebec, Canada
*
Correspondence to: N. Alami Laroussi, Pediatric cardiology, CHU Sainte Justine, 3175 chemin de la Côte Sainte Catherine, Montreal, Quebec H3T 1C5, Canada. Tel: 514-345-4931; Fax: 514-345-4896; E-mail: [email protected]
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Abstract

Introduction

Dilatation of the ascending aorta is described in Turner’s syndrome with variable prevalence (6.8–32%). Reported series typically include patients with associated cardiac anomalies.

Objective

To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner’s syndrome patients free of structural cardiac anomalies. Potential risk factors such as karyotype and growth hormone therapy were analysed for correlation with aortic dilatation.

Methods

We carried out a retrospective study with data collected from medical records and echocardiography studies. Patients with Tuner’s syndrome followed-up between 1992 and 2010 with at least two echocardiography studies were eligible. Patients with previous cardiac surgery or under anti-hypertensive medication were excluded. Ascending aorta diameter measurements were adjusted for body surface area, and dilatation was defined as Z-score>2.

Results

The study population consisted of 44 patients, aged 11.9±7.4 years at the first echocardiogram and 17.9±7.3 years at the last follow-up, with a follow-up duration of 6.0±3.7 years. A total of 13 (29.5%) patients exhibited aortic dilatation during follow-up, suggesting an actuarial estimate of the freedom from aortic dilatation dropping from 86 to 70% and then to 37% at 10, 20, and 30 years of age, respectively. There was no statistically significant impact of karyotype or growth hormone therapy on aortic Z-score progression.

Conclusion

The prevalence of dilatation of the ascending aorta in Turner’s syndrome patients free of structural aortic anomalies is comparable with published data with associated lesions. Growth hormone therapy and karyotype had no significant impact; however, longitudinal follow-up is warranted.

Keywords

Turner’s syndromeaortic dilatationpaediatric
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 3 , March 2016 , pp. 539 - 546
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Copyright
© Cambridge University Press 2015 

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