title: Aortic dilatation in patients with Turner's syndrome without structural cardiac anomaly.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 1Division of Pediatric Cardiology,CHU Ste-Justine,University of Montreal,Montreal,Quebec,Canada.
authors.forename: Nassiba
authors.initials: N
authors.lastname: Alami Laroussi
authors.affiliation: 1Division of Pediatric Cardiology,CHU Ste-Justine,University of Montreal,Montreal,Quebec,Canada.
authors.forename: Nagib
authors.initials: N
authors.lastname: Dahdah
authors.affiliation: 2Department of Pediatrics,CHU Sherbrooke,Sherbrooke,Quebec,Canada.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Dallaire
authors.affiliation: 1Division of Pediatric Cardiology,CHU Ste-Justine,University of Montreal,Montreal,Quebec,Canada.
authors.forename: Johanne
authors.initials: J
authors.lastname: Thérien
authors.affiliation: 1Division of Pediatric Cardiology,CHU Ste-Justine,University of Montreal,Montreal,Quebec,Canada.
authors.forename: Anne
authors.initials: A
authors.lastname: Fournier
mesh_terms: D000293:Adolescent; D000328:Adult; D001011:Aorta / Q000000981:diagnostic imaging*; D001018:Aortic Diseases / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic / Q000000981:diagnostic imaging; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D007621:Karyotyping; D011792:Quebec; D012044:Regression Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D014424:Turner Syndrome / Q000150:complications*; D055815:Young Adult
keywords: Turner’s syndrome; aortic dilatation; paediatric