title: Coronary artery anomalies in Turner Syndrome.
abstract: BACKGROUND
  Congenital heart disease, primarily involving the left-sided structures, is often seen in patients with Turner Syndrome. Moreover, a few case reports have indicated that coronary anomalies may be more prevalent in Turner Syndrome than in the normal population. We therefore set out to systematically investigate coronary arterial anatomy by computed tomographic coronary angiography (coronary CTA) in Turner Syndrome patients.
  
  
  METHODS
  Fifty consecutive women with Turner Syndrome (mean age 47 years [17-71]) underwent coronary CTA. Patients were compared with 25 gender-matched controls.
  
  
  RESULTS
  Coronary anomaly was more frequent in patients with Turner Syndrome than in healthy controls [20% vs. 4% (p = 0.043)]. Nine out of ten abnormal cases had an anomalous left coronary artery anatomy (absent left main trunk, n = 7; circumflex artery originating from the right aortic sinus, n = 2). One case had a tubular origin of the right coronary artery above the aortic sinus. There was no correlation between the presence of coronary arterial anomalies and karyotype, bicuspid aortic valve, or other congenital heart defects.
  
  
  CONCLUSIONS
  Coronary anomalies are highly prevalent in Turner Syndrome. The left coronary artery is predominantly affected, with an absent left main coronary artery being the most common anomaly. No hemodynamically relevant coronary anomalies were found.
authors: Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark.
authors: Mette H
authors: MH
authors: Viuff
authors: Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark.
authors: Christian
authors: C
authors: Trolle
authors: Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark.
authors: Jan
authors: J
authors: Wen
authors: Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark.
authors: Jesper M
authors: JM
authors: Jensen
authors: Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark.
authors: Bjarne L
authors: BL
authors: Nørgaard
authors: Department of Aerospace Engineering and Engineering Mechanics, CEAS, University of Cincinnati, Cincinnati, OH, USA; UC Department of Otolaryngology - Head and Neck Surgery, Cincinnati, OH, USA.
authors: Ephraim J
authors: EJ
authors: Gutmark
authors: UC Department of Pediatrics, Cincinnati, OH, USA; Division of Endocrinology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors: Iris
authors: I
authors: Gutmark-Little
authors: Cardiovascular Imaging Department, Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, United Kingdom.
authors: Kristian H
authors: KH
authors: Mortensen
authors: Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark; Department of Molecular Medicine, Aarhus University Hospital, Aarhus, Denmark.
authors: Claus Højbjerg
authors: CH
authors: Gravholt
authors: Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark. Electronic address: holmark@ki.au.dk.
authors: Niels H
authors: NH
authors: Andersen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D000072226:Computed Tomography Angiography; D017023:Coronary Angiography / Q000379:methods*; D003330:Coronary Vessel Anomalies / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000503:physiopathology; D003331:Coronary Vessels / Q000000981:diagnostic imaging* / Q000503:physiopathology; D003718:Denmark / Q000453:epidemiology; D005260:Female; D006439:Hemodynamics; D006801:Humans; D007621:Karyotyping; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D014424:Turner Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics; D055815:Young Adult
keywords: Abnormal karyotype; Anatomy; Coronary artery disease; Embryology; Pathology