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Original research article
Coronary anatomy in Turner syndrome versus patients with isolated bicuspid aortic valves
  1. Wilke M C Koenraadt1,
  2. Hans-Marc J Siebelink1,
  3. Margot M Bartelings2,
  4. Martin J Schalij1,
  5. Maureen J van der Vlugt3,
  6. Annemien E van den Bosch4,
  7. Ricardo P J Budde4,5,
  8. Jolien W Roos-Hesselink4,
  9. Anthonie L Duijnhouwer3,
  10. Allard T van den Hoven4,
  11. Marco C DeRuiter2,
  12. Monique R M Jongbloed1,2
  1. 1Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands
  2. 2Department of Anatomy and Embryology, Leiden University Medical Center, Leiden, The Netherlands
  3. 3Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands
  4. 4Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands
  5. 5Department of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
  1. Correspondence to Dr Monique R M Jongbloed, Department of Anatomy and Embryology and Cardiology, Leiden University Medical Center, Leiden RC 2300, The Netherlands; M.R.M.Jongbloed{at}lumc.nl

Abstract

Objective Variations in coronary anatomy, like absent left main stem and left dominant coronary system, have been described in patients with Turner syndrome (TS) and in patients with bicuspid aortic valves (BAV). It is unknown whether coronary variations in TS are related to BAV and to specific BAV subtypes.

Aim To compare coronary anatomy in patients with TS with/without BAV versus isolated BAV and to study BAV morphology subtypes in these groups.

Methods Coronary anatomy and BAV morphology were studied in 86 patients with TS (20 TS-BAV, 66 TS-tricuspid aortic valve) and 86 patients with isolated BAV (37±13 years vs 42±15 years, respectively) by CT.

Results There was no significant difference in coronary dominance between patients with TS with and without BAV (25% vs 21%, p=0.933). BAVs with fusion of right and left coronary leaflets (RL BAV) without raphe showed a high prevalence of left coronary dominance in both TS-BAV and isolated BAV (both 38%). Absent left main stem was more often seen in TS-BAV as compared with isolated BAV (10% vs 0%). All patients with TS-BAV with absent left main stem had RL BAV without raphe.

Conclusion The equal distribution of left dominance in RL BAV without raphe in TS-BAV and isolated BAV suggests that presence of left dominance is a feature of BAVs without raphe, independent of TS. Both TS and RL BAV without raphe seem independently associated with absent left main stems. Awareness of the higher incidence of particularly absent left main stems is important to avoid complications during hypothermic perfusion.

  • bicuspid aortic valve
  • chronic coronary disease
  • genetics
  • congenital heart disease

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Footnotes

  • Contributors All authors contributed to refinement of the study protocol and approved the final manuscript.

  • Funding This work was supported by the Netherlands Heart Foundation (grant number NHS2013T093) to the research consortium ‘Unravelling etiology and risk factors in patients with Bicuspid Aortic valves’.

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval The medical ethical committee of LUMC, Erasmus MC and Radboud MC.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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