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Original research article
Coronary anatomy in children with bicuspid aortic valves and associated congenital heart disease
  1. Wilke M C Koenraadt1,
  2. Margot M Bartelings2,
  3. Regina Bökenkamp3,
  4. Adriana C Gittenberger-de Groot1,2,
  5. Marco C DeRuiter2,
  6. Martin J Schalij1,
  7. Monique RM Jongbloed1,2
  1. 1 Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands
  2. 2 Department of Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands
  3. 3 Department of Paediatric Cardiology, Leiden University Medical Center, Leiden, The Netherlands
  1. Correspondence to Dr Monique RM Jongbloed, Department of Anatomy and Embryology and Cardiology, Leiden University Medical Center, Postal zone: S-1-P, P.O. Box 9600, 2300 RC Leiden, The Netherlands; M.R.M.Jongbloed{at}


Objective In patients with bicuspid aortic valve (BAV), coronary anatomy is variable. High take-off coronary arteries have been described, but data are scarce, especially when associated with complex congenital heart disease (CHD). The purpose of this study was to describe coronary patterns in these patients.

Methods In 84 postmortem heart specimens with BAV and associated CHD, position and height of the coronary ostia were studied and related to BAV morphology.

Results High take-off right (RCA) and left coronary arteries (LCA) were observed in 23% and 37% of hearts, respectively, most frequently in hearts with hypoplastic left ventricle (HLV) and outflow tract anomalies. In HLV, high take-off was observed in 18/40 (45%) more frequently of LCA (n=14) than RCA (n=6). In hearts with aortic hypoplasia, 8/13 (62%) had high take-off LCA and 6/13 (46%) high take-off RCA. High take-off was seen 19 times in 22 specimens with perimembranous ventricular septal defect (RCA 8, LCA 11). High take-off was associated with type 1A BAV (raphe between right and left coronary leaflets), more outspoken for the RCA. Separate ostia of left anterior descending coronary artery and left circumflex coronary artery were seen in four hearts (5%), not related to specific BAV morphology.

Conclusion High take-off coronary arteries, especially the LCA, occur more frequently in BAV with associated CHD than reported in normal hearts and isolated BAV. Outflow tract defects and HLV are associated with type 1A BAV and high take-off coronary arteries. Although it is unclear whether these findings in infants with detrimental outcome can be related to surviving adults, clinical awareness of variations in coronary anatomy is warranted.

  • Word count: 3239

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  • Contributors WMCK is the main author of this article and planned and executed most of the research, analysis and reporting. MMB has contributed to the study conception, data collection and interpretation and has critically reviewed the article with valuable suggestions. RB has contributed to the study conception and data collection and has thoroughly reviewed the article and made some helpful suggestions. ACGdG has contributed to the study conception and reviewed the article thoroughly with valuable suggestions. MCD has thoroughly reviewed the article and has made some valuable remarks. MJS has contributed to the study conception and reviewing it thoroughly with valuable remarks. MRMJ has contributed to conception of the article, data analysis and interpretation, drafting the article and critically reviewing it several times. Authors responsible for overall content: WMCK, MRMJ. All authors approved of the final version of the article to be published.

  • Funding The research of MCDR on bicuspid aortic valve disease is funded by a grant of the Dutch Heart Foundation (grant NHS2013T093).

  • Competing interests None declared.

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

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