Coronary anatomy in Turner syndrome versus patients with isolated bicuspid aortic valves
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.
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The effect of congenital coronary artery anomalies on mortality risk is unknown.
“Congenital coronary artery anomalies appear relatively common in TS, but their effect on mortality risk is unknown.396”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [396]
Congenital coronary artery anomalies appear relatively common in TS.
“Congenital coronary artery anomalies appear relatively common in TS, but their effect on mortality risk is unknown.396”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [396]
Non-invasive imaging is an essential part of cardiovascular screening and surveillance across the life span of individuals with TS.
“Non-invasive imaging is an essential part of cardiovascular screening and surveillance across the life span of individuals with TS,233,388,396,418-423 but imaging remains underutilized and not systematically applied.420,424”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [396]
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- Authors
- Koenraadt, W. M. C., Siebelink, H.-M. J., Bartelings, M. M., Schalij, M. J., van der Vlugt, M. J., van den Bosch, A. E., Budde, R. P. J., Roos-Hesselink, J. W., Duijnhouwer, A. L., van den Hoven, A. T., DeRuiter, M. C., & Jongbloed, M. R. M.
- Affiliations
- Leiden University Medical Center (NL)
- Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands
- Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands
- Erasmus MC (NL)
- Radboud University Medical Center (NL)
- Corresponding author
- Jongbloed, Monique R M
- ORCID
- Venue
- Heart
- Publisher
- BMJ
- Published
- 2018-10-27
- Volume
- 105
- Issue
- 9
- Pages
- 701-707
- ISSN
- 1355-6037 (ISSN-L)
- 1468-201X
- OpenAlex
- W2898399244
- Cited by
- 10
- References
- 27
- Citations by year
- 2025: 1 · 2024: 2 · 2023: 1 · 2022: 3 · 2020: 2 · 2019: 1
- FWCI
- 0.57
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Heart (publishedVersion)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Leiden Repository (Leiden University) (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Funders
- Hartstichting
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Coronary Artery Anomalies
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Keywords
- Turner syndrome, bicuspid aortic valve, coronary anatomy, coronary artery dominance, raphe, congenital heart disease, hypothermic perfusion
- MeSH terms
- Computed Tomography Angiography, Bicuspid Aortic Valve Disease, Adult, Aortic Valve, Coronary Artery Disease, Coronary Vessels, Echocardiography, Female, Heart Valve Diseases, Humans, Male, Netherlands, Turner Syndrome, Incidence, Prevalence, Coronary Angiography
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