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.Conclusion 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.
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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 [422]
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Additional file 1 of X chromosome dosage and the genetic impact across human tissues
Viuff, M. H., Skakkebaek, A. H., Johannsen, E. B., Chang, S., Pedersen, S. B., Lauritsen, K. M., Pedersen, M. G. B., Trolle, C., Just, J., & Gravholt, C. H. (2023) · Figshare · doi:10.6084/m9.figshare.22619594
Additional file 2 of X chromosome dosage and the genetic impact across human tissues
Viuff, M. H., Skakkebaek, A. H., Johannsen, E. B., Chang, S., Pedersen, S. B., Lauritsen, K. M., Pedersen, M. G. B., Trolle, C., Just, J., & Gravholt, C. H. (2023) · Figshare · doi:10.6084/m9.figshare.22619597
- Authors
- Viuff, M. H., Trolle, C., Wen, J., Jensen, J. M., Nørgaard, B. L., Gutmark, E. J., Gutmark-Little, I., Mortensen, K. H., Gravholt, C. H., & Andersen, N. H.
- Affiliations
- Aarhus University Hospital (DK)
- University of Cincinnati (US)
- Great Ormond Street Hospital (GB)
- Corresponding author
- Andersen, Niels H.
- ORCID
- Venue
- Journal of cardiovascular computed tomography
- Publisher
- Elsevier BV
- Published
- 2016-11
- Volume
- 10
- Issue
- 6
- Pages
- 480-484
- ISSN
- 1876-861X (ISSN-L)
- 1934-5925
- OpenAlex
- W2506843884
- Cited by
- 42
- References
- 24
- Citations by year
- 2025: 4 · 2024: 3 · 2023: 6 · 2022: 7 · 2021: 3 · 2020: 5 · 2019: 4 · 2018: 4 · 2017: 5 · 2016: 1
- FWCI
- 4.18
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of cardiovascular computed tomography (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Coronary Artery Anomalies
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiac Imaging and Diagnostics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Keywords
- Turner syndrome, coronary artery anomalies, congenital heart disease, coronary artery origin, coronary CT angiography, karyotype, bicuspid aortic valve
- MeSH terms
- Computed Tomography Angiography, Adolescent, Adult, Aged, Coronary Vessel Anomalies, Coronary Vessels, Denmark, Female, Hemodynamics, Humans, Karyotyping, Middle Aged, Predictive Value of Tests, Turner Syndrome, Prevalence, Case-Control Studies, Coronary Angiography, Young Adult
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