The DOI matches its Crossref record; 40 of the 40 references it cites were found in a registry or an index.
Major Vascular Anomalies in Turner Syndrome: Prevalence and Magnetic Resonance Angiographic Features
Abstract
BACKGROUND: Turner syndrome (TS) is associated with aortic coarctation and dissection; hence, echocardiographic evaluation of all patients is currently recommended. X-ray angiography in clinically symptomatic patients has suggested a range of other vascular anomalies, but the true prevalence of such lesions in TS is unknown. To better understand the prevalence and pathogenesis of cardiovascular defects in TS, we prospectively evaluated a group of asymptomatic adult volunteers with TS using magnetic resonance (MR) angiography. METHODS AND RESULTS: A total of 85 adults with TS and 27 normal female adult volunteers underwent gadolinium-enhanced 3D MR angiography. A high prevalence of aortic anomalies was seen in women with TS, including elongation of the transverse arch (49%), aortic coarctation (12%), and aberrant right subclavian artery (8%). Venous anomalies were also prominent, including persistent left superior vena cava (13%) and partial anomalous pulmonary venous return (13%). None of these anomalies were found in healthy female controls. The constellation of elongation of the transverse arch, aortic coarctation, and persistent left superior vena cava was significantly associated with women with TS. Neck webbing and increased thoracic anterior-to-posterior dimension diameters were strong predictors for arterial and venous anomalies. CONCLUSIONS: Thoracic vascular anomalies are common in TS, occurring in approximately 50% of a group not preselected for cardiovascular disease. The highly significant association between neck webbing, increased chest diameter, and these vascular anomalies suggests that in utero, centrally localized lymphatic obstruction may contribute to these cardiovascular deformities in TS. Improved recognition of these often-undetected vascular lesions may be important for identification of patients in need of closer cardiovascular monitoring.
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“Vascular anomalies, including partial anomalous pulmonary venous return, left superior vena cava, elongated transverse arch, and dilation of the brachiocephalic arteries, often remain undetected unless advanced imaging modalities are used.388,395”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [395]
In the citing paper
“CMR is not constrained by anatomic factors and is thus more sensitive for extra-cardiac lesions such as partial anomalous pulmonary venous return.388,395,420”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [395]
In the citing paper
“CMR is also superior to TTE for the diagnosis of BAV, aortic dilation and aortic arch anomalies395,429-433 while also providing important data on shunt fractions and ventricular volumes that may inform clinical management.388”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [395]
In the citing paper
“While venous anomalies (anomalous pulmonary veins, left superior vena cava) are more common in TS, the question remains if malformations of the inferior vena cava or pelvic veins increase the risk for deep venous thrombosis of the lower extremities.395,475”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [395]
Citations
Cites
A SYNDROME OF INFANTILISM, CONGENITAL WEBBED NECK, AND CUBITUS VALGUS1
TURNER, H. H. (1938) · Endocrinology · doi:10.1210/endo-23-5-566 · Cited by 1,124
Recommendations for the Diagnosis and Management of Turner Syndrome
Saenger, P. H., Wikland, K. A., Conway, G. S., Davenport, M., Gravholt, C. H., Hintz, R. L., Hovatta, O., Hultcrantz, M., Landin‐Wilhelmsen, K., Lin, A. E., et al. (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.7.7683 · Cited by 419
Cardiovascular Malformations and Complications in Turner Syndrome
Sybert, V. P. (1998) · PEDIATRICS · doi:10.1542/peds.101.1.e11 · Cited by 326
Three-dimensional gadolinium-enhanced MR angiography of the thoracic aorta.
Prince, M. R., Narasimham, D. L., Jacoby, W. T., Williams, D. M., Cho, K. J., Marx, M. V., & Deeb, G. M. (1996) · American Journal of Roentgenology · doi:10.2214/ajr.166.6.8633452 · Cited by 292
Congenital heart disease in patients with Turner’s syndrome
Mazzanti, L., & CACCIARI, E. (1998) · The Journal of Pediatrics · doi:10.1016/s0022-3476(98)70119-2 · Cited by 261
CONGENITAL ANOMALIES ASSOCIATED WITH GONADAL APLASIA
Haddad, H. M., & Wilkins, L. (1959) · PEDIATRICS · doi:10.1542/peds.23.5.885 · Cited by 235
Further Delineation of Aortic Dilation, Dissection, and Rupture in Patients With Turner Syndrome
Lin, A. E., Lippe, B. M., & Rosenfeld, R. G. (1998) · PEDIATRICS · doi:10.1542/peds.102.1.e12 · Cited by 231
Congenital anomalies associated with gonadal aplasia; review of 55 cases.
Haddad, M., & Wilkins, L. (1959) · PubMed · Cited by 204
The XO syndrome: A study of the differentiated phenotype in 25 patients
Lemli, L., & Smith, D. W. (1963) · The Journal of Pediatrics · doi:10.1016/s0022-3476(63)80368-6 · Cited by 184
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Research works printed but not linked: 10
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Items 301–315 of 315
Chen, M. Y., & Arai, A. E. (2010) · Elsevier eBooks · doi:10.1016/b978-1-4160-6135-9.50011-5
Katsuragi, S., & Niwa, K. (2018) · Springer eBooks · doi:10.1007/978-981-10-1993-7_13
Prieto, L. (2009) · Springer eBooks · doi:10.1007/978-3-540-29676-8_1503
Lin, A. E., & Crenshaw, M. (2020) · Wiley eBooks · doi:10.1002/9781119432692.ch60
Analysis of a streamlined pathway for aortic surveillance for Turner syndrome in a single centre
Glatzel, H., Njue, F., Turner, H. E., & Orchard, E. (2023) · Clinical Endocrinology · doi:10.1111/cen.14968
Ansah, D., Mejia, A. A. S., & Fatusin, O. O. (2023) · Springer eBooks · doi:10.1007/978-3-030-42937-9_25-1
Ansah, D., Mejia, A. A. S., & Fatusin, O. O. (2024) · Springer eBooks · doi:10.1007/978-3-031-07563-6_25
Drevet, G., Soldea, V., & Tronc, F. (2024) · Thoracic Cancer · doi:10.1111/1759-7714.15422
Aortic Coarctation in the Adult
Fisher, R., Love, B. A., Fischer, G. W., Stelzer, P. E., & Fuster, V. (2024) · Springer eBooks · doi:10.1007/978-1-4471-4999-6_184-2
Adult care of Turner syndrome women
Gravholt, C. H. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-13825-6.00164-3
Diagnosis of Turner syndrome after presenting with ischemic cardiomyopathy: A case report
Zheng, H., Guo, L., Wei, Y., Zhang, Y., & Guan, Y. (2025) · SAGE Open Medical Case Reports · doi:10.1177/2050313x251318519
Lifelong aortic risk in Turner syndrome: Unexpected aortic events in asymptomatic patients
Jaiswal, M., Mansoorshahi, S., Zachariah, S., Macedo, T. A., Cecchi, A. C., Tanaka, A., Estrera, A. L., Afifi, R. O., Oderich, G. S., Milewicz, D. M., et al. (2025) · Journal of Vascular Surgery Cases and Innovative Techniques · doi:10.1016/j.jvscit.2025.101919
Partial Anomalous Pulmonary Venous Connections and the Scimitar Syndrome
Ellis, A. R. (2025) · Elsevier eBooks · doi:10.1016/b978-0-702-08231-3.00033-8
Elsaim, S., Vernier, B., Truong, V. T. T., Patel, R. T., Brown, M., Chacón-Portillo, M. A., Rogge, M. N., Rodriguez‐Buritica, D., & Prakash, S. K. (2026) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.70200
Coarctation and Interrupted Aortic Arch
Cheung, Y., Spicer, D. E., Anderson, R. H., & Crucean, A. C. (2026) · Elsevier eBooks · doi:10.1016/b978-0-443-11851-7.00050-1
Metadata
- Authors
- Ho, V. B., Bakalov, V. K., Cooley, M., Van, P. L., Hood, M. N., Burklow, T. R., & Bondy, C. A.
- Affiliations
- National Institutes of Health (US)
- Venue
- Circulation
- Published
- 2004-09-21
- Volume
- 110
- Issue
- 12
- Pages
- 1694-1700
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2105153540
- Cited by
- 316
- References
- 40
- Citations by year
- 2026: 2 · 2025: 7 · 2024: 13 · 2023: 11 · 2022: 15 · 2021: 14 · 2020: 12 · 2019: 15 · 2018: 26 · 2017: 14 · 2016: 14 · 2015: 22 · 2014: 16 · 2013: 21 · 2012: 16
- FWCI
- 13.27
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Vascular anomalies and interventions
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Congenital Heart Disease Studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Keywords
- Turner syndrome, vascular anomalies, coarctation of the aorta, aberrant right subclavian artery, persistent left superior vena cava, partial anomalous pulmonary venous return, lymphatic obstruction, cardiovascular defects, prevalence, magnetic resonance angiography
- MeSH terms
- Adult, Anthropometry, Aortic Arch Syndromes, Aortic Coarctation, Blood Pressure, Female, Humans, Magnetic Resonance Imaging, Middle Aged, Neck, Pulmonary Veins, Subclavian Artery, Thorax, Turner Syndrome, Vena Cava, Superior, Prevalence, Single-Blind Method, Cardiovascular Abnormalities, Imaging, Three-Dimensional
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