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Hypertension in Turner syndrome: a review of proposed mechanisms, management and new directions
Abstract
Acquired cardiovascular diseases account for much of the increased risk of premature death in patients with Turner syndrome (TS). Hypertension is a major modifiable cardiovascular risk factor. It has a high prevalence in TS developing at an early age and thus leading to prolonged exposure to high blood pressure. The aetiology for hypertension in TS is largely unknown. It is likely multifactorial, and recent hypotheses include altered sympathetic tone, vasculopathy and endocrine factors. In this review article we aim to provide a comprehensive review of data on mechanisms of hypertension in TS and their implication for diagnostics and optimal choice of antihypertensive treatments. Ultimately this knowledge should help prevent hypertension-related complications, and improve quality of life and life expectancy for patients with TS.
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While hypertension is correlated with the presence of aortic dilation in TS, no studies have demonstrated that antihypertensive therapies slow or prevent aortic dilation.
“While hypertension is correlated with the presence of aortic dilation in TS, no studies have demonstrated that antihypertensive therapies slow or prevent aortic dilation.447,456,458”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [458]
Two systematic reviews of hypertension in pediatric and primarily adult TS case series were published in 2022.
“Two systematic reviews of hypertension in pediatric (127 full texts) and primarily adult (63 full texts) TS case series were published in 2022.453,458”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [458]
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Uçar, A., Öz, F., Baş, F., Oflaz, H., Nişli, K., Tuğrul, M., Yetim, A., Darendelıler, F., Saka, N., Poyrazoğlu, Ş., et al. (2014) · Clinical Endocrinology · doi:10.1111/cen.12626 · Cited by 18
Natural history of hypertension in Turner syndrome during a 12-year pragmatic interventional study
Sandahl, K., Wen, J., Erlandsen, M., Andersen, N. H., & Gravholt, C. H. (2020) · Hypertension · doi:10.1161/hypertensionaha.120.15292 · Cited by 17
Relationship between arterial stiffness and circadian pattern of blood pressure
Park, J., Shin, J., Park, J.-B., Choi, D., Youn, H., Park, C. G., Kwan, J., Ahn, Y., Kim, D.-W., Rim, S., et al. (2019) · Medicine · doi:10.1097/md.0000000000014953 · Cited by 16
Pees, C., Heno, J., Haeusler, G., Ertl, D., Gulesserian, T., & Michel‐Behnke, I. (2018) · Heart and Vessels · doi:10.1007/s00380-018-1187-4 · Cited by 13
Brun, S., Berglund, A., Mortensen, K. H., Hjerrild, B. E., Hansen, K. W., Andersen, N. H., & Gravholt, C. H. (2019) · Clinical Endocrinology · doi:10.1111/cen.13983 · Cited by 13
Tenekecioğlu, E., Özlük, Ö. A., Kuzeytemiz, M., Topal, D., Yılmaz, M., Karaagac, K., & Vatansever, F. (2014) · Eurasian Journal of Medicine · doi:10.5152/eajm.2014.27 · Cited by 12
The prevalence of hypertension in children with renal scars
Hooman, N., Tafreshi, R. I., Mostafavi, S.-H., Hallaji, F., Tavasoli, A., & Otukesh, H. (2017) · Minerva Pediatrics · doi:10.23736/s0026-4946.16.04217-1 · Cited by 11
Evaluation of function and structure of arterial wall in girls and young women with Turner syndrome
Radetti, G., Mazzanti, L., Somma, C. D., Salerno, M. C., Gottardi, E., Capalbo, D., Tamburrino, F., & Colao, A. (2015) · Journal of Endocrinological Investigation · doi:10.1007/s40618-015-0268-9 · Cited by 9
Non-invasive assessment of aortic stiffness and blood pressure in young Turner syndrome patients
Fox, D. A., Kang, K. T., Potts, J. E., Bradley, T. J., Stewart, L. L., Dionne, J. M., & Sandor, G. G. S. (2019) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem-2018-0346 · Cited by 9
Long-Term Cortisol Exposure and Associations With Height and Comorbidities in Turner Syndrome
Savas, M., Wester, V. L., Dykgraaf, R., van den Akker, E. L. T., Roos‐Hesselink, J. W., Dessens, A. B., de Graaff, L. C. G., de Rijke, Y. B., & van Rossum, E. F. C. (2019) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2019-00148 · Cited by 8
Lee, Y. J., Kim, S. M., Lee, Y. A., Kim, G. B., Shin, C. H., & Yang, S. W. (2019) · Clinical Endocrinology · doi:10.1111/cen.13995 · Cited by 7
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Coronary plaque burden in Turner syndrome a coronary computed tomography angiography study
Funck, K. L., Budde, R. P. J., Viuff, M. H., Wen, J., Jensen, J. M., Nørgaard, B. L., Bons, L. R., Duijnhouwer, A. L., Dey, D., Mortensen, K. H., et al. (2021) · Heart and Vessels · doi:10.1007/s00380-020-01660-7 · Cited by 6
Progression of Vasculopathy in Young Individuals with Turner Syndrome
Blunden, C. E., Urbina, E. M., Lawson, S. A., Gutmark‐Little, I., SHAH, A. S., Khoury, P. R., & Backeljauw, P. F. (2020) · Pediatric Cardiology · doi:10.1007/s00246-020-02505-w · Cited by 6
RELATIONSHIP BETWEEN ARTERIAL STIFFNESS AND CIRCADIAN PATTERN OF BLOOD PRESSURE
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Arterial stiffness in young women with Turner syndrome using cardio-ankle vascular index
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Flagged first, then by citations
1 of the 116 references couldn’t be matched to a known paper.
Research works printed but not linked: 2
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Clinical practice guidelines for the care of girls and women with Turner syndrome.
Gravholt, C. H., Andersen, N. H., Christin-Maitre, S., Davis, S. M., Duijnhouwer, A., Gawlik, A., Maciel-Guerra, A. T., Gutmark-Little, I., Fleischer, K., Hong, D., et al. (2024) · European Journal of Endocrinology · doi:10.1093/ejendo/lvae050 · Cited by 224
Hypertension: Causes and Consequences of Circadian Rhythms in Blood Pressure
Faraci, F. M., & Scheer, F. A. J. L. (2024) · Circulation Research · doi:10.1161/circresaha.124.323515 · Cited by 91
JCS/JCC/JSPCCS 2024 Guideline on Genetic Testing and Counseling in Cardiovascular Disease
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JCS/JCC/JSPCCS 2024 Guideline on Genetic Testing and Counseling in Cardiovascular Disease
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Sabbadin, C., Marin, L., Manso, J., Mozzato, C., Camozzi, V., Andrisani, A., Sacchetti, C., Mian, C., Scaroni, C. M., Guazzarotti, L., et al. (2024) · Expert Review of Endocrinology & Metabolism · doi:10.1080/17446651.2024.2347265 · Cited by 2
Refractory hypertension complicated with Turner syndrome: A case report
Hu, S., Liu, J., Tang, H., Xie, X., & Wei, Y. (2024) · Open Life Sciences · doi:10.1515/biol-2022-0934 · Cited by 1
Malignant hypertension in a patient with Turner syndrome: A case report
Yang, Y., Ye, Y., Wang, H., Wu, H., Zhang, J., Lv, Z., Li, W., & Yang, J. M. (2024) · Medicine · doi:10.1097/md.0000000000039128 · Cited by 1
Efforts to Prevent Hypertension in The Kaili Tribe Community
Mustaqima, Thaha, R. M., & Syafar, M. (2024) · Revista de Gestão Social e Ambiental · doi:10.24857/rgsa.v18n7-127
Chromosomal Abnormalities Associated with Congenital Heart Disease
Maeda, J. (2024) · Japanese Society of Pediatric Cardiology and Cardiac Surgery · doi:10.9794/jspccs.40.41
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
Aortic dissection in a patient with Turner syndrome: a case report
Butenko, D. S., Alekberov, R. I., Spasennikov, V. V., Kleschevnikova, T. M., Talybova, A. Y., & Сабирович, Т. Р. (2025) · Russian Journal of Cardiology · doi:10.15829/1560-4071-2025-6425
Exercise capacity in girls with Turner syndrome
Debo, B., Coomans, I., Vandekerckhove, K., & Groote, K. D. (2025) · European Journal of Pediatrics · doi:10.1007/s00431-025-06664-x
Metadata
- Authors
- Jones, L., Blair, J., Hawcutt, D. B., Lip, G. Y. H., & Shantsila, A.
- Affiliations
- University of Liverpool (GB)
- Alder Hey Children's NHS Foundation Trust (GB)
- Corresponding author
- Shantsila, Alena
- Venue
- Journal of Hypertension
- Published
- 2022-11-02
- Volume
- 41
- Issue
- 2
- Pages
- 203-211
- ISSN
- 0263-6352 (ISSN-L)
- 1473-5598
- OpenAlex
- W4313296101
- Cited by
- 13
- References
- 116
- Citations by year
- 2025: 5 · 2024: 8
- FWCI
- 0.50
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Hypertension (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Blood Pressure and Hypertension Studies
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
- Keywords
- Turner syndrome, hypertension, sympathetic nervous system, vasculopathy, hormonal factors, antihypertensive therapy, cardiovascular risk, premature mortality
- MeSH terms
- Antihypertensive Agents, Heart, Humans, Hypertension, Quality of Life, Turner Syndrome
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