Prevalence and management of hypertension in Turner syndrome: data from the International Turner Syndrome (I-TS) registry

Introduction: Cardiovascular disease is the most common cause of death in Turner syndrome (TS) for which arterial hypertension has a direct influence and is a key modifiable risk factor. Objective: To investigate the prevalence and patterns of hypertension diagnosis and management in adult patients...

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Main Authors: Shani A D Mathara Diddhenipothage, Katharina J Beck, Gayana Amiyangoda, Jillian Bryce, Luminita Cima, Katya De Groote, Yana Deyanova, Evgenia Globa, Gloria Herrmann, Anders Juul, Anna Sophie L Kjaer, Anette Tønnes Pedersen, Sukran Poyrazoglu, Ursina Probst-Scheidegger, Theo C J Sas, Simona Fica, Sumudu Nimali Seneviratne, Justyna Karolina Witczak, Elizabeth Orchard, Jeremy W Tomlinson, S Faisal Ahmed, Helen E Turner
Format: Article
Language:English
Published: Bioscientifica 2025-01-01
Series:Endocrine Connections
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Online Access:https://ec.bioscientifica.com/view/journals/ec/14/2/EC-24-0477.xml
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Summary:Introduction: Cardiovascular disease is the most common cause of death in Turner syndrome (TS) for which arterial hypertension has a direct influence and is a key modifiable risk factor. Objective: To investigate the prevalence and patterns of hypertension diagnosis and management in adult patients with TS who are registered in a large international multicentre database (TS-HTN study). Methods: Retrospective multicentre observational study of patients aged ≥18 years included in the I-TS (International-TS) registry (2020–2022), using registry and participating centre-collected data. Results: Twelve international centres participated, including 182 patients with a median age of 28 years (IQR 23–37.2). Arterial hypertension was recorded in 13.2% (n = 24). The median age at hypertension diagnosis was 27 years (range 10–56), with 92% aged less than 50 years at diagnosis. The majority (75%) were classified as primary hypertension (n = 18). In binomial regression analysis, higher body mass index was the only parameter significantly associated with the occurrence of hypertension (B = 1.487, P = 0.004). Among patients with aortic disease (n = 9), 50% had systolic BP ≥ 130 mmHg and 66.6% had diastolic BP ≥ 80 mmHg during the last clinic review. Angiotensin-converting enzyme inhibitors were the most common (n = 16) medication prescribed, followed by angiotensin receptor blockers (n = 6), beta-blockers (n = 6) and calcium channel blockers (n = 6). Conclusions: Arterial hypertension is common in TS and occurs at a young age. Overweight/obesity was a notable risk factor for hypertension. The frequency of suboptimal BP control among high-risk patients highlights the importance of increased awareness and TS-specific consensus guidance on management.
ISSN:2049-3614