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

Abstract

Introduction: Cardiovascular disease is the commonest 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 multi-centre observational study of patients aged ≥18 years, included in the I-TS (International-TS) registry (2020-2022) utilising registry and participating centre collected data.

Results: Twelve international centres participated, including 182 patients with the 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 the diagnosis. The majority (75%) were classified as primary hypertension (n=18). In binomial regression analysis, higher body-mass-index (BMI) 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 (ACEi) were the commonest (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 form a young age. Overweight/obesity was a notable risk factors for hypertension. The frequency of sub-optimal BP control among high-risk patients highlights the importance of increased awareness and TS-specific consensus guidance on management.