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

Abstract

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.

Keywords: International Turner Syndrome Registry; Turner syndrome; hypertension.

Figure 1

Age distribution (at the inclusion in the study) of the cohort in relation to the hypertension diagnosis (n = 182).

Figure 2

Blood pressure control recorded at the most recent clinic visit among patients with and without aortic pathology. Blood pressure data were available for 92 individuals (50.5%) of the total cohort and 22 individuals (91.7%) with hypertension diagnosis. DBP:diastolic blood pressure; SBP: systolic blood pressure.