Natural History of Hypertension in Turner Syndrome During a 12-Year Pragmatic Interventional Study

Abstract

Turner syndrome is caused by complete or partial X monosomy in some or all cells. Cardiovascular complications are dominant, including increased blood pressure (BP), leading to early-onset hypertension. The aim is to describe the debut, development, and treatment of hypertension in Turner syndrome during a 12-year pragmatic interventional study to help identify risk factors associated with hypertension. One hundred and two women (aged 38±11 years, range: 18–62 years) with Turner syndrome verified by karyotyping (45, X: n=58 [57%]) were included consecutively. Ambulatory BPs were recorded over 24 hours with oscillometric measurements every 20 minutes. Antihypertensive treatment was recommended if the BP was above 135/85 mm Hg during the daytime. Overall, systolic BP, diastolic BP, and pulse pressure increased during the study, while heart rate decreased. The number of patients treated with antihypertensive medicine increased from 29 (28.71%) at baseline to 34 (53.13%) at the end of study. Twenty-four–hour systolic BP and 24-hour pulse pressure increased significantly with age, while 24-hour heart rate decreased with age, and diastolic BP was insignificantly affected by age. Antihypertensive treatment lowered systolic BP (24-hour: −5 mm Hg), diastolic BP (24-hour: −5 mm Hg), and diminished the pulse pressure (24-hour: −6 mm Hg) but did not affect nighttime systolic BP. Antihypertensive treatment did not affect heart rate. Our study showed that both systolic and diastolic BP increases significantly in women with Turner syndrome resulting in an increased risk of cardiovascular comorbidities. This increment should be considered of multifactorial origin with many contributing factors which is supported by our results.

Hypertension in Turner syndrome: a review of proposed mechanisms, management and new directions

“…Patients with TS develop hypertension at an early age, and observational studies have reported that the prevalence of hypertension is between 21 and 40% in girls and adolescent females with TS [15][16][17]. A similar pattern in which the prevalence of hypertension increases with age in essential hypertension is also observed in TS [18,19]. A pragmatic, observational 12-year study, of 102 women age 18-62 years, followed for 12 years, demonstrated that age was positively associated with systolic BP: 53% of participants were treated with antihypertensive medication at the end of the study, an increase of 24% over the duration of the study [19].…”

Clinical assessment of blood pressure in 60 girls with Turner syndrome compared to 1888 healthy Danish girls

“…However, it is crucial for treating physicians to be aware of the importance of standardised BP measurement in this group of patients with increased risk of CVD as well as adhesion to guidelines recommending BP measurements at every visit already in childhood to identify hypertension earliest possible. High BP during routine care should alarm clinicians to organise a thorough follow‐up with 24‐h ambulatory BP monitoring to distinguish between white coat and essential hypertension 17 . A recent study showed that without initiation of antihypertensive medication BP increased steadily throughout adulthood in patients with TS while antihypertensive treatment effectively lowered BP, underlining the importance of early intervention with lifestyle changes and medication according to guidelines, if hypertension is diagnosed 3,7,17 …”

“…High BP during routine care should alarm clinicians to organise a thorough follow‐up with 24‐h ambulatory BP monitoring to distinguish between white coat and essential hypertension 17 . A recent study showed that without initiation of antihypertensive medication BP increased steadily throughout adulthood in patients with TS while antihypertensive treatment effectively lowered BP, underlining the importance of early intervention with lifestyle changes and medication according to guidelines, if hypertension is diagnosed 3,7,17 …”

“…Systemic hypertension is an important risk factor for CVD and studies have shown that BP steadily increases in females with TS through adulthood if antihypertensive treatment is not initiated 17 . Thus, repeated BP monitoring during childhood in girls with TS may be important to identify hypertension as soon as it presents and initiate treatment to prevent long‐term risk factors.…”

Turner syndrome: French National Diagnosis and Care Protocol (NDCP; National Diagnosis and Care Protocol)

“…The following acquired and potentially severe cardiovascular complications may be observed: hypertension, aortic dilation (aneurysm) with a potential risk of aortic dissection, more rarely, atherosclerosis and cerebrovascular accidents [47][48][49][50][51][52][53][54][55][56]. There is also a high risk of hypertension (HT) from childhood onwards, and HT is reported in about half of all adult patients [57][58][59][60][61]. The main objective of management is to limit aortic dilation and the risk of dissection.…”