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Published on: May 3, 2018
Increased arterial stiffness in children with Williams syndrome and normal blood pressure
Pier Paolo Bassareo1, Giuseppe Mercuro
1Department of Cardiovascular and Neurological Sciences, University of Cagliari, Policlinico Universitario, Monserrato, Cagliari, Italy. piercard@inwind.it
Insights
Children with Williams syndrome (WS) exhibit increased arterial stiffness, even when their blood pressure (BP) is normal. This finding suggests potential future cardiovascular risks for individuals with WS.
Area of Science:
- Cardiovascular Medicine
- Pediatrics
- Genetics
Background:
- Williams syndrome (WS) is associated with a high prevalence of hypertension, often linked to reduced arterial elasticity.
- Early detection of cardiovascular abnormalities is crucial for managing WS patients.
Purpose of the Study:
- To investigate arterial compliance in normotensive children with WS compared to healthy controls.
- To determine if arterial stiffness is elevated in young WS patients, irrespective of blood pressure status.
Main Methods:
- Employed the QKd100-60 method for noninvasive measurement of arterial stiffness in 29 children with WS.
- Conducted 24-hour ambulatory blood pressure monitoring and transthoracic echocardiography.
Main Results:
- All WS patients demonstrated significantly lower arterial compliance (higher stiffness) compared to the control group (P<0.0001).
- No significant difference in arterial stiffness was observed between hypertensive and normotensive WS children (P=NS).
- Hypertensive WS children showed distinct ambulatory blood pressure monitoring profiles versus normotensive WS individuals (P<0.0001).
Conclusions:
- Arterial stiffness is a significant finding in Williams syndrome, present even in normotensive individuals.
- Elevated arterial stiffness in WS patients may indicate a predisposition to adverse cardiovascular outcomes, regardless of current blood pressure levels.
Objective:
High blood pressure (BP), probably related to the loss of arterial elasticity, frequently arises in patients affected by Williams syndrome (WS). This study aimed at proving that arterial compliance, evaluated by automated recording of the QKd interval, was lower even in young normotensive patients with WS than in a control group of healthy individuals with normal BP values.
Methods:
Twenty-nine children (12.8±4.1 years) suffering from WS (both hypertensive and normotensive) were enroled. Arterial stiffness was measured by the standardized noninvasive QKd100-60 method. A 24-h ambulatory BP monitoring and a transthoracic echocardiography were also performed.
Results:
The WS hypertensive children showed significant differences in ambulatory BP monitoring profile in comparison with WS normotensive individuals (systolic BP: P<0.0001, diastolic BP: P<0.0001, mean BP: P<0.0001). All of the 29 WS patients in the study showed disadvantageous differences in QKd100-60 value (P<0.0001) compared with control group. No statistical significant differences were found in QKd100-60 value between the hypertensive and normotensive children affected by WS (P=not significant).
Conclusion:
Our data show that arterial stiffness is increased in WS, even in patients with normal BP. It might signify a poor future outcome even in those WS patients with apparently no severe cardiovascular involvement.
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