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.