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.
Abstract

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