Heart 2000;84:290-293 ( September )

Cardiovascular medicine

Mechanical properties of the common carotid artery in Williams syndrome Y Aggoun^a, D Sidi^a, B I Levy^b, S Lyonnet^c, J Kachaner^a, D Bonnet^a

^a INSERM 0016 and Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants Malades, 149 rue de Sèvres 75743, Paris Cedex 15, France, ^b Service d'Explorations Fonctionnelles and INSERM U141, Hôpital Lariboisière, Paris, ^c Département de Génétique Médicale, Hôpital Necker-Enfants Malades, Paris

Correspondence to: Dr Bonnet email: damien.bonnet{at}nck.ap-hop-paris.fr

Accepted 28 June 2000

OBJECTIVETo determine whether arterial wall hypertrophy in elastic arteries was associated with alteration in their mechanical properties in young patients with Williams syndrome.
METHODSArterial pressure and intima-media thickness, cross sectional compliance, distensibility, circumferential wall stress, and incremental elastic modulus of the common carotid artery were measured non-invasively in 21 Williams patients (mean (SD) age 8.5 (4) years) and 21 children of similar age.
RESULTSSystolic and diastolic blood pressures were higher in Williams patients (125/66 v 113/60 mm Hg, p < 0.05). The mean (SD) intima-media thickness was increased in Williams patients, at 0.6 (0.07) v 0.5 (0.03) mm (p < 0.001). Normotensive Williams patients had a lower circumferential wall stress (2.1 (0.5) v 3.0 (0.7) mm Hg, p < 0.01), a higher distensibility (1.1 (0.3) v 0.8 (0.3) mm Hg¹.10², p < 0.01), similar cross sectional compliance (0.14 (0.04) v 0.15 (0.05) mm².mm Hg¹, p > 0.05), and lower incremental elastic modulus (7.4 (2.0) v 14.0 (5.0) mm Hg.10²; p < 0.001).
CONCLUSIONSThe compliance of the large elastic arteries is not modified in Williams syndrome, even though increased intima-media thickness and lower arterial stiffness are consistent features. Therefore systemic hypertension cannot be attributed to impaired compliance of the arterial tree in this condition.


Keywords: elastin; Williams syndrome; hypertension; compliance

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© 2000 by Heart
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