Generalized arteriopathy in Williams syndrome: an intravascular ultrasound study.

OBJECTIVES

We used intraluminal ultrasound imaging to provide additional information about the vascular pathology in Williams syndrome.

BACKGROUND

The cardiovascular pathology of Williams syndrome consists of medial hypertrophy in both systemic and pulmonary arteries, which results in lumen narrowing.

METHODS

Systemic and pulmonary arteries were examined in vivo using intravascular ultrasound imaging (5F, 30-MHz catheter) in three children with Williams syndrome.

RESULTS

All arteries exhibited severe wall thickening with secondary lumen narrowing. Balloon dilation of a branch pulmonary artery in two children resulted in a significant localized increase in lumen caliber associated with a tear in the vessel wall.

CONCLUSIONS

Intravascular ultrasound imaging in patients with Williams syndrome may permit better understanding of the pathophysiology of the syndrome and a more rational approach to therapeutic interventions.