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Generalized arteriopathy in Williams syndrome: an intravascular ultrasound study
A J Rein1, T J Preminger, S B Perry
1Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115.
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.