Rein A J, Preminger T J, Perry S B, Lock J E, Sanders S P
Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115.
J Am Coll Cardiol. 1993 Jun;21(7):1727-30. doi: 10.1016/0735-1097(93)90394-g.
We used intraluminal ultrasound imaging to provide additional information about the vascular pathology in Williams syndrome.
The cardiovascular pathology of Williams syndrome consists of medial hypertrophy in both systemic and pulmonary arteries, which results in lumen narrowing.
Systemic and pulmonary arteries were examined in vivo using intravascular ultrasound imaging (5F, 30-MHz catheter) in three children with Williams syndrome.
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.
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.
我们使用腔内超声成像来提供有关威廉姆斯综合征血管病变的更多信息。
威廉姆斯综合征的心血管病变包括体循环和肺动脉的中层肥厚，这会导致管腔狭窄。
使用血管内超声成像（5F，30兆赫导管）对三名威廉姆斯综合征患儿的体循环和肺动脉进行了体内检查。
所有动脉均表现出严重的管壁增厚并伴有继发性管腔狭窄。两名患儿的一支肺动脉球囊扩张导致管腔口径显著局部增加，并伴有血管壁撕裂。
对威廉姆斯综合征患者进行血管内超声成像可能有助于更好地理解该综合征的病理生理学，并为治疗干预提供更合理的方法。
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