患有威廉姆斯综合征的患者因瓣上主动脉狭窄接受主动脉成形术的麻醉管理。

Anaesthetic management of a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis.

作者信息

Kawahito S, Kitahata H, Kimura H, Tanaka K, Sakai Y, Hirose Y, Oshita S

机构信息

Department of Anaesthesiology, Tokushima University School of Medicine, Japan.

出版信息

Can J Anaesth. 1998 Dec;45(12):1203-6. doi: 10.1007/BF03012465.

DOI:10.1007/BF03012465

PMID:10051941

Abstract

PURPOSE

A case of a patient associated with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis is presented.

CLINICAL FEATURES

Williams syndrome is a rare disease associated with a characteristic facies, supravalvular aortic stenosis, and mental retardation. A 15-yr-old girl with Williams syndrome underwent aortoplasty for supravalvular aortic stenosis. Anaesthesia was induced with fentanyl and thiamylal, and maintained with nitrous oxide, oxygen, sevoflurane, and continuous intravenous infusion of fentanyl. Supravalvular aortic stenosis was evaluated using a multiplane transesophageal echocardiography (TEE) probe before and after repair.

CONCLUSION

Multiplane TEE was found to be very useful for anaesthetic management in a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis.

摘要

目的

介绍1例患有威廉姆斯综合征的患者因主动脉瓣上狭窄接受主动脉成形术的病例。

临床特征

威廉姆斯综合征是一种罕见疾病，伴有特殊面容、主动脉瓣上狭窄和智力发育迟缓。一名15岁患有威廉姆斯综合征的女孩因主动脉瓣上狭窄接受了主动脉成形术。麻醉诱导采用芬太尼和硫喷妥钠，维持采用氧化亚氮、氧气、七氟醚，并持续静脉输注芬太尼。在修复前后使用多平面经食管超声心动图（TEE）探头评估主动脉瓣上狭窄情况。