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马方综合征:青春期前主动脉破裂合并左冠状动脉瘤和瘘管。

Marfan's syndrome: pre-pubertal aortic rupture with left coronary artery aneurysms and fistulas.

作者信息

Williams-Phillips S

机构信息

Andrews Memorial Hospital, TAI Wing, 27 Hope Road, Kingston 10, Jamaica.

出版信息

West Indian Med J. 2012 Dec;61(9):937-40. doi: 10.7727/wimj.2012.233.

Abstract

Aortic dissection and rupture occur in 20-40% of patients with Marfan's syndrome. This occurs predominantly in the third and fourth decade of life, contributing to the increased morbidity and mortality of this specific group of patients. This is the first known documented case report of prepubertal left coronary sinus rupture with left coronary artery aneurysms with fistulous communication to both the superior vena cava and right superior pulmonary vein, presenting with a continuous murmur

摘要

20%至40%的马凡综合征患者会发生主动脉夹层和破裂。这主要发生在生命的第三个和第四个十年,导致这一特定患者群体的发病率和死亡率增加。这是首例有记录的青春期前左冠状动脉窦破裂合并左冠状动脉瘤并与上腔静脉和右上肺静脉形成瘘管交通的病例报告,表现为连续性杂音

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