Total Aortic Replacement for a 9-Year-Old Boy With Loeys-Dietz Syndrome

被引：7

作者：

Inoue, Yosuke ^{[1
]}

Minatoya, Kenji ^{[1
]}

Oda, Tatsuya ^{[1
]}

Itonaga, Tatsuya ^{[1
]}

Seike, Yoshimasa ^{[1
]}

Tanaka, Hiroshi ^{[1
]}

Sasaki, Hiroaki ^{[1
]}

Kobayashi, Junjiro ^{[1
]}

机构：

[1] Natl Cerebral & Cardiovasc Ctr, Dept Cardiovasc Surg, 5-7-1 Fujishirodai, Suita, Osaka 5650858, Japan

来源：

ANNALS OF THORACIC SURGERY | 2016年 / 101卷 / 03期

关键词：

SURGICAL EXPERIENCE;

D O I：

10.1016/j.athoracsur.2015.05.071

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Loeys-Dietz syndrome (LDS) is a recently identified rare connective tissue disorder caused by mutations of the transforming growth factor-beta receptors and first described in 2005. It is an autosomal dominant syndrome with 2 different phenotypic expressions-LDS I and II. LDS is characterized by the triad of arterial tortuosity and aneurysm, hypertelorism, and a bifid uvula or cleft palate. We present a case of a 9-year-old boy diagnosed with LDS who underwent urgent thoracoabdominal aortic aneurysm repair followed by total arch replacement and aortic valve-sparing root replacement (AVSRR). (C) 2016 by The Society of Thoracic Surgeons

引用

页码：1185 / 1188

页数：5

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