Dilated cardiomyopathy associated with elephant trunk in Loeys-Dietz syndrome

被引：1

作者：

Yamana, Koji ^{[1
]}

Sakurai, Hajime ^{[2
]}

Nonaka, Toshimichi ^{[2
]}

Sakurai, Takahisa ^{[2
]}

机构：

[1] Nagoya Grad Sch Med, Dept Cardiac Surg, Nagoya, Aichi, Japan

[2] Chukyo Childrens Heart Ctr, Dept Cardiovasc Surg, Nagoya, Aichi, Japan

来源：

EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY | 2017年 / 51卷 / 04期

关键词：

Loeys-Dietz syndrome; Dilated cardiomyopathy; Elephant trunk;

D O I：

10.1093/ejcts/ezw376

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

A 14-year-old boy presented to our institution with a diagnosis of acute type A dissection. He was diagnosed with Loeys-Dietz syndrome and underwent aortic valve sparing repair at the age of 9 years. Emergency total arch repair with elephant trunk (ET) was performed successfully; echocardiogram before discharge showed normal left ventricular function and size. However, he was readmitted 1 month after discharge with significant left ventricular dysfunction and dilatation. The small folded ET caused a pressure gradient between the upper and lower body, which might deteriorate left ventricular function. Urgent balloon arterioplasty was performed to unfold the ET graft, resulting in no improvement of left ventricular function. ET removal and descending aorta replacement with an 18-mm graft was performed eventually. Left ventricular function and brain natriuretic peptide gradually improved after approximately 2 years of follow-up.

引用

页码：797 / 798

页数：2