Type a Aortic Dissection and Impaired Systolic Function in a Child with Loeys-Dietz Syndrome

Yan Li, Qian Chang, Xiangyang Qian, Xiaogang Sun and Mingyao Luo

Department of Cardiovascular Surgery, National Center for Cardiovascular Diseases and Fuwai Hospital, China

^*Correspondance to: Yan Li 

 PDF  Full Text DOI: 10.25107/2474-1647.1329

Abstract

Loeys-Dietz syndrome (LDS) is a recently recognized genetic aortic aneurysm syndrome characterized by the triad of hypertelorism, bifid uvula or cleft palate, and generalized arterial tortuosity with aneurysms and dissections throughout the arterial tree with a high risk of earlyonset aortic dissection but with no known myocardial involvement [1]. We report a child who was diagnosed as acute type A aortic dissection, a novel transforming growth factor beta (TGFβ) receptor type I (TGFBR1) mutation, and impaired systolic function after urgent surgical repair.

Keywords

LDS: Loeys-dietz Syndrome; TGFβ: Transforming Growth Factor β; TGFBR1: TGF Beta-receptor Type I; LVEF: Left Ventricular Ejection Fraction

Cite the article

Li Y, Chang Q, Qian X, Sun X, Luo M. Type a Aortic Dissection and Impaired Systolic Function in a Child with Loeys- Dietz Syndrome. Clin Surg. 2017; 2: 1329.