Journal Basic Info
- Impact Factor: 1.995**
- H-Index: 8
- ISSN: 2474-1647
- DOI: 10.25107/2474-1647
Major Scope
- Colon and Rectal Surgery
- Transplant Surgery
- General Surgery
- Surgical Oncology
- Endocrine Surgery
- Oral and Maxillofacial Surgery
- Orthopaedic Surgery
- Ophthalmic Surgery
Abstract
Citation: Clin Surg. 2017;2(1):1329.Case Report | Open Access
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
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.
