Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome.
10.5090/kjtcs.2015.48.4.272
- Author:
Hyung Tae SIM
1
;
Dong Ju SEO
;
Jeong Jin YU
;
Jae Suk BAEK
;
Hyn Woo GOO
;
Jeong Jun PARK
Author Information
1. Department of Thoracic and Cardiovascular Surgery, Saint Carollo General Hospital, Korea.
- Publication Type:Case Report
- Keywords:
Loeys-Dietz syndrome;
Aortic root;
Aortic valve, surgery;
Aneurysm;
Aorta
- MeSH:
Aneurysm;
Aorta;
Aortic Aneurysm;
Aortic Diseases;
Child*;
Connective Tissue;
Humans;
Loeys-Dietz Syndrome*;
Transplants
- From:The Korean Journal of Thoracic and Cardiovascular Surgery
2015;48(4):272-276
- CountryRepublic of Korea
- Language:English
-
Abstract:
Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.