Multiple Aortic Operations in Loeys-Dietz Syndrome: Report of 2 Cases.
10.5090/kjtcs.2014.47.6.536
- Author:
Kwon Joong NA
1
;
Kay Hyun PARK
Author Information
1. Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Korea. drkpark@gmail.com
- Publication Type:Case Report
- Keywords:
Loeys-Dietz syndrome;
Marfan syndrome;
Aortic aneurysm;
Aorta, surgery
- MeSH:
Aortic Aneurysm;
Connective Tissue Diseases;
Diagnosis, Differential;
Humans;
Loeys-Dietz Syndrome*;
Marfan Syndrome;
Pathology;
Prevalence
- From:The Korean Journal of Thoracic and Cardiovascular Surgery
2014;47(6):536-540
- CountryRepublic of Korea
- Language:English
-
Abstract:
Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often mis-diagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology. However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making. We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.
