Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome

  • Sim, Hyung-Tae (Department of Thoracic and Cardiovascular Surgery, Saint Carollo General Hospital) ;
  • Seo, Dong Ju (Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Yu, Jeong Jin (Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Baek, Jae Suk (Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Goo, Hyn Woo (Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, Jeong-Jun (Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine)
  • Received : 2014.08.27
  • Accepted : 2014.10.27
  • Published : 2015.08.05
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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.

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References

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  1. Surgery on a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents vol.29, pp.5, 2015, https://doi.org/10.1017/s1047951118002299