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Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome
Hyung-Tae Sim1, Dong Ju Seo2, Jeong Jin Yu3
1Department of Thoracic and Cardiovascular Surgery, Saint Carollo General Hospital.
Insights
Loeys-Dietz syndrome (LDS) patients with aortic root aneurysms can be treated with valve-sparing aortic root replacement (VSRR). This surgical approach is safe and effective for children, avoiding the need for prosthetic valve replacement.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Pediatric Cardiology
Background:
- Loeys-Dietz syndrome (LDS) is a genetic disorder causing aggressive arterial and aortic disease.
- Aortic aneurysms are a common and dangerous complication in LDS patients.
- Current treatment options for pediatric LDS with aortic aneurysms require careful consideration.
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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