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Aortic Surgical Emergencies in Young Children With Loeys-Dietz Syndrome
Qian Chang1, Yan Li1, Xiangyang Qian1
1Department of Cardiovascular Surgery, National Center for Cardiovascular Diseases and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Insights
Loeys-Dietz syndrome can cause aortic emergencies in children, even when undiagnosed. Early intervention is crucial for managing aortic disease in these young patients.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Pediatric Cardiology
Background:
- Loeys-Dietz syndrome is a genetic disorder associated with progressive aortic aneurysmal disease, often presenting early in life.
- Aortic emergencies in young children with Loeys-Dietz syndrome are rare but critical events.
- Delayed diagnosis can lead to urgent surgical intervention for aortic complications.
Abstract:
Loeys-Dietz syndrome presents early in life with rapidly progressive aortic aneurysmal disease. Aortic emergency in young children with Loeys-Dietz syndrome is an extremely rare occurrence. In this communication we report on 2 young children whose diagnoses were missed and consequently underwent urgent aortic repair due to aortic emergencies. For personalized management of aortic disease in Loeys-Dietz syndrome patients, when and how do we intervene?
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