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Massive aortic root aneurysm in an infant with the Loeys-Dietz syndrome
Tatiana Molina-Sánchez1, Juan Calderón-Colmenero1, Juan Pablo Sandoval1
1Department of Pediatric Cardiology,Ignacio Chavez National Institute of Cardiology,Mexico City,Mexico.
Cardiology in the Young
|June 14, 2017
Insights
Aortic root aneurysm in a 10-month-old with Loeys-Dietz syndrome was successfully repaired using valve-sparing surgery. This case highlights early intervention for aortic root dilation in pediatric patients.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Genetics
Background:
- Loeys-Dietz syndrome (LDS) is a rare genetic connective tissue disorder.
- Aortic root aneurysm is a common and serious complication of LDS, increasing risk of dissection or rupture.
- Early diagnosis and management are crucial for improving outcomes in affected children.
Observation:
- A 10-month-old female infant diagnosed with Loeys-Dietz syndrome type I presented with a significant aortic root aneurysm.
- Imaging via chest X-ray and CT reconstruction clearly visualized the aortic root dilation.
- The patient's young age and the conspicuous nature of the aneurysm prompted surgical consideration.
Findings:
- The infant underwent a successful valve-sparing repair of the ascending aorta.
- This surgical approach preserved the native aortic valve function.
- The procedure effectively addressed the aortic root aneurysm in this pediatric patient.
Implications:
- Valve-sparing aortic root repair is a viable and effective option for managing aortic aneurysms in infants with Loeys-Dietz syndrome.
- Early surgical intervention can prevent life-threatening aortic events in young children with LDS.
- This case underscores the importance of vigilant cardiovascular monitoring and timely surgical management in pediatric genetic syndromes affecting the aorta.
Abstract:
A 10-month-old girl with type I Loeys-Dietz syndrome developed a conspicuous aortic root aneurysm that was well demonstrated on chest X-ray/CT reconstruction. She underwent successful valve-spare repair of the ascending aorta.
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