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Bentall Procedure in a Patient with Loeys-Dietz Syndrome: A Case Report
Dheeraj Arora1, Supriya Chaudhary1, Rajesh Madavathazathil Gopalakrishnan1
1Department of Anaesthesiology, Amrita Institute of Medical Sciences and Research, Faridabad, Haryana, India.
Abstract:
Loeys-Dietz syndrome (LDS) is a rare autosomal dominant connective tissue disorder characterized by aggressive aortopathy, craniofacial anomalies and systemic involvement. Rapid aortic root dilatation and valvular dysfunction mandate an early surgical intervention. We report a case of 15-year-old boy with LDS, rapidly progressive aortic root dilatation and severe aortic regurgitation (AR) who underwent successful Bentall procedure. The case highlights the need of early detection, multidisciplinary involvement and meticulous anesthetic care.
