Ann Card Anaesth. 2026 Oct 1;29(4):622-626. doi: 10.4103/aca.aca_317_25. Epub 2026 Oct 12.
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.
PMID:42862682 | DOI:10.4103/aca.aca_317_25