Abstract
Aortic dilation following single ventricle palliation is increasingly recognized, although its long-term clinical implications remain unclear. We present the case of a 38-year-old male born with a bicuspid aortic valve, d-transposition of the great arteries, ventricular septal defect, right ventricular hypoplasia with a straddling right atrioventricular valve. He was palliated with Fontan circulation and developed severe neo-aortic root and arch dilation with native aortic valve stenosis, requiring surgical intervention. His neo-aortic root measured 81 × 65 mm, necessitating a Bentall procedure with total arch and native aortic valve replacement. This case underscores the variable progression of aortic dilation in patients with palliated single ventricle physiology and the need for strict surveillance and individualized management. Larger cohort studies are needed to further refine the criteria for surgical intervention in this unique population.
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