Pediatric David procedure for aneurysmally dilated ascending aorta in Loeys-Dietz syndrome.

Loeys-Dietz syndrome (LDS) is a rare congenital disorder that necessitates surgical intervention for a dilated ascending aorta. This case report details a case involving a 3-year-old patient diagnosed with Loeys-Dietz syndrome, presenting with a significantly dilated ascending aorta and severe aortic insufficiency, alongside various connective tissue anomalies associated with the syndrome. Intraoperative assessment revealed severe dilatation of the aortic root and ascending aorta. The aortic valve was tricuspid. Given the valve's near-normal anatomy, a valve-sparing root replacement (VSRR) was preferred; the David procedure (reimplantation) using a 22-mm Dacron tube graft was performed. The patient is presently in a satisfactory condition, 11 months post-operatively. This case exemplifies the successful surgical correction of an aneurysmally dilated ascending aorta through the preservation of the native valve, thereby minimizing the risk of subsequent surgical intervention. Nevertheless, regular follow-up evaluations remain essential.