Annulus downsizing in valve-sparing aortic root replacement predicts aortic valve reoperation in children and young adults.

Objective: Valve-sparing root replacement effectively treats aortic root pathology in adults, but data in pediatric patients are limited. We analyzed the midterm outcomes of valve-sparing root replacement in children and adolescents to define the risk factors for reintervention. Methods: From 2006 to 2023, 80 patients underwent valve-sparing root replacement at 15 [11-19] years; 31 had connective tissue disorders, and 34 had concomitant greater than mild regurgitation. The primary end points were postoperative aortic valve dysfunction and reintervention with secondary outcome of mortality. Root and graft diameters were compared using Cox regression to determine the effect of intraoperative sizing. Results: The reimplantation technique with straight-tube (n = 38) or Valsalva (n = 40) grafts was used in all patients (except n = 2 remodeling with straight-tube). Thirty-seven patients underwent concurrent valvuloplasty, and 62 patients underwent ascending aortic replacement. Two patients had more than mild regurgitation at discharge with no perioperative mortality. The most common complication was arrhythmia requiring medication (11%). At 3.6 [0.6-8.2] years follow-up, 4 patients died, 20 patients required reoperation (4 re-repairs, 16 replacements), and 13 patients developed more than mild regurgitation. At 12 years, freedom from death, reintervention, and recurrent regurgitation were 92.0% (80.0%-96.9%), 54.3% (36.1%-69.4%), and 48.7% (25.1%-68.7%). For every 0.1 decrease in ratio of graft size to preoperative annulus diameter, reintervention risk increased by 40% (hazard ratio, 1.40 [1.04-1.90], P = .028). A ratio of less than 1.05 maximized model discrimination (hazard ratio, 3.30 [1.15-9.50], C-index 0.68). Conclusions: Valve-sparing root replacement is safe and effective for aortic root aneurysms in children and young adults. Early arrhythmias, recurrent regurgitation, and midterm reoperation remain concerns. Graft upsizing or leaflet modification should be considered if ratio of intended graft to preoperative diameter is less than 1.05. Preoperative imaging can guide appropriate graft selection and plication extent to mitigate reintervention risk associated with excessive downsizing.