Aortic root replacement after repaired congenital conotruncal defects: A surgical challenge

Aortic root replacement (ARR) for patients with repaired conotruncal defects is challenging. We report outcomes of ARR in this complex patient population.

After querying 2710 ARR cases at 2 academic centers between 2004 and 2021, 18 patients (0.7%) with previously repaired conotruncal defects were identified. These included 10 patients with tetralogy of Fallot, 4 with transposition, 2 with double-outlet right ventricle, 1 with truncus arteriosus, and 1 with single ventricle with pulmonary atresia. Surgical history, baseline characteristics, hospital courses, and outcomes were reviewed from the medical records. Survival probability was estimated with Kaplan-Meier analysis.

The patients ranged in age from 15 years to 68 years, and 2 (11.1%) were female. Mean root diameter was 54 ± 13 mm. The indication for ARR was aneurysm with aortic insufficiency in 12 patients (66.7%), aneurysm alone in 5 (27.8%), and pseudoaneurysm in 1 (5.7%). Nine patients (50%) had 1 previous sternotomy, 6 (33%) had 2 previous sternotomies, and 3 (16.7%) had 3 or more previous sternotomies. A valve-sparing operation might have been possible in 12 patients but ultimately was performed in 3 (16.7%). Concomitant procedures included pulmonary valve replacement (n = 7; 38.9%), coronary artery bypass grafting (n = 3; 16.7%), and tricuspid (n = 1) and mitral (n = 1) valve repairs. Mean bypass and cross-clamp times were 245 ± 77 minutes and 174 ± 74 minutes, respectively. There were 3 in-hospital deaths from stroke and multiorgan failure. The 5-year survival probability was 76.2% (95% confidence interval, 58.2%-99.7%).

The development of aortic root pathology requiring ARR following repaired conotruncal defects occurs infrequently. Valve-sparing operations are rarely performed or even possible. Given the significant mortality, careful consideration should be given to performing ARR in this complex patient population.