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

BACKGROUND

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

METHODS

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.

RESULTS

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%).

CONCLUSIONS

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.