Fate of the hypoplastic proximal aortic arch in infants undergoing repair for coarctation of the aorta through a left thoracotomy.

Background: Extended end-to-end anastomosis (EEEA) through a left thoracotomy for coarctation of the aorta (CoA) and tubular hypoplasia of the aortic arch (THAA) leaves an unaugmented hypoplastic proximal aortic arch (PAA) segment, which may increase late reintervention for PAA obstruction. We sought to assess PAA growth and reintervention for PAA obstruction after EEEA.

Methods: Preoperative and follow-up echocardiographic images of 140 patients who underwent EEEA for CoA from 2005 to 2012 were reviewed. Patients were divided into two groups on the basis of preoperative PAA z-scores: THAA group, z-score less than -3; non-THAA group, z-score greater than or equal to -3.

Results: Eighty (57%) patients were identified as having THAA. There were three surgical reinterventions (PAA in 2 patients and distal aortic arch in 1 patient) and nine catheter reinterventions (all related to anastomotic stenosis) during a median follow-up period of 18 months. Both patients who required PAA reintervention had preoperative PAA z-scores below -8. Freedom from reintervention at 3 years was comparable between the groups (THAA group, 90.0% vs non-THAA group, 87.9%, p = 0.483). Follow-up echocardiography revealed PAA catch-up growth in the THAA group (z-score, preoperative -4.63 vs follow-up -1.17, p < 0.001); however, there was a nonsignificant trend toward smaller PAA in the THAA group (z-score: THAA, -1.17 vs non-THAA, -0.55, p = 0.057). All but 2 patients with preoperative PAA z-scores above -6 did not have any PAA obstruction.

Conclusions: The hypoplastic PAA segment in patients with CoA/THAA grew significantly after EEEA but remained smaller than in those without THAA. Our data support that CoA and PAA with z-scores as small as -6 can be repaired through a thoracotomy approach with a low risk of reintervention.