Long-term outcomes of clinically significant vascular rings associated with congenital tracheal stenosis

Congenital tracheal stenosis (CTS) associated with vascular rings (VRs) is considered a secondary condition, but relative indications for selective VRs or combined VR and CTS repairs are unclear. Here, we report long-term outcomes after VR repair associated with tracheal narrowing (TN).

Retrospective analysis of patients admitted to a single institution with VR from 1982 to 2004. Of 131 patients with VR (Cardiac Registry Database), 29 (19 males, 10 females; age range, 0 days-4 years) associated with symptomatic TN. Patients were followed until age 18 years.

One hundred thirty-one patients with VR were categorized into 3 treatment groups. There were 102 patients with VR with no symptomatic TN treated by VR repair (4 deaths, 4% mortality). Of the 29 patients with VR + TN symptoms, 16 were treated by selective VR repair (mean age, 5.3 months; range, 17-288 days) with 3 deaths (19% mortality) and 13 underwent combined VR and CTS repairs (mean age, 11 months; range, 1 day-4 years) with 6 deaths (46% mortality), for an overall mortality of 31% (P < .01, chi2 test). Of the 102 patients with VR, 17 had intracardiac anomalies with 3 deaths (18% mortality). In contrast, 7 of 29 patients with VR with TN and intracardiac anomalies died (57% mortality); 3 patients in combined cardiac and CTS repairs (2 deaths, 67% mortality) and 4 in selective VR repairs (2 deaths, 50% mortality).

Not all cases of TN associated with VR require combined repairs. The presence of TN and/or intracardiac anomalies in patients with VR, however, increases mortality risk to the repair of VR. Surgical decision requires individualized clinical pathway.