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


METHODS
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.


RESULTS
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).


CONCLUSIONS
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.
Division of General Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, M5G 1X8, Canada.
Priscilla P L
PP
Chiu
Maria
M
Rusan
William G
WG
Williams
Christopher A
CA
Caldarone
Peter C W
PC
Kim
D000015:Abnormalities, Multiple / Q000601:surgery*; D001808:Blood Vessels / Q000002:abnormalities*; D002675:Child, Preschool; D019091:Critical Pathways; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D013997:Time Factors; D014135:Tracheal Stenosis / Q000150:complications* / Q000151:congenital / Q000601:surgery*; D016896:Treatment Outcome