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.
Chiu
Priscilla P L
PP
Division of General Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, M5G 1X8, Canada.
Rusan
Maria
M
Williams
William G
WG
Caldarone
Christopher A
CA
Kim
Peter C W
PC
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