Abstract 15706: Survival in Children With Trisomy 21 Undergoing Single Ventricle Palliation
Background: Children with Trisomy 21 (T21) and single ventricle anatomy (SV) may have increased mortality compared to those without T21. Supporting data are limited. Our objectives were to describe survival in children with T21 and SV and determine risk factors associated with mortality.
Methods: We performed a single-center retrospective review. All patients born since 1980 with T21 and SV were included. We used Kaplan-Meier (KM) analysis with comparison by log rank to evaluate characteristics potentially associated with mortality in this cohort.
Results: Of 28 patients with T21 and SV, 26 (93%) had unbalanced atrioventricular septal defects with 13 (50%) being RV dominant. One patient had hypoplastic left heart syndrome and one had tricuspid atresia. 22 patients (79%) underwent pulmonary artery (PA) banding. Superior cavopulmonary connection (SCPC) was performed in 17 patients (61%) at a median age of 13 months (range 3 mo [[Unable to Display Character: –]] 13 y), with a banded PA left undivided in most. Three of these (18%) went on to total cavopulmonary connection (TCPC). Single-stage TCPC was performed in 2 other patients (7%). Median age at follow-up was 5 years (range 0.1 [[Unable to Display Character: –]] 33 y) overall and 7.5 years (range 1.5 [[Unable to Display Character: –]] 33 y) in survivors. One-year KM survival was 75% (95% CI 59-91%). Of 7 patients who died < 1 year old, only 1 had undergone SCPC. Two-year KM survival was 60% (95% CI 42-79%) with limited mortality beyond 2 years (Figure). Seventeen patients (61%) reached 5 years of age with 13 having SCPC by that age. Of 5 patients with TCPC, 4 are alive (age range 7 [[Unable to Display Character: –]] 14 y). Pulmonary vascular resistance (PVR) ≥ 3 WU*m2 in the first year of life was associated with increased mortality (p = 0.028) (Figure). No patient with PVR < 3 WU*m2 died. There was a trend toward association between ≥ moderate atrioventricular valve regurgitation and mortality (p = 0.071).
Conclusions: While T21 patients with SV remain a high-risk group, survival is excellent when PVR is < 3 WU*m2 in the first year of life with minimal attrition beyond 2 years of age.
Author Disclosures: J. Colquitt: None. S.A. Morris: None. S. Denfield: None. C.D. Fraser: None. W.B. Kyle: None.
- © 2014 by American Heart Association, Inc.