Abstract 12358: Heterotaxy Syndrome and Intestinal Malrotation: Impact of the Ladd Procedure
Introduction: Heterotaxy syndrome (HS), defined as abnormal left-right arrangement of thoraco-abdominal organs, is associated with intestinal malrotation. While there is risk of mid-gut volvulus and ischemia in children with HS and malrotation, controversy exists regarding the risks and benefits of prophylactic Ladd procedure. The purpose of this study is to assess the impact of the Ladd procedure on mortality and major morbidity for children with HS and intestinal malrotation.
Hypothesis: The Ladd procedure is associated with increased mortality and morbidity in children with HS and intestinal malrotation.
Methods: We performed a retrospective cohort study using the Pediatric Health Information System (PHIS) database. We included all children with HS and intestinal malrotation initially admitted to a PHIS institution at ≤30 d of age between 1/1/2004 and 1/1/2011. The database was surveyed for subsequent admissions through 1/1/2013 to ensure a minimum 2-yr follow-up. The primary outcomes were in-hospital mortality and major morbidity, defined as a composite variable for intestinal obstruction/volvulus, ischemia, or resection. Univariable and multivariable analyses were performed.
Results: There were 347 patients identified with HS and intestinal malrotation (61% female), including 96 (28%) patients with single ventricle. Mean gestational age was 37.9 ± 2.1 wk and birthweight 3040 ± 650 g; 54% were first identified on birth admission. Median follow-up was 4.9 yr (range 2.1-9.1 yr). Ladd procedure was performed during the initial hospitalization on 210 (61%) children. Overall mortality was 10%, with no difference between those who did and did not receive Ladd procedure (10% vs. 9%; P=0.70). Major morbidity occurred in 68 (20%), with greater morbidity in patients who underwent Ladd procedure than in those who did not (25% vs. 11%; P=0.001). Only Ladd procedure and birthweight were associated with major morbidity on multivariable analysis (both P=0.002).
Conclusions: Ladd procedure was associated with increased subsequent admissions for intestinal obstruction/volvulus, ischemia, or resection in children with HS and malrotation. Further investigation is needed to clarify potential confounders and to determine if this association is causal.
- © 2013 by American Heart Association, Inc.