Abstract 16903: 22q11.2 Status Impacts on Perioperative Outcome in Tetralogy of Fallot
Background: Tetralogy of Fallot (TOF) is commonly associated with genetic syndromes. In particular, deletion of 22q11.2 (del22q) is seen in up to 17% of patients. The influence of del22q on surgical outcomes in TOF is not completely defined. Therefore, we sought to investigate the impact of del22q on perioperative outcomes in TOF.
Methods: We conducted a retrospective review of patients with TOF who underwent complete surgical reconstruction at The Children's Hospital of Philadelphia from1995 to 2006. Inclusion criteria included confirmed diagnosis of TOF and known del22q genotype. Fisher's exact and Mann Whitney tests were used for categorical and continuous variables, respectively. Univariate and multivariate regression analyses were used to determine the association of deletion status with outcome.
Results: We studied 208 subjects, 164 (79%) without, and 44 (20%) subjects with a del22q. There was no difference in sex, race, gestational age, age at diagnosis, admission weight, presence of extracardiac malformations, or presenting anatomy (pulmonary valve stenosis, atresia or absent pulmonary valve leaflets and TOF associated with common atrioventricular canal) between groups. Duration of mechanical ventilation, mortality, number of complications and re-operations were also comparable. Those with del22q had more aortopulmonary shunts preceding complete surgical repair (21% vs. 7%, p= 0.02). This association was present after adjustment for presenting pulmonary valve anatomy, surgical era and extra-cardiac malformations. In addition, those with del22q had longer cardiopulmonary bypass time (84 vs. 72 minutes, p=0.02), and longer duration of intensive care as defined by the time from post-operative admission to the intensive care unit to extubation, removal of chest tubes and intracardiac lines, and discontinuation of vasopressors (6 days vs. 4 days, p=0.007).
Conclusions: Del22q appears to affect early operative outcomes in TOF resulting, in particular, in prolonged intensive care. Future studies are required to determine specific factors that contribute to such differences in this susceptible population. This study highlights that genotype is an important consideration when counseling and caring for TOF patients.
- © 2012 by American Heart Association, Inc.