Abstract 12201: Infants With 22q11.2 Deletion Syndrome Undergoing Repair of Tetralogy of Fallot, Interrupted Aortic Arch, and Truncus Arteriosus have Longer Hospitalizations and Increased Rates of Infection
Introduction: 22q11.2 deletion syndrome (22q11del) is common in patients with certain types of congenital heart disease. The prevalence of 22q11del and its impact on clinical outcomes remains unclear as only small single center studies are available and have shown conflicting data.
Methods: All infants with tetralogy of Fallot (TOF), truncus arteriosus (TA), interrupted aortic arch (IAA), or IAA+TA included in the STS Congenital Heart Surgery Database from 1/10 to 6/15 were included. Infants with single ventricle lesions, pulmonary atresia with MAPCAs, and trisomies were excluded. Perioperative mortality and STS-defined major morbidities were compared between patients with and without 22q11del. Analyses were performed using Wilcoxon rank sum or chi-square tests.
Results: A total of 5,179 patients were included. 22q11del was present in 255 (6.3%) patients with TOF, 160 (28.8%) with TA, 206 (38.4%) with IAA, and 19 (28.8%) with IAA+TA undergoing repair. For all procedural cohorts, there was no significant difference in perioperative mortality between the two groups. Infants with 22q11del had a longer hospitalization and a higher incidence of postoperative infections (Table). Within the TOF cohort only, infants with 22q11del had a higher incidence of the STS-defined composite major morbidity measure (12.5% vs. 6.1%, p<0.01), largely driven by an increased incidence of unplanned re-interventions (9.9% vs. 4.3%, p<0.01) and phrenic nerve injuries (2.5% vs. 0.8%, p=0.01).
Conclusions: 22q11del is common in infants undergoing repair of TOF, TA, IAA, and IAA+TA. While operative mortality for the diagnostic groups was not impacted by 22q11del, patients with 22q11del did have longer hospitalizations and a higher incidence of infectious complications. Families should be counseled appropriately and further strategies should be developed to limit the morbidity in patients with 22q11del undergoing congenital heart surgery.
Author Disclosures: M.K. Olive: None. C.M. Mery: None. A.S. Wallace: None. K.D. Hill: None. J.P. Jacobs: None. E.H. Austin: None. S.K. Pasquali: None. M.L. Jacobs: None. J.S. Heinle: None. D.J. Penny: None.
- © 2016 by American Heart Association, Inc.