title: 22q11.2 Deletion syndrome is associated with increased perioperative events and more complicated postoperative course in infants undergoing infant operative correction of truncus arteriosus communis or interrupted aortic arch.
abstract: OBJECTIVE
  The effect of genotype on the outcomes of infant cardiac operations has not been well established. The purpose of the present study was to investigate the effect of 22q11.2 deletion (22q11del) on infants with truncus arteriosus communis (TA) and interrupted aortic arch (IAA) undergoing operative correction during infancy.
  
  
  METHODS
  We conducted a retrospective cohort study of all infants who had undergone operative correction of TA or IAA at the Children's Hospital of Philadelphia from 1995 to 2007, comparing the perioperative outcomes (hospital length of stay, intensive care, mechanical ventilation, risk of cardiac and noncardiac events, number of consultations, and number of discharge medications) by 22q11del status.
  
  
  RESULTS
  A total of 104 patients were studied (55 with TA and 49 with IAA), of whom 40 (38%) were 22q11del positive. The 22q11del status was unknown in 9 (7 with TA and 2 with IAA). In patients with known deletion status, those with 22q11del had a longer hospital and intensive care length of stay. Subjects with 22q11del also required more frequent operative reintervention and more consultations and were prescribed more medications at discharge. No significant difference was found in method of feeding between those with and without 22q11del at discharge.
  
  
  CONCLUSIONS
  In this study, 22q11del is associated with perioperative outcomes in infants undergoing operative correction of TA and IAA, with longer hospital stays and greater resource utilization in the perioperative period. These findings should inform counseling and risk stratification and warrant additional study to identify genotype-specific management strategies to improve outcomes.
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa. Electronic address: obyrnem@email.chop.edu.
authors.forename: Michael L
authors.initials: ML
authors.lastname: O'Byrne
authors.affiliation: Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa.
authors.forename: Wei
authors.initials: W
authors.lastname: Yang
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa.
authors.forename: Laura
authors.initials: L
authors.lastname: Mercer-Rosa
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Healthcare of Mississippi, University of Mississippi Medical Center, Jackson, Miss.
authors.forename: Aimee S
authors.initials: AS
authors.lastname: Parnell
authors.affiliation: Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Matthew E
authors.initials: ME
authors.lastname: Oster
authors.affiliation: Department of Anesthesiology and Critical Care, Alfred I. duPont Hospital for Children, Wilmington, Del, and Jefferson Medical College, Philadelphia, Pa.
authors.forename: Yosef
authors.initials: Y
authors.lastname: Levenbrown
authors.affiliation: Department of Pediatrics, University of California, San Francisco, School of Medicine, and Division of Pediatric Cardiology, UCSF Benioff Children's Hospital, San Francisco, Calif.
authors.forename: Ronn E
authors.initials: RE
authors.lastname: Tanel
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Goldmuntz
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities*; D006348:Cardiac Surgical Procedures; D004062:DiGeorge Syndrome / Q000150:complications*; D005260:Female; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006330:Heart Defects, Congenital / Q000235:genetics* / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007231:Infant, Newborn; D007902:Length of Stay / Q000706:statistics & numerical data; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology / Q000235:genetics; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome