title: A genetic contribution to risk for postoperative junctional ectopic tachycardia in children undergoing surgery for congenital heart disease.
abstract: BACKGROUND
  Junctional ectopic tachycardia (JET) is a common arrhythmia complicating pediatric cardiac surgery, with many identifiable clinical risk factors but no genetic risk factors to date.
  
  
  OBJECTIVE
  To test the hypothesis that the angiotensin-converting enzyme insertion/deletion (ACE I/D) polymorphism associates with postoperative JET.
  
  
  METHODS
  DNA samples were collected from children undergoing the Norwood procedure; arterial switch operation; and repairs of Tetralogy of Fallot, balanced atrioventricular septal defect, and ventricular septal defect at a single center. The incidence of postoperative JET was associated with previously identified clinical risk factors and ACE I/D genotype.
  
  
  RESULTS
  Of the 174 children who underwent the above-mentioned surgeries, 21% developed JET. Postoperative JET developed in 31% of children with the D/D genotype but only in 16% of those with the I/I genotype or the I/D genotype (P = .02). Clinical predictors of JET were selected a priori and included age, inotrope score, cardiopulmonary bypass time, and cross-clamp time. Multivariable logistic regression identified a significant correlation between the D/D genotype and postoperative JET independent of these predictors (odds ratio = 2.4; 95% confidence interval, 1.04-5.34; P = .04). A gene-dose effect was apparent in the homogeneous subset of subjects with atrioventricular septal defect (58% JET in D/D subjects, 12% JET in I/D subjects, and 0% JET in I/I subjects; P <.01).
  
  
  CONCLUSIONS
  The common ACE deletion polymorphism is associated with a greater than 2-fold increase in the odds of developing JET in children undergoing surgical repair of atrioventricular septal defect, Tetralogy of Fallot, ventricular septal defect or the Norwood and arterial switch procedures. These findings may support the potential role of the renin-angiotensin-aldosterone system in the etiology of JET.
authors: Thomas P. Graham Jr. Division of Cardiology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital at Vanderbilt, Vanderbilt University School of Medicine, Nashville, Tennessee, USA. Kristie.Borgman@Vanderbilt.edu
authors: Kristie Y
authors: KY
authors: Borgman
authors: Andrew H
authors: AH
authors: Smith
authors: Jill P
authors: JP
authors: Owen
authors: Frank A
authors: FA
authors: Fish
authors: Prince J
authors: PJ
authors: Kannankeril
mesh_terms: D002648:Child; D002675:Child, Preschool; D004247:DNA / Q000032:analysis; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D058327:Norwood Procedures; D007703:Peptidyl-Dipeptidase A / Q000235:genetics*; D011110:Polymorphism, Genetic; D011183:Postoperative Complications / Q000235:genetics*; D012084:Renin-Angiotensin System / Q000235:genetics*; D013613:Tachycardia, Ectopic Junctional / Q000150:complications / Q000235:genetics*
chemical_list: D004247:DNA; C488034:ACE protein, human; D007703:Peptidyl-Dipeptidase A