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 (TOF), balanced atrioventricular septal defect (AVSD), and ventricular septal defect (VSD) at a single center. The incidence of postoperative JET was associated with previously identified clinical risk factors and ACE I/D genotype.
Results
Of 174 children who underwent the above surgeries, 21% developed JET. Post-operative JET developed in 31% of children with the D/D genotype and only 16% of those with I/I or I/D genotype (p=0.02). Clinical predictors of JET were selected a priori and included age, inotrope score, cardiopulmonary bypass and cross clamp times. Multivariable logistic regression identified a significant correlation between the D/D genotype and postoperative JET independent of these predictors (OR=2.4; 95% CI, 1.04-5.34; p=0.04). A gene-dose effect was apparent in the homogenous subset of AVSD subjects (58% JET in D/D subjects, 12% JET in I/D, and 0% JET in I/I; p<0.01).
Conclusion
The common ACE deletion polymorphism is associated with a greater than two-fold increase in the odds of developing JET in children undergoing surgical repair of AVSD, TOF, VSD, or Norwood or arterial switch procedures. These findings may support the potential role of the renin-angiotensin-aldosterone system in the etiology of JET.