417 Sudden death risk in patients with tetralogy of Fallot and 22q11.2 deletion syndrome

Abstract

achieve sufficiently high heparin blood concentration after the initial heparin bolus in 52% of cases and required an additional heparin bolus prior to bypass initiation. Most patients (361, 58%) received additional heparin during the surgery, despite normal activated clotting time ( 400s). Patients in the postHMS era required significantly lower amount of allogeneic red blood cell (87ml/kg vs. 118 ml/kg, P 0.001) and platelets (8ml/kg vs. 14ml/kg, P 0.001). Use of recombinant factor VIIa in post-operative period was less likely in the post-HMS era (1.1% vs. 3.5%, P 0.03). Intubation time (9 vs. 27 hours, P 0.001), intensive care unit stay (2 vs. 3 days, P 0.009) and hospital stay (6.3 vs. 8.0 days, P 0.02) were all shorter in the post-HMS period. CONCLUSION: Failure of the HMS system to achieve sufficient blood heparin concentration after the initial dose shows that further refinements in the pediatric protocol are necessary. Even considering this, the use of the HMS system for all pediatric cardiac surgeries was associated with system-wide improvement in post-surgical outcomes. Although the effect cannot be entirely ascribed to the use of the HMS because of additional concurrent changes in surgical and post-operative protocols, the reduction of allogeneic blood transfusions and bleeding-related complications suggest that the HMS system was a contributor to the improved surgical outcomes observed between the 2 periods.