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Objective: This study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy., Design: Retrospective observational cohort study., Setting: Single quaternary academic referral center., Participants: The authors reviewed the records of all children with WS at the authors' institution who underwent general anesthesia for cardiac catheterization, diagnostic imaging, or any type of surgery between November 2008 and August 2019. The authors identified 142 patients with WS, 48 of whom underwent 118 general anesthesia administrations. A historic group (HG) was compared with the intervention group (IG)., Interventions: Change in perioperative management (three-stage risk stratification: preoperative intravenous hydration, intravenous anesthesia induction, and early use of vasoactives)., Measurements and Main Results: The authors determined event rates within 60 minutes of anesthesia induction. Standardized mean difference (SMD) was calculated (SMD >0.2 suggests clinically meaningful difference). Sixty-seven general anesthesia encounters were recorded in the HG (mean age, 4.8 years; mean weight, 16.3 kg) and 51 in the IG (mean age, 6.0 years; mean weight, 18.2 kg). The change in systolic blood pressure was -17.5% (-30.0, -5.0) in the HG versus -9% (-18.0, 5.0) in the IG (p = 0.015; SMD = 0.419), and the incidence of ACEs was 6% in the HG and 2% in the IG (p = 0.542; SMD = 0.207)., Conclusions: Preoperative risk stratification, preoperative intravenous hydration, intravenous induction, and early use of continuous vasoactives resulted in greater hemodynamic stability, with a 2% incidence of ACEs., Competing Interests: Conflict of Interest The authors report no conflict of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

This article discusses the indications, perioperative management, postoperative complications, and patient outcome of pediatric heart transplantation and pediatric lung transplantation. Special emphasis is placed on the anesthetic considerations relevant for children who are undergoing or have received a solid thoracic organ transplant.

Objectives: To examine whether coagulation tests, sampled before and during cardiopulmonary bypass (CPB), are related to blood loss and blood product transfusion requirements, and to determine what test value(s) provide the best sensitivity and specificity for prediction of excessive hemorrhage., Design: Prospective., Setting: University-affiliated, pediatric medical center., Participants: Four hundred ninety-four children., Interventions: Coagulation tests., Measurements and Main Results: Demographic, coagulation test, blood loss, and transfusion data were noted in consecutive children undergoing cardiac surgery. Laboratory tests included hematocrit (Hct), prothrombin time, partial thromboplastin time (PTT), platelet count, fibrinogen concentration, and thromboelastography. Stepwise linear regression analysis indicated that platelet count during CPB was the variable most significantly associated with intraoperative blood loss (in milliliters per kilogram) and 12-hour chest tube output (in milliliters per kilogram). Other independent variables associated with blood loss were thromboelastography maximum amplitude (MA) during CPB, preoperative PTT, preoperative Hct, and preoperative thromboelastography angle and shear modulus values. Thromboelastography MA during CPB was the only variable associated with total products transfused (in milliliters per kilogram). Of all tests studied, platelet count during CPB (< or = 108,000/microL) provided the maximum sensitivity (83%) and specificity (58%) for prediction of excessive blood loss (receiver operating characteristic analysis). Blood loss was inversely related to patient age; neonates received the most donor units (median, 8 units; range, 6 to 10 units)., Conclusions: During cardiac surgery, coagulation tests (including thromboelastography) drawn pre-CPB and during CPB are useful to identify children at risk for excessive bleeding. Platelet count during CPB was the variable most significantly associated with blood loss.

Objective: To compare coagulation test results, blood loss, and blood product transfusions between patients receiving prophylactic epsilon-aminocaproic acid (EACA) and a control group matched for age, resternotomy, and surgery in children undergoing cardiac surgery., Design: Nested case-control study., Setting: University-affiliated, pediatric medical center., Participants: Same study period; 70 patients in EACA group and 70 patients in control group., Interventions: Prophylactic EACA administered intravenously (load, 150 mg/kg, infusion; 30 mg/kg/h) to 70 patients at increased risk for bleeding (reoperation or Ross procedure)., Measurements and Main Results: Coagulation test values were measured before, during, and after cardiopulmonary bypass (CPB). Intraoperative blood loss, postoperative chest tube output, and allogenic blood product transfusions were recorded. Comparison of demographic and surgical data indicated close matching of the EACA and control groups. The EACA group ([median, 25th to 75th quartile] 15.6 mL/kg; 9.2 to 26.3 mL/kg) had less intraoperative blood loss than the control group (22.2 mL/kg; 14.3 to 36.3 mL/kg; p = 0.02). Postoperative chest tube output at 6 hours (p = 0.08), 12 hours (p = 0.07), and 24 hours (p = 0.08) was not significantly different between groups. Fewer EACA group patients required reexploration for bleeding (p < 0.05). There was no difference between groups in blood products transfused (in milliliters per kilogram or allogenic exposure per patient). Thromboelastography values (maximum amplitude [MA], whole blood clot lysis index at 30 minutes after MA) during CPB were better preserved in the EACA group., Conclusion: EACA reduced intraoperative blood loss but did not significantly decrease blood product transfusions. Lack of efficacy may be related to relative underdosing and should be further studied.

Objective: Thromboelastographic evaluation of the influence of fibrinolysis on blood loss and blood product transfusions in children during cardiac surgery., Design: Prospective study., Setting: University-affiliated, pediatric medical center., Participants: Two hundred seventy-eight consecutive children undergoing cardiac surgery., Interventions: Blood sampling for coagulation tests, including native and protamine-modified thromboelastography., Measurements and Main Results: Blood coagulation tests were measured before, during, and after cardiopulmonary bypass (CPB). Demographic data, perioperative blood loss, and blood product transfusions were prospectively recorded. Fibrinolysis was defined as thromboelastography of A30/MA less than 0.85 (MA, maximum amplitude; A30, amplitude 30 minutes after MA) and was noted in 3% of children pre-CPB, 16% during CPB, and 3% post-CPB. Fibrinolysis before CPB was associated with poor cardiac output. Fibrinolysis during CPB occurred in young children (aged 350 +/- 836 days) undergoing complex surgery with prolonged CPB (119 +/- 48.8 minutes) and deep hypothermia (25.6 degrees C +/- 4.7 degrees C). These patients received blood products after CPB and were not fibrinolytic after transfusion. They incurred similar blood loss (in mL/kg) and received similar volumes of blood products (mL/kg) as age-matched and surgery-matched patients without fibrinolysis., Conclusion: A group of children at risk for fibrinolysis during CPB was identified. However, fibrinolysis during CPB did not influence blood loss or the total volume of blood products transfused.

Objective: To determine if vein localization with an audio Doppler increases successful central venous cannulation and decreases complications in infants and children when performed by inexperienced operators, compared with vein localization by anatomic landmarks (ALs)., Design: A prospective cohort of infants and children undergoing central venous cannulation for cardiac surgery., Setting: A university-affiliated children's hospital with a pediatric anesthesia fellowship program., Participants: All infants and children undergoing cardiac surgery between July 1, 1996, and January 1, 1997., Interventions: Subjects had central venous catheters (CVCs) placed by an anesthesia fellow by either ALs or audio-Doppler localization of the veins., Measurements and Main Results: Eighty-four children were studied. Internal jugular vein (IJV) cannulation was attempted in 71 (85%) children and femoral vein cannulation in 13 (15%) children. Time to catheter insertion, number of needle passes, and artery puncture were noted. Sixty-one of 63 (97%) children had successful central venous cannulation by an anesthesia fellow using audio-Doppler vein localization. This was significantly greater than the 13 of 21 (62%) successful cannulations among children who had veins localized by ALs. Time to insertion did not differ by method of vein localization; however, the number of needle passes was significantly greater in the AL group. Artery puncture did not differ significantly by method of vein localization., Conclusion: Vein localization by audio Doppler significantly increases the rate of successful central venous cannulation and decreases the number of needle passes in pediatric patients when used by inexperienced operators.

Objective: To determine the effects of red cell separation and ultrafiltration on heparin concentration., Design: Prospective study., Setting: University-affiliated, pediatric medical center., Participants: Thirty-one children undergoing cardiac surgery., Interventions: Blood sampled for heparin concentration and coagulation tests., Measurements and Main Results: Thirteen infants under-went modified veno-venous ultrafiltration (UF) after cardiopulmonary bypass (CPB). In addition, residual blood in the CPB circuit was hemoconcentrated by UF and reinfused (UF group). Heparin concentration increased from 2.0 +/- 0.6 to 2.5 +/- 0.8 U/mL, following modified UF; while activated coagulation time (ACT) decreased from 701 +/- 177 to 627 +/- 107 seconds. Heparin concentration of CPB circuit residual increased from 1.9 +/- 0.7 to 3.1 +/- 1.0 U/mL. In 18 children (older than 1 year old), the residual blood in the CPB circuit was hemoconcentrated by cell separation (CS) and reinfused (CS group). Heparin concentration of CPB circuit residual decreased from 2.6 +/- 0.6 to 0.3 +/- 0.2 U/mL. After reinfusion, patient heparin concentration remained unchanged at < 0.05 U/mL. Thrombin time increased from 28 +/- 6 to 48 +/- 29 seconds and did not correlate with H., Conclusions: The plasma concentration of heparin increased after veno-venous modified UF of the patient. Heparin concentration also increased after UF of residual CPB circuit blood. In contrast, circuit blood hemoconcentrated by CS contained minimal heparin, and, when infused, did not increase patient's heparin concentration. ACT and thrombin time did not correlate with heparin concentration.