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BACKGROUND: Red blood cell (RBC) transfusions are used frequently in pediatric patients admitted to the intensive care unit (ICU) after cardiac surgery. To improve data-driven transfusion decision-making in the ICU, we conducted a retrospective analysis to assess the effect of RBC transfusion on cerebral and somatic regional oxygenation (rSO2). METHODS: We evaluated post- versus pre-RBC transfusion cerebral rSO2 and somatic rSO2 in all consecutive pediatric patients (age >28 days to <18 years) who underwent biventricular cardiac surgery at a single center between July 2016 and April 2020. RESULTS: The final data set included 263 RBC postoperative transfusion events in 75 patients who underwent 83 surgeries. The median pretransfusion hemoglobin was 10.6 g/dL (25th-75th percentile, 9.3-11.6). The median pretransfusion cerebral and somatic rSO2 were 63% (54-71) and 69% (55-80), which increased by a median of 3 percentage points (-2 to 6) and 2 percentage points (-3 to 6), respectively, after transfusion. After adjusting for pretransfusion hemoglobin, change in hemoglobin posttransfusion versus pretransfusion, and potential confounders (age, sex, and STAT surgical mortality risk score), the posttransfusion versus pretransfusion change in cerebral or somatic rSO2 was not statistically significant. Pretransfusion cerebral rSO2 (crSO2) was ≤50%, a previously described threshold for increased risk for unfavorable neurological outcome, for 22 of 138 (16%) transfusion events with complete pre- and post-crSO2 data. Sixteen of these 22 (73%) transfusions resulted in a posttransfusion crSO2 >50%. When restricting analysis to the first (index) transfusion after arrival to the ICU from the operating room (administered at a median of 1.15 postoperative days [25th-75th percentile, 0.84-1.93]), between-patient pretransfusion hemoglobin was not associated with pretransfusion crSO2 but within-patient posttransfusion versus pretransfusion hemoglobin difference was significantly associated with posttransfusion versus pretransfusion crSO2 difference (mean posttransfusion versus pretransfusion crSO2 difference, 2.54; 95% confidence interval, 0.50-4.48). CONCLUSIONS: In this study, neither cerebral nor somatic rSO2 increased significantly post- versus pre-RBC transfusion in pediatric cardiac surgery patients admitted to the ICU after biventricular repairs. However, almost three-quarters of transfusions administered when pretransfusion crSO2 was below the critical threshold of 50% resulted in a posttransfusion crSO2 >50%. In addition, the significant within-patient change in crSO2 in relation to the change in posttransfusion versus pretransfusion hemoglobin in the immediate postoperative period suggests that a personalized approach to transfusion following within-patient trends of crSO2 rather than absolute between-patient values may be an important focus for future research.

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Pediatric pulmonary hypertension is a disease that has many etiologies and can present anytime during childhood. Its newly revised hemodynamic definition follows that of adult pulmonary hypertension: a mean pulmonary artery pressure >20 mmHg. However, the pediatric definition stipulates that the elevated pressure must be present after the age of three months. The definition encompasses many different etiologies, and diagnosis often involves a combination of noninvasive and invasive testing. Treatment often is extrapolated from adult studies or based on expert opinion. Moreover, although general anesthesia may be required for pediatric patients with pulmonary hypertension, it poses certain risks. A thoughtful, multidisciplinary approach is needed to deliver excellent perioperative care.

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BACKGROUND: Marfan syndrome and Loeys-Dietz syndrome are connective tissue disorders associated with cardiac and vascular disease. Patients often require surgical repair, but limited data exist to describe their perioperative management. AIMS: Our goals were to review the perioperative features of patients with Marfan and Loeys-Dietz syndrome that may affect anesthesia care and to describe the differences in preoperative clinical characteristics and intra-operative anesthetic management. METHODS: We conducted a retrospective cohort study of patients with Marfan and Loeys-Dietz syndrome who underwent cardiac surgery at a single institution. We collected demographic and perioperative data from the electronic medical record and performed descriptive statistics to characterize the patient populations and describe their anesthetic management. RESULTS: In 71 patients (40 Marfan, 31 Loeys-Dietz), we found significant differences between the Marfan and Loeys-Dietz patients in airway difficulty, preoperative weight, blood utilization, valvular disease, and age at first cardiac surgery. Patients with Loeys-Dietz syndrome had higher preoperative rates of severe noncardiac comorbidities, including gastroesophageal reflux and asthma that required chronic medical therapy. CONCLUSIONS: Despite undergoing similar surgical procedures, patients with Marfan and Loeys-Dietz syndrome have different intrinsic patient characteristics and comorbidities that may affect their perioperative care. This retrospective cohort study identified some factors, but additional collection and reporting of patient data based on multicenter experience are essential for the ongoing optimization of perioperative care in these patient populations.

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BACKGROUND: Prior research on red blood cell (RBC) storage duration and clinical outcomes in paediatric cardiac surgery has shown conflicting results. The purpose of this study was to evaluate whether blood stored for a longer duration is harmful in these patients. METHODS: We performed a retrospective cohort study of paediatric patients undergoing cardiac surgery at our institution between January 2011 and June 2015. Patients were stratified based on whether they were transfused RBCs stored for ≤15 days (fresher blood) or >15 days (older blood). The primary outcome was composite morbidity, with prolonged length of stay (LOS) as a secondary outcome. Subgroup analyses were performed after stratification by RBC transfusion volume (≤2 vs. >2 RBC units). Multivariable logistic regression models were used to assess the impact of RBC storage duration on composite morbidity and prolonged LOS. RESULTS: Of 461 patients, 122 (26·5%) received fresher blood and 339 (73·5%) received older blood. The overall rate of composite morbidity was 18·0% (n = 22) for patients receiving fresher blood and 13·6% (n = 46) for patients receiving older blood (P = 0·24). In the risk-adjusted model, patients receiving older blood did not exhibit an increased risk of composite morbidity (OR: 0·74, 95% CI: 0·37-1·47, P = 0·40) or prolonged LOS (OR: 0·72, 95% CI: 0·38-1·35, P = 0·30) compared to patients receiving fresher blood. Similar results were seen after stratification by RBC transfusion volume. CONCLUSIONS: Transfusing RBCs stored for a longer duration was not associated with an increased risk of morbidity or prolonged LOS in paediatric cardiac surgery patients.

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BACKGROUND: We sought to examine potential associations between pediatric postcardiac surgical hematocrit values and postoperative complications or mortality. METHODS: A retrospective, cross-sectional study from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and Congenital Cardiac Anesthesia Society Database Module (2014-2019) was completed. Multivariable logistic regression models, adjusting for covariates in the STS-CHSD mortality risk model, were used to assess the relationship between postoperative hematocrit and the primary outcomes of operative mortality or any major complication. Hematocrit was assessed as a continuous variable using linear splines to account for nonlinear relationships with outcomes. Operations after which the oxygen saturation is typically observed to be <92% were classified as cyanotic and ≥92% as acyanotic. RESULTS: In total, 27,462 index operations were included, with 4909 (17.9%) being cyanotic and 22,553 (82.1%) acyanotic. For cyanotic patients, each 5% incremental increase in hematocrit over 42% was associated with a 1.31-fold (95% confidence interval [CI], 1.10-1.55; P = .003) increase in the odds of operative mortality and a 1.22-fold (95% CI, 1.10-1.36; P < .001) increase in the odds of a major complication. For acyanotic patients, each 5% incremental increase in hematocrit >38% was associated with a 1.45-fold (95% CI, 1.28-1.65; P < .001) increase in the odds of operative mortality and a 1.21-fold (95% CI, 1.14-1.29; P < .001) increase in the odds of a major complication. CONCLUSIONS: High hematocrit on arrival to the intensive care unit (ICU) is associated with increased operative mortality and major complications in pediatric patients following cardiac surgery.

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BACKGROUND: Patients requiring extracorporeal membrane oxygenation (ECMO) support are critically ill and have substantial transfusion requirements, which convey both risks and benefits. A retrospective analysis was conducted to assess the association between blood component administration and adverse outcomes in adult, pediatric, and neonatal ECMO patients. METHODS: We evaluated 217 ECMO patients at a single center hospitalized between January 2009 and June 2016. Three cohorts (88 adult, 57 pediatric, and 72 neonatal patients) were included for assessment of patient characteristics, blood utilization, and clinical outcomes. Univariable and multivariable analyses were used to assess the association between transfusions and clinical outcomes (primary outcome: mortality and secondary outcomes: morbid events). The analysis included the main exposure of interest (total number of blood component units transfused) and potential confounding variables (age group cohort, case mix index, sex, ECMO mode and duration, and primary ECMO indication). RESULTS: After adjustment for confounders, with each additional blood component unit transfused, there was an estimated increase in odds for mortality by 1% (odds ratio [OR] = 1.01; 95% confidence interval [CI], 1.00-1.02; P = .013) and an increase in odds for thrombotic events by 1% (OR = 1.01; 95% CI, 1.00-1.02; P = .007). Mortality was higher in the adult (57 of 88; 64.8%) and pediatric (37 of 57; 64.9%) than in the neonatal cohort (19 of 72; 26.4%) (P < .0001). Median total blood components transfused per day followed a similar pattern for the adult (2.3 units; interquartile range [IQR] = 0.8-7.0), pediatric (2.9 units; IQR = 1.1-10), and neonatal (1.0 units; IQR = 0.7-1.6) cohorts (P < .0001). Over the entire hospitalization, the total median blood components transfused was highest in the neonatal (41 units; IQR = 24-94) and pediatric (41 units; IQR = 17-113) compared to the adult (30 units; IQR = 9-58) cohort (P = .007). There was no significant interaction between total units transfused over the hospital stay and age cohort for mortality (P = .35). CONCLUSIONS: Given the association between transfusion and adverse outcomes, effective blood management strategies may be beneficial in ECMO patients.

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Anticoagulation is an essential component for patients undergoing cardiopulmonary bypass or extracorporeal membrane oxygenation and for those with ventricular assist devices. However, thrombosis and bleeding are common complications. Heparin continues to be the agent of choice for most patients, likely owing to practitioners' comfort and experience and the ease with which the drug's effects can be reversed. However, especially in pediatric cardiac surgery, there is increasing interest in using bivalirudin as the primary anticoagulant. This drug circumvents certain problems with heparin administration, such as heparin resistance and heparin-induced thrombocytopenia, but it comes with additional challenges. In this manuscript, the authors review the literature on the emerging role of bivalirudin in pediatric cardiac surgery, including its use with cardiopulmonary bypass surgery, extracorporeal membrane oxygenation, ventricular assist devices, and interventional cardiology. Moreover, they provide an overview of bivalirudin's pharmacodynamics and monitoring methods.

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Perfusion strategies for cardiopulmonary bypass have direct consequences on pediatric cardiac surgery outcomes. However, inconsistent study results and a lack of uniform evidence-based guidelines for pediatric cardiopulmonary bypass management have led to considerable variability in perfusion practices among, and even within, institutions. Important aspects of cardiopulmonary bypass that can be optimized to improve clinical outcomes of pediatric patients undergoing cardiac surgery include extracorporeal circuit components, priming solutions, and additives. This review summarizes the current literature on circuit components and priming solution composition with an emphasis on crystalloid, colloid, and blood-based primes, as well as mannitol, bicarbonate, and calcium.

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Cardiopulmonary bypass perfusion management significantly affects postoperative outcomes. In recent years, the principles of goal-directed therapy have been applied to the field of cardiothoracic surgery to improve patient outcomes. Goal-directed therapy involves continuous peri- and postoperative monitoring of vital clinical parameters to tailor perfusion to each patient's specific needs. Closely measured parameters include fibrinogen, platelet count, lactate, venous oxygen saturation, central venous oxygen saturation, mean arterial pressure, perfusion flow rate, and perfusion pulsatility. These parameters have been shown to influence postoperative fresh frozen plasma transfusion rate, coagulation state, end-organ perfusion, and mortality. In this review, we discuss the recent paradigm shift in pediatric perfusion management toward goal-directed perfusion.

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BACKGROUND: Clinical studies measuring cerebral blood flow in infants during deep hypothermia have demonstrated diminished cerebrovascular pressure autoregulation. The coexistence of hypotension in these cohorts confounds the conclusion that deep hypothermia impairs cerebrovascular pressure autoregulation. AIM: We sought to compare the lower limit of autoregulation and the static rate of autoregulation between normothermic and hypothermic piglets. METHODS: Twenty anesthetized neonatal piglets (5-7 days old; 10 normothermic and 10 hypothermic to 20°C) had continuous measurements of cortical red cell flux using laser Doppler flowmetry, while hemorrhagic hypotension was induced without cardiopulmonary bypass. Lower limit of autoregulation was determined for each subject using piecewise regression and SRoR was determined above and below each lower limit of autoregulation as (%change cerebrovascular resistance/%change cerebral perfusion pressure). RESULTS: The estimated difference in lower limit of autoregulation was 1.4 mm Hg (lower in the hypothermic piglets; 95% C.I. -10 to 14 mm Hg; P=0.6). The median lower limit of autoregulation in the normothermic group was 39 mm Hg [IQR 38-51] vs 35 mm Hg [31-50] in the hypothermic group. Intact steady-state pressure autoregulation was defined as static rate of autoregulation >0.5 and was demonstrated in all normothermic subjects (static rate of autoregulation=0.72 [0.65-0.87]) and in 9/10 of the hypothermic subjects (static rate of autoregulation=0.65 [0.52-0.87]). This difference in static rate of autoregulation of 0.06 (95% C.I. -0.3 to 0.1) was not significant (P=0.4). CONCLUSION: Intact steady-state cerebrovascular pressure autoregulation is demonstrated in a swine model of profound hypothermia. Lower limit of autoregulation and static rate of autoregulation were similar in hypothermic and normothermic subjects.

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