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Gaseous microemboli (GME) are known to be delivered to the arterial circulation of patients during cardiopulmonary bypass (CPB). An increased number of GME delivered during adult CPB has been associated with brain injury and postoperative cognitive dysfunction. The GME load in children exposed to CPB and its consequences are not well characterized. We sought to establish a baseline of arterial limb emboli counts during the conduct of CPB for our population of patients requiring surgery for congenital heart disease. We used the emboli detection and counting (EDAC) device to measure GME activity in 103 consecutive patients for which an EDAC machine was available. Emboli counts for GME 40 μ were quantified and indexed to CPB time (minutes) and body surface area (BSA) to account for the variation in patient size and CPB times. Patients of all sizes had a similar embolic burden when indexed to bypass time and BSA. Furthermore, patients of all sizes saw a three-fold increase in the 40 μ embolic burden when regular air was noted in the venous line. The use of kinetic venous-assisted drainage did not significantly increase arterial limb GME. Efforts for early identification and mitigation of venous line air are warranted to minimize GME transmission to congenital cardiac surgery patients during CPB.

OBJECTIVES Extracorporeal membrane oxygenation is increasingly utilized to provide cardiopulmonary support to critically ill children. Although life-saving in many instances, extracorporeal membrane oxygenation support is associated with considerable morbidity and mortality. This study evaluates the effect of extracorporeal membrane oxygenation complications and extracorporeal membrane oxygenation hospital characteristics on mortality in neonates and children supported with extracorporeal membrane oxygenation. DESIGN Retrospective analysis of administrative data. SETTING Data from 31 U.S. states included in 2012 Healthcare Cost and Utilization Project Kids' Inpatient Database. PATIENTS Children treated with extracorporeal membrane oxygenation. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS Study subject were identified using International Classification of Diseases, 9th Edition Clinical Modification code 39.65 and classified into six diagnostic categories: 1) cardiac surgery, 2) non-surgical heart disease, 3) congenital diaphragmatic hernia, 4) neonatal respiratory failure, 5) pediatric respiratory failure, and 6) sepsis. Demographics, hospital characteristics, and outcome information were used in a multivariate logistic regression analysis to determine factors associated with mortality. We identified 1,465 children treated with extracorporeal membrane oxygenation. Overall mortality was 40% (591/1,465). Mortality was independently associated with diagnosis (heart disease: odds ratio, 1.7; p = 0.01; congenital diaphragmatic hernia: odds ratio, 5.1; p < 0.001; and sepsis odds ratio: 2.4; p = 0.003 compared with neonatal respiratory failure) time from hospital admission to extracorporeal membrane oxygenation of more than 10 days (odds ratio, 4.5; p < 0.001) and extracorporeal membrane oxygenation complications (renal [odds ratio: 5; p < 0.001] and neurologic [odds ratio, 1.4; p = 0.03] injury). In addition, hospitals with bed size less than 400 had higher mortality (odds ratio, 1.4; p = 0.02). In patients with any extracorporeal membrane oxygenation complication, probability of mortality was lower for extracorporeal membrane oxygenation patients in larger hospitals, 38% (95% CI, 37-39) versus 44% (95% CI, 43-46) with p value of less than 0.001. CONCLUSIONS Extracorporeal membrane oxygenation mortality was significantly associated with patient diagnosis, time to extracorporeal membrane oxygenation initiation, extracorporeal membrane oxygenation complications, and extracorporeal membrane oxygenation hospital bed size. Improved survival in larger hospitals supports centralization of extracorporeal membrane oxygenation services to larger centers.

Summary Background The incidence and risk factors for postoperative vomiting (POV) after pediatric cardiac surgery has not been studied. Aims This study sought to assess the incidence and risk factors for POV in children undergoing surgical repair of an atrial septal defect (ASD). Methods We retrospectively collected perioperative data from 160 patients who underwent surgical repair of an ASD and met early extubation criteria. Demographic and clinical data that could potentially influence the incidence of POV were collected. Univariate analysis was performed using Student t-test or Wilcoxon rank test to identify factors associated with POV. Continuous variables were dichotomized based on the cutoff values derived from the receiver operating characteristic (ROC) curve analysis and the Youden-J index. We used multivariate logistic regression analysis using backward selection to determine the independent predictors using a univariate cutoff of P 0.05 for removal to determine factors independently associated with POV. The accuracy of our multivariate model was assessed by the area under the ROC curve (AUC). Results Overall the incidence of POV was 37.5% in all the children who underwent surgical ASD repair. POV did not significantly differ between patients who received and did not receive antiemetics intraoperatively. Age of ≥4 years and cardiopulmonary bypass (CPB) time ≥51 min were identified as independent risk predictors for POV and the AUC of logistic regression model was 0.650 (95% confidence interval; 0.565–0.735). Conclusions The incidence of POV in children undergoing surgical ASD repair was 37.5%. Age ≥4 years and CPB time ≥51 min were identified as independent predictors.

This article reviews pharmacotherapies currently available to manage sedation, analgesia, and neuromuscular blockade for pediatric cardiac critical patients.The knowledge base of an expert panel of pharmacists, cardiac anesthesiologists, and a cardiac critical care physician involved in the care of pediatric cardiac critical patients was combined with a comprehensive search of the medical literature to generate the data source.The panel examined all studies relevant to management of sedation, analgesia, and neuromuscular blockade in pediatric cardiac critical patients.Each member of the panel was assigned a specific subset of the studies relevant to their particular area of expertise (pharmacokinetics, pharmacodynamics, and clinical care) to review and analyze.The panel members each crafted a comprehensive summary of the literature relevant to their area of expertise. The panel, as a whole, then collaborated to cohesively summarize all the available, relevant literature.In the cardiac ICU, management of the cardiac patient requires an individualized sedative and analgesic strategy that maintains hemodynamic stability. Multiple pharmacological therapies exist to achieve these goals and should be selected based on the patient's underlying physiology, hemodynamic vulnerabilities, desired level of sedation and analgesia, and the projected short- or long-term recovery trajectory.

Inotropic medications are routinely used to increase cardiac output and arterial blood pressure during critical illness. However, few comparative data exist between these medications, particularly independent of their effects on venous capacitance and systemic vascular resistance. We hypothesized that an isolated working heart model that maintained constant left atrial pressure and aortic blood pressure could identify load-independent differences between inotropic medications. In an isolated heart preparation, the aorta and left atrium of Sprague Dawley rats were cannulated and placed in working mode with fixed left atrial and aortic pressure. Hearts were then exposed to common doses of a catecholamine (dopamine, epinephrine, norepinephrine, or dobutamine), milrinone, or triiodothyronine (n = 10 per dose per combination). Cardiac output, contractility (dP/dtmax), diastolic performance (dP/dtmin and tau), stroke work, heart rate, and myocardial oxygen consumption were compared during each 10-min infusion to an immediately preceding baseline. Of the catecholamines, dobutamine increased cardiac output, contractility, and diastolic performance more than clinically equivalent doses of norepinephrine (second most potent), dopamine, or epinephrine (P0.001). The use of triiodothyronine and milrinone was not associated with significant changes in cardiac output, contractility or diastolic function, either alone or added to a baseline catecholamine infusion. Myocardial oxygen consumption was closely related to dP/dtmax (r(2) = 0.72), dP/dtmin (r(2) = 0.70), and stroke work (r(2) = 0.53). In uninjured, isolated working rodent hearts under constant ventricular loading conditions, dobutamine increased contractility and cardiac output more than clinically equivalent doses of norepinephrine, dopamine, and epinephrine; milrinone and triiodothyronine did not have significant effects on contractility.

BACKGROUND Although the traditional surgical approach for left hypoplastic heart syndrome is to perform staged, palliative procedures as a single ventricle lesion, certain anatomical subsets of patients are candidates for a 2-ventricle repair either as a primary or as a staged procedure. The pulmonary blood flow (Q(P))/systemic blood flow (Q(S)) range necessary to optimize systemic oxygen delivery (DO(2)) and systemic venous oxygen saturation has been delineated for patients undergoing conventional interventions as a single ventricle physiology where the left ventricle is assumed to make no contribution to systemic cardiac output. However, in the transitional circulations created during staging to a 2-ventricle repair, the left ventricle does contribute to cardiac output. The Q(P)/Q(S) at which systemic DO(2) and systemic venous oxygen saturation are optimized in the latter circulations has not yet been evaluated. Using computer modeling, we investigated parameters to optimize systemic oxygen delivery. METHODS We designed model circulations after both modified stage I operation and modified bidirectional Glenn shunt with Sano shunt, which are transitional circulations created during staging to a 2-ventricle repair. Mathematical equations were derived to describe DO(2) in both models. Using a computer and an Excel spreadsheet, we used the equations to examine the relationships between DO(2) and arterial oxygen saturation (Sao(2)), venous oxygen saturation (SvO(2)), SaO(2) - SvO(2), Q(P)/Q(S), and the oxygen excess factor SaO(2)/(SaO(2) - SvO(2)). RESULTS In both circulations, SaO(2) or SvO(2) alone does not accurately predict DO(2) or Q(P)/Q(S). The relationships between these variables are further altered by the degree of systemic cardiac output supplied by the left ventricle. To the contrary, DO(2) demonstrates the linear relationship with the oxygen excess factor Sao(2)/(Sao(2) - Svo(2)) irrespective of the degree of systemic cardiac output supplied by the left ventricle. CONCLUSIONS Commonly obtained clinical values such as SaO(2) and SvO(2) alone are not accurate assessments of DO(2) or Q(P)/Q(S). Therefore, these cannot be used in isolation to guide perioperative therapy.

Neonates and infants undergoing cardiac surgery with cardiopulmonary bypass are exposed to multiple blood products from different donors. The volume of the bypass circuit is often as large as the patient’s total blood volume and asanguineous bypass primes are unusual. As a result, blood products are required for the cardiopulmonary bypass prime and are often used to treat the postbypass dilutional coagulopathy. We review clot formation and strength, cardiopulmonary bypass prime considerations, assessment of postbypass coagulopathy, component therapy use, ultrafiltration techniques, and use of antifibrinolytic medications. A combined approach including techniques to minimize the prime volume, utilization of ultrafiltration, administration of antifibrinolytics during surgery, and the proper treatment of the dilutional coagulopathy can limit the transfusion requirements.

Physiological disturbances induced by cardiopulmonary bypass (CPB) and hypothermia during cardiac surgery are particularly pronounced in certain unique patient populations, such as patients with sickle cell disease (SCD) and cold agglutinin disease. Red blood cells containing hemoglobin S (HbS) are at increased risk of sickling under conditions encountered during cardiac surgery, leading to SCD-related complications such as vaso-occlusive events. While a target level of HbS has not been determined for patients with SCD undergoing CPB, a safe practice includes increasing the Hb level to 10 g/dL and reducing the proportion of HbS to approximately 30%. This can be accomplished through simple or exchange transfusion prior to surgery or via the modification of the CPB circuit prime. There is no clear consensus on the formulation or the delivery temperature of the cardioplegia solution necessary to prevent sickling and microvascular occlusion. The presence of cold agglutinins is another entity requiring extra vigilance for the conduct of CPB, where hypothermia can lead to activation of cold agglutinins inducing massive hemagglutination, hemolysis, microvascular thrombosis, and possibly intracoronary thrombosis. Determination of thermal amplitude is important to provide a safe reference range of temperature during surgery. High-volume plasmapheresis may be warranted to reduce cold agglutinin titers. Both warm blood cardioplegia and cold crystalloid cardioplegia above the thermal amplitude have been utilized with success.

Despite remarkable improvements in perioperative care, adverse neurobehavioral outcomes following neonatal and infant cardiac surgery are commonplace and are associated with substantial morbidity. It is becoming increasingly clear that complex congenital heart disease is associated with both abnormalities in neuroanatomic development and a delay in fetal brain maturation. Substantial cerebral ischemic/hypoxic injury has been detected in neonates with complex congenital heart disease both prior to and following corrective cardiac surgery. The brain of the neonate with complex congenital heart disease appears to be uniquely vulnerable to the types of ischemic/hypoxic injury associated with perioperative care. It remains to be determined whether delaying surgical correction to allow for brain maturation will be associated with improvements in neurobehavioral outcomes.

Background— Near-infrared spectroscopy monitoring of cerebral oxygen saturation (rS o 2 ) has become routine in many centers, but no studies have reported the relationship of intraoperative near-infrared spectroscopy to long-term neurodevelopmental outcomes after cardiac surgery. Methods and Results— Of 104 infants undergoing biventricular repair without aortic arch reconstruction, 89 (86%) returned for neurodevelopmental testing at 1 year of age. The primary near-infrared spectroscopy variable was the integrated rS o 2 (area under the curve) for rS o 2 ≤45%; secondary variables were the average and minimum rS o 2 by perfusion phase and at specific time points. Psychomotor and mental development indexes of the Bayley scales, head circumference, neurological examination, and abnormalities on brain magnetic resonance imaging did not differ between subjects according to a threshold level for rS o 2 of 45%. Lower Psychomotor Development Index scores were modestly associated with lower average ( r =0.23, P =0.03) and minimum ( r =0.22, P =0.04) rS o 2 during the 60-minute period after cardiopulmonary bypass but not with other perfusion phases. Hemosiderin foci on brain magnetic resonance imaging were associated with lower average rS o 2 from postinduction to 60 minutes post cardiopulmonary bypass (71±10% versus 78±6%, P =0.01) and with lower average rSO 2 during the rewarming phase (72±12% versus 83±9%, P =.003) and during the 60-minute period following cardiopulmonary bypass (65±11% versus 75±10%, P =0.009). In regression analyses that adjusted for age ≤30 days, Psychomotor Development Index score ( P =0.02) and brain hemosiderin ( P =0.04) remained significantly associated with rS o 2 during the 60-minute period following cardiopulmonary bypass. Conclusions— Perioperative periods of diminished cerebral oxygen delivery, as indicated by rS o 2 , are associated with 1-year Psychomotor Development Index and brain magnetic resonance imaging abnormalities among infants undergoing reparative heart surgery. Clinical Trial Registration— URL: http://clinicaltrials.gov. Unique identifier: NCT00006183.

Summary This study reviewed the hypertensive response of a large population of children to high-dose dexmedetomidine sedation with the aim of determining the incidence and predictors of hypertension. Background: When dexmedetomidine is used to provide sedation for children, fluctuations in blood pressure have been described in case reports. We report the incidence and predictors of hypertension in a large series of children who received dexmedetomidine. Methods/Materials: At our institution, a computerized database holds patient demographics, sedation outcomes, adverse events, and hemodynamic data for all children who receive dexmedetomidine sedation for radiological imaging studies. After Institutional Review Board approval, this database was reviewed. Results: Three thousand five hundred twenty-two (3522) children received dexmedetomidine sedation between May 1, 2007 and December 31, 2008 for magnetic resonance imaging studies. Median age was 3.6 years (interquartile range: 1.8–5.9). A total of 172 patients (4.9%) developed hypertension, with a higher incidence in the younger age group (0–3 years) when compared to the older age groups (3–18 years) (P

Despite a relatively universally applicable knowledge base and skill set, training and experience in pediatric cardiac anesthesia in currently organized basic anesthesia and Adult Cardiothoracic Anesthesia fellowship programs are very limited and not uniformly available. Experience during Pediatric Anesthesia fellowship training is uniformly available but of limited duration and varying intensity. We present a schema, developed by a working group of the Congenital Cardiac Anesthesia Society, for training in pediatric cardiac anesthesia that pediatric cardiac anesthesia educators internationally should consider as a template to be modified as necessary.

The purpose of our study was to examine the reliability of nurse reports of adverse events related to procedural sedation in children. A descriptive, correlational design was used to analyze for inter-rater agreement between prospective adverse event reporting and that identified on independent review of the medical record. All sedation documentation at a pediatric hospital over one calendar year was reviewed, and inter-rater reliability of reporting was analyzed using K statistics. Five thousand forty-five sedation documentation records were reviewed. An adverse event rate of 6.52% was identified: 1.92% of adverse events were serious, and 4.60% were minor. Although overall agreement between nurse reports and independent review was greater than 99%, subanalysis suggested greater agreement for serious events than for minor ones (K values: 0.85 vs 0.49, P.01). The results of our study revealed that minor adverse events associated with procedural sedation were under-reported, despite clear perianesthesia documentation in the medical record that an event had occurred. Improved education for perianesthesia nurses regarding the importance of monitoring both for serious and minor adverse events will help to identify opportunities to improve sedation protocols.

OBJECTIVES Despite aggressive measures to miniaturize the cardiopulmonary bypass (CPB) circuit in neonates and infants, the CPB prime volume is often at least as large as the patients' blood volume. We conducted an observational study to characterize the hemostatic consequences of a CPB prime consisting of either non-fresh or reconstituted whole blood. METHODS Hematocrit, fibrinogen, platelet count, plasminogen, anti-thrombin III (AT-III), and factors (F) II, V, VII, IX, and X of 30 neonates and infants undergoing cardiac surgery with CPB utilizing either a non-fresh or reconstituted whole blood prime were prospectively evaluated at eight time points. Following protamine administration, microvascular bleeding was treated by protocol. RESULTS The hemostatic composition of the CPB prime was the same following the use of either non-fresh or reconstituted whole blood. The CPB prime platelet count (mean +/- SD) was 5.87 +/- 2.84 x 10(3) microl(-1) when compared to a preoperative platelet count of 298 +/- 142 x 10(3) microl(-1) (P < 0.0001). Twenty patients received 17.3 +/- 9.2 ml x kg(-1) (0.86 +/- 0.46 units x kg(-1)) of platelets with significant improvement in platelet count. Nine patients received 16.7 +/- 13.4 ml x kg(-1) (0.84 +/- 0.67 units x kg(-1)) of cryoprecipitate with significant improvements in FVIII and fibrinogen. CONCLUSIONS Non-fresh or reconstituted whole blood as a component of a small volume CPB prime in neonates and infants induces clinically significant dilutional thrombocytopenia in conjunction with less significant reductions in fibrinogen, FII, FV, FVII, FVIII, FIX, FX, plasminogen, and AT-III.

We examined changes in cerebral oxygen saturation during infant heart surgery and its relationship to anatomic diagnosis and early outcome.Regional cerebral oxygen saturation (rSO(2)) was measured by near-infrared spectroscopy in 104 infants undergoing biventricular repair without aortic arch obstruction as part of a randomized trial of hemodilution to a hematocrit of 25% vs 35%.Before cardiopulmonary bypass (CPB), infants with tetralogy of Fallot had higher rSO(2) values compared to those with D-transposition of the great arteries (D-TGA) or ventricular septal defect (P0.001). During CPB cooling, low flow, and at the termination of CPB, D-TGA subjects had the highest rSO(2) values (P0.001). There were no significant associations between intraoperative rSO(2) and early postoperative outcomes after adjustment for diagnosis. In 39 D-TGA subjects withor =5 min of deep hypothermic circulatory arrest (DHCA), there was no correlation between the rSO(2) (91% +/- 6%) or hematocrit (29.2% +/- 5.5%) at the onset of arrest and the rate of decline in rSO(2) during arrest.Intraoperative rSO(2) varies according to anatomic diagnosis but accounts for very little of the variance in early outcome. As measured by frontal near-infrared spectroscopy, higher levels of hematocrit and current perfusion techniques appear to provide an adequate oxygen reservoir prior to relatively short periods of DHCA.

The frequency of anesthesia-related cardiac arrests during pediatric anesthesia has been reported between 1.4 and 4.6 per 10,000 anesthetics. ASA physical statusIII and younger age are risk factors. Patients with congenital cardiac disease may also be at increased risk. Therefore, in this study, we evaluated the frequency of cardiac arrest in patients with congenital heart disease undergoing cardiac surgery at a large pediatric tertiary referral center.Using an established data registry, all cardiac arrests from January 2000 through December 2005 occurring in the cardiac operating rooms were reviewed. A cardiac arrest was defined as any event requiring external or internal chest compressions, with or without direct cardioversion. Events determined to be anesthesia-related were classified as likely related or possibly related.There were 41 cardiac arrests in 40 patients (median age, 2.9 mo; range, 2 days to 23 yr) during 5213 anesthetics over the time period, for an overall frequency of 0.79%; 78% were open procedures requiring cardiopulmonary bypass and 22% closed procedures not requiring cardiopulmonary bypass. Eleven cardiac arrests (26.8%) were classified as either likely (n = 6) or possibly related (n = 5) to anesthesia, (21.1 per 10,000 anesthetics) but with no mortality; 30 were categorized as procedure-related. The incidence of anesthesia-related and procedure-related cardiac arrests was highest in neonates (P0.001). There was no association with year of event or experience of the anesthesiologist.The frequency of anesthesia-related cardiac arrest in patients undergoing cardiac surgery is increased, but is not associated with an increase in mortality. Neonates and infants are at higher risk. Careful preparation and anticipation is important to ensure timely and effective resuscitation.

Introduction: The use of systemic vasodilators is known to decrease the incidence of postoperative cardiac arrest in infants with hypoplastic left heart syndrome (HLHS) following stage 1 palliation (S1P) with Blalock-Taussig shunt. Their impact on measured oxygen delivery (DO2) are uncharacterized. Methods: In 2013, we implemented a treatment algorithm at our institution to standardize care and lower systemic vascular resistance (SVR) in the postoperative period (A). We measured standard hemodynamic variables in consecutive neonates prior to (n=32) and following (n=24) its implementation. 92% of these infants received a Sano modification. We continuously measured oxygen consumption (E-COVX module™, GE Healthcare) and venous oxyhemoglobin saturation (SvO2, PediaSat, Edwards) for Fick-based calculation of DO2 and SVR in a subset (n=21) of patients. Variables were compared pre and post-implementation using linear mixed effects models. Results: Demographic, anatomic and echocardiographic risk factors were similar between groups, although aortic cross-clamp (ACC) time was significantly shorter following protocol implementation (120.9 vs 91.9 mins, P Conclusions: The implementation of a phosphodiesterase-based treatment algorithm decreased SVR, lactate accumulation and the incidence of cardiac arrest in infants with HLHS following S1P with Sano modification.

Introduction: Phosphodiesterase III inhibitors are known to improve cardiac output in patients with heart failure. Whether this is due to a reduction in afterload, a positive inotropic effect, or an interaction of these factors is uncertain. We compared the inotropic and lusitropic effects of milrinone to those of commonly used catecholamines in a working Langendorff model under constant loading conditions. Methods: Sprague Dawley rats (n=35, 350-400 grams) were anesthetized and heparinized for cardiac explantation. The aorta and left atrium were immediately cannulated by a single experimenter. Left atrial pressure (10 mmHg) and aortic pressure (90 mmHg) were fixed. A conductance catheter (Millar) was inserted into the left ventricle. Following baseline measurements, infusions of milrinone, dopamine, dobutamine, epinephrine, or norephinephrine, alone and in commonly-used combinations, were initiated into the left atrium for 10 minute periods. Changes in cardiac output, contractility (dP/dTmax), diastolic performance (-dP/dT and Tau) relative to baseline were compared between groups by linear regression analysis. Results: Cardiac output increased in linear fashion for each of the catecholamines: Dobutamine>>Dopamine>Norepinephrine>Epinephrine (P Conclusions: When afterload is fixed using a Starling resistor, milrinone at commonly used doses does not acutely change systolic or diastolic performance or cardiac output. It is possible that clinical improvements are due to milrinone’s vasodilatory properties.

Introduction: Real time estimates of pulmonary (Qp) and systemic blood flow (Qs) could improve pediatric cardiac care. Current generation inert gas rebreathing (IGR) devices are safe, portable, and easy to use; can estimate Qp and Qs rapidly at the bedside; and can now be adapted for use in intubated patients. We compared IGR and two types of Fick Qp estimates, those using measured (FickM) and assumed (FickA) oxygen consumption (VO2) values, in mechanically ventilated pediatric cardiac patients with no shunt lesion or exclusive right-to-left shunt. Secondarily, we compared measured VO2 with assumed values and values back-calculated from the Fick equation (using measured saturations, hemoglobin and IGR Qp). Methods: In 18 intubated patients in the pediatric catheterization laboratory, the modified ventilator-compatible InnocorTM device was used to measure IGR Qp and breath-by-breath VO2; assumed VO2 was taken from LaFarge tables. Sampled pulmonary arterial and venous saturations were used to calculate FickM and FickA. Bland-Altman agreement and Spearman correlation were assessed for IGR Qp with FickA Qp and FickM Qp. Secondarily, agreement was analyzed between measured VO2, assumed VO2, and VO2 calculated from the Fick equation. Results: Subjects were aged 4-23 years, with a range of cardiac diagnoses. The figure shows Bland-Altman plots for Qp. Compared with FickA, IGR Qp had mean bias -0.9 L/min, 95% limits of agreement (=±1.96 SD) -2.8 to +1.0 L/min, and r=0.75. Compared with FickM, IGR Qp had mean bias -0.2 L/min, 95% limits of agreement -1.3 to +1.0 L/min, and r=0.90. Agreement of assumed and measured VO2 was poor (bias +23%, ±55%); measured VO2 agreed better with Fick calculated VO2 than assumed VO2. Conclusions: IGR Qp estimates agree well with Fick Qp estimates. This agreement improves when VO2 is measured, as assumed VO2 agrees poorly with measured VO2. IGR is an attractive option for bedside monitoring of Qp in intubated children.

Children with congenital heart disease are at increased risk of thrombotic complications (thrombosis and thromboembolism). This study sought to assess the incidence and predictors of thrombotic complications in children with surgical and nonsurgical heart diseases.We performed a retrospective analysis of the Health Care Cost and Use Project Kid's Inpatient Database. Children with surgical and nonsurgical heart diseases were categorized into the following four subgroups: (1) septal defects, (2) single ventricle physiology, (3) right ventricle outflow tract obstruction, and (4) left ventricle outflow tract obstruction. Demographic information, comorbidities, and outcomes, such as mortality, acute kidney injury, sepsis, neurologic complications, thrombotic complications, extracorporeal membrane oxygenation, and ventricular assist device use, were identified. We used propensity-matched analysis and multivariate logistic regression analysis to determine the variables associated with thrombotic complications.After propensity-matched analysis, the incidence of thrombotic complications was 3.90% (947/24,251) in children with surgical and 2.13% (516/24,251) in children with nonsurgical heart disease (p0.001). Multivariate logistic regression analysis revealed that single ventricle physiology or right ventricle outflow tract obstruction, extracorporeal membrane oxygenation, ventricular assist device, acute kidney injury, sepsis, and the presence of a coagulopathy increased the risk of thrombotic complications in children with surgical or nonsurgical heart disease. Age younger than 1 year increased the risk of thrombotic complications in the surgical population, whereas age older than 12 years increased the risk in the nonsurgical population.Children with both surgical and nonsurgical heart disease have increased risk of thrombotic complications. The risk is increased in patients with cyanotic heart disease and is highest in the presence of single ventricle physiology.

The sessions of the symposium held in December 2014 allow us to capitalize on the shared knowledge and experience that arise from both cardiac anesthesia and cardiac intensive care. During this session, topics that crossed traditional boundaries of pediatric cardiac intensive care and pediatric cardiac anesthesia were presented and discussed. This article summarizes the five topics presented at the symposium.

To assess the characteristics associated with high hospital cost for patients receiving extracorporeal membrane oxygenation (ECMO) to identify a cohort of high-resource users.Cost for hospitalization, during which ECMO support was used, was calculated from hospital charges reported in the 2012 Health Care Cost and Use Project Kid's Inpatient Database. Patients were categorized into 6 diagnostic groups: (1) cardiac surgery; (2) nonsurgical heart disease; (3) congenital diaphragmatic hernia; (4) neonatal respiratory failure; (5) pediatric respiratory failure; and (6) sepsis. We categorized cost into 4 groups based on quartiles. We compared ECMO cost with hospital cost for bone marrow, liver, and kidney transplants performed during the same year.Median hospital cost for children supported with ECMO (n = 1465) was $230,425 (IQR: $126,599-$420,960). In a multivariable model, lower cost was associated with neonatal respiratory failure (OR: 0.19) and sepsis (OR 0.53) compared with cardiac surgery (OR: 1.88), whereas greater cost was associated with smaller hospital bed-size99 (OR: 3.49) and 100-399 beds (OR: 3.03) compared with hospitals400 beds, hospital location (Midwest [OR: 1.74] and West [OR 2.18] compared with North-East), and complications such as renal failure (OR: 3.77) and thromboembolic complications (OR 1.60). Hospital cost per survivor was greater for ECMO ($519,450) than bone marrow transplantation ($207,212), liver ($231,755), or kidney transplantation ($82,008) groups.Hospitalization cost for children supported with ECMO is high. Diagnosis, hospital characteristics, and presence of complications are associated with increased cost.

A 57-year-old right-handed man was admitted to the hospital because of the sudden onset of slurred speech and left hemiparesis. Imaging studies revealed an infarct in the area of the right middle cerebral artery. With thrombolysis, recanalization of the vessels was achieved, but the patient's condition continued to worsen.

ObjectiveUsing age-matched controls, this study prospectively evaluated coagulation factor abnormalities and hemodynamic variables in children who had undergone the Fontan operation.MethodsCoagulation factors were assayed in 20 children (mean age 6.4 ± 2.9 years), at a mean 3.7 ± 2.3 years after the Fontan procedure; 24 healthy children (mean age 6.8 ± 2.8 years) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, X; ATIII; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals based on the control patients were determined using 95% confidence limits. Patient demographic, hemodynamic variables, and elapsed time after the Fontan procedure were evaluated as possible predictors of coagulation abnormalities.ResultsConcentrations of protein C; factors II, V, VII, X; plasminogen; and ATIII were significantly lower in Fontan patients compared with age-matched controls (P < .01); factor VIII was significantly elevated in 6 patients (35%), 2 of whom had a thromboembolic event. A higher superior vena cava pressure was predictive of an elevated factor VIII level (P = .003). No other specific hemodynamic variables were predictive of a procoagulant or anticoagulant abnormality.ConclusionProcoagulant and anticoagulant factor levels were significantly lower in patients after the Fontan operation independent of hemodynamic variables peculiar to the Fontan circulation. Increased factor VIII level requires further evaluation as a cause of thrombosis in patients with Fontan physiology and may also indicate a subset of these patients in whom anticoagulation is indicated.

EBSTEIN’S ANOMALY accounts for less than 1% of congenital heart disease. Although the pathologic anatomy may vary, all patients presenting with this anomaly have in common a displaced basal insertion of the septal and posterior leaflet of the tricuspid valve.1,2 The clinical picture of Ebstein’s anomaly may vary from no clinical finding with minor malformations to gross defects that are incompatible with life.3-5 The authors report the anesthetic management for surgical repair of Ebstein’s anomaly.

Objective: Thromboembolic events in patients who have undergone the Fontan operation have been reported to be as high as 20% to 33%. A hypercoagulable state with deficiencies in proteins C and S has been implicated. Using age-matched control subjects, we evaluated whether an altered coagulation state is present earlier in the course of staged single-ventricle repair. Methods: After informed consent had been obtained, coagulation factors were assayed in 36 infants (mean age, 7.7 ± 3.6 months) with single-ventricle cardiac defects immediately before undergoing the bidirectional Glenn procedure; 34 infants (mean age, 8.4 ± 2.6 months) without cardiac disease were assayed as control subjects. Concentration of factors II, V, VII, VIII, IX, and X; antithrombin III; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals on the basis of the control subjects were determined by using 95% confidence limits. Patient demographic and hemodynamic variables were evaluated as possible predictors of coagulation abnormalities. Results: Concentrations of protein C; factors II, V, VII, IX, and X; plasminogen; fibrinogen; and antithrombin III were significantly lower in the pre-Glenn infants compared with in the age-matched control subjects (all P

This study analyzes clinically indicated arterial blood gas values from patients with cyanotic congenital heart disease to determine whether oxyhemoglobin saturation or arterial oxygen tension provides a better measure of oxygenation.

Background Blood transfusion has well-documented adverse effects. As part of a blood conservation initiative at our center, we began routine use of cell saver for all congenital heart surgery performed on cardiopulmonary bypass since 2014. Aims This study aimed to compare transfusion rates prior to, and in the first and second year after this initiative. We hypothesized that cell saver use would decrease transfusion requirements in second year after use of the cell saver compared to the pre cell saver group. Methods Consecutive patients under 18 years undergoing congenital heart surgery on cardiopulmonary bypass were retrospectively analyzed as 3 one-year cohorts defined above. We excluded patients who required mechanical support or reoperation at index admission. Baseline characteristics, and use of blood intraoperatively and postoperatively were compared between groups. Results The 3 groups had similar baseline characteristics. Blood use was significantly lower in year 2 after cell saver initiation as compared to the pre cell saver group both intra- and postoperatively. The median difference in volume of intraoperative blood transfusion was lower by 138 mL/m2 (-266, -10 mL/m2 ) in year 2 when compared to the pre cell saver group. Similarly, the proportion of subjects requiring red blood cell transfusion postoperatively on day of surgery was lower by 10% (-15%, -6%). Conclusion Standardized use of cell saver significantly decreased perioperative blood use in children undergoing cardiac surgery at our center. A risk-adjusted transfusion threshold for children undergoing heart surgery needs to be developed to further decrease exposure to blood products and associated costs.

Background: In response to increasing demand to optimize hospital resource use, we developed a methodology to predict extended length of stay (LOS) in the cardiac intensive care unit (CICU) following surgery for congenital heart disease. Methods: Patients undergoing congenital heart surgery at

i m EXTUBATION IN THE OPERATING ROOM after surgery for correction of congenital heart defects in children, even in selected cohorts, has no proven benefit to patients or families, is associated with disguised resource consumption, and has never been demonstrated conclusively to shorten the duration of intensive care unit (ICU) or hospital stay. Furthermore, although extubation in the ICU after a short (2to 6-hour) period of stabilization is appropriate in selected patients, the routine application of early extubation after pediatric cardiac surgery cannot be justified. A systematic review and meta-analysis of the literature addressing early extubation, defined as extubation in the operating room or 6 hours after pediatric cardiac surgery, has been performed.1 Nine studies were deemed eligible for meta-analsis, with only 1 randomized, controlled trial identified. All 9 tudies were determined to be of poor methodologic quality. our studies that examined an early extubation cohort without n appropriate comparison group were identified. It was conluded that, although the practice of early extubation appears to e safe, sufficient evidence does exist to permit endorsement of his strategy on a routine basis.1 To date, there is only 1 randomized controlled trial comparing extubation in the operating room with delayed extubation. This trial enrolled 100 consecutive pediatric cardiac surgical patients (age range 1 month to 15 years) for extubation in the operating room or elective prolonged mechanical ventilation (approximately 25 hours).2 The study demonstrated no differnce between the groups in reintubation rate, mortality, septic r cardiac complications, abnormal radiographic findings, or he need for chest re-exploration. However, the study was owered inadequately to address these issues. Furthermore, the roup with extubation in the operating room was significantly lder and weighed more than the mandatory ventilation group. he investigators concluded that extubation in the operating oom was associated with shorter ICU and hospital stays. owever, because these time differences almost exactly atched the period of mandatory mechanical ventilation, this nding is expected and precludes establishing any meaningful ink between extubation in the operating room and shorter ength of stay in the ICU or the hospital. A key component of the success of extubation in the operting room is determining the patient population to which his strategy can be applied safely. Given the anatomic and hysiologic heterogeneity of the congenital heart disease opulation, it is no wonder that multiple factors, including ounger age, lower weight, prolonged cardiopulmonary byass time, use of circulatory arrest, pulmonary hypertension, risomy 21, and higher inotrope scores, have been associated ith prolonged ventilation after cardiac surgery.3-5 The difficulty of identifying risk factors reliably is illustrated by the lack of consensus on whether pulmonary hypertension represents a contraindication to extubation in the operating room.6 Pulmonary hypertension, defined simply as a mean hreshold pulmonary artery pressure, does not provide any eaningful insight into the suitability for extubation without nformation about pulmonary vascular resistance and pulonary vascular reactivity.7

Background In response to societal pressure to reduce expenditures and increase quality, we sought to develop a methodology to predict hospital charges related to congenital heart surgery. Methods Patients undergoing congenital heart surgery at Boston Children's Hospital in fiscal years 2007 to 2009 comprised the derivation cohort. Clinical data, including Current Procedural Terminology coding of the primary surgical intervention, were collected prospectively and linked to total hospital charges for an episode of care. Surgical charge categories were developed to group surgical procedure types using empiric data and expert consensus. A multivariable model was built using surgical charge categories and additional patient and procedural characteristics to predict the outcome, total hospital charges. A contemporary cohort for fiscal years 2010 to 2012 was used to validate surgical charge categories and the multivariable model. Results In the derivation cohort, 2,105 cases met inclusion criteria. One hundred three surgical procedure types were categorized into seven surgical charge categories, yielding a grouper variable with an R 2 explanatory value of 47.3%. Explanatory value increased with consideration of patient age, admission status, and preoperative ventilator dependence ( R 2 = 59.4%), as well as weight category, noncardiac abnormality, and genetic syndrome other than trisomy 21 ( R 2 = 61.5%). Additional variability in charge was explained when extracorporeal membrane oxygenation utilization and greater than one operating room visit during the episode of care were added ( R 2 = 74.3%). The contemporary cohort yielded an R 2 explanatory value of 67.7%. Conclusions The combination of clinical data with resource utilization information resulted in a statistically valid predictive model for total hospital charges in congenital heart surgery.

Summary Background Optimizing systemic oxygen delivery (DO2) and hemodynamics in children with hypoplastic left heart syndrome (HLHS) is a clinical challenge. Mathematical modeling of the HLHS circulation has been used to determine the relationship between oxygen kinetic parameters and DO2 and to determine how DO2 might be optimized. The model demonstrates that neither arterial oxygen saturation (SaO2) nor mixed venous oxygen saturation (SvO2) alone accurately predicts DO2. Objectives Oxygen delivery kinetics predicted by previously described mathematical modeling were compared with actual patients' hemodynamic data. We sought to determine which patient derived parameters correlated best with DO2. Methods Patients with HLHS who underwent cardiac catheterization prior to surgery to create a superior cavopulmonary anastomosis from 2007 to 2011 were identified. Hemodynamic data obtained were compared with the data derived from the mathematical model. Correlations between SaO2, SvO2, SaO2-SvO2, SaO2/(SaO2-SvO2), pulmonary-to-systemic blood flow ratio (Qp/Qs), and DO2 were evaluated using both linear and nonlinear analyses, and R2 was calculated. Results/Conclusions Patients' data fit most aspects of the mathematical model. DO2 had the best correlation with SaO2/(SaO2-SvO2; R2 = 0.8755) followed by SaO2-SvO2 (R2 = 0.8063), while SaO2 or SvO2 alone did not demonstrate a significant correlation as predicated by the mathematical model (R2 = 0.09564 and 0.4831, respectively). SaO2/(SaO2-SvO2) would be useful clinically to track changes in DO2 that occur with changes in patient condition or with interventions.

This article reviews the effect of deep hypothermic circulatory arrest (DHCA) in children with congenital heart disease on subsequent cognitive and motor performance.

Children in the cardiac intensive care unit (CICU) with congenital or acquired heart disease are at risk for hematologic complications, both hemorrhage and thrombosis. The overall incidence of hematologic complications in the CICU is unknown, but risk factors and target groups have been identified where the essential physiologic balance between bleeding and clotting has been disrupted. Although the best management of life-threatening bleeding and clotting is prevention, the cardiac intensivist is often faced with managing life-threatening hematologic events involving patients from within the unit or those who present from outside. Part I of this review deals with the propensity of children with congenital and acquired heart disease to complications of both bleeding and clotting, and includes discussions of perioperative bleeding, thromboses in single-ventricle patients, clotting of Blalock-Taussig shunts and thrombotic complications of mechanical valves. Part II deals with the subject of stroke in children with heart disease. Part III reviews monitoring the effectiveness of anticoagulation and thrombolysis in the CICU. Currently available diagnostics modalities, medications and management strategies are reviewed and future directions discussed.

Sepsis continues to result in high morbidity and mortality. General anesthesia is often administered to septic patients, but the impacts of general anesthesia on host defense are not well understood. General anesthesia can be given by volatile and intravenous anesthetics. Our previous in vitro study showed that volatile anesthetic isoflurane directly inhibits leukocyte function-associated antigen-1 (LFA-1) and macrophage-1 antigen (Mac-1), critical adhesion molecules on leukocytes. Thus, the role of isoflurane exposure on in vivo LFA-1 and Mac-1 function was examined using polymicrobial abdominal sepsis model in mice. As a comparison, intravenous anesthetic propofol was given to a group of mice. Wild type, LFA-1, Mac-1, and adhesion molecule-1 knockout mice were used. Following the induction of polymicrobial abdominal sepsis by cecal ligation and puncture, groups of mice were exposed to isoflurane for either 2 or 6 h, or to propofol for 6 h, and their outcomes were examined. Bacterial loads in tissues and blood, neutrophil recruitment to the peritoneal cavity and phagocytosis were studied. Six hours of isoflurane exposure worsened the outcome of abdominal sepsis (P < .0001) with higher bacterial loads in tissues, but 2 h of isoflurane or 6 h of propofol exposure did not. Isoflurane impaired neutrophil recruitment to the abdominal cavity by inhibiting LFA-1 function. Isoflurane also impaired bacterial phagocytosis via complement receptors including Mac-1. In conclusion, prolonged isoflurane exposure worsened the outcome of experimental polymicrobial abdominal sepsis and was associated with impaired neutrophil recruitment and bacterial phagocytosis via reduced LFA-1 and Mac-1 function.

Objective To assess the feasibility and accuracy of inert gas rebreathing (IGR) pulmonary blood flow (Qp) estimation in mechanically ventilated pediatric patients, potentially providing real-time noninvasive estimates of cardiac output. Study design In mechanically ventilated patients in the pediatric catheterization laboratory, we compared IGR Qp with Qp estimates based upon the Fick equation using measured oxygen consumption (VO 2 ) (Fick True ); for context, we compared Fick True with a standard clinical short-cut, replacing measured with assumed VO 2 in the Fick equation (Fick LaFarge , Fick Lundell , Fick Seckeler ). IGR Qp and breath-by-breath VO 2 were measured using the Innocor device. Sampled pulmonary arterial and venous saturations and hemoglobin concentration were used for Fick calculations. Qp estimates were compared using Bland-Altman agreement and Spearman correlation. Results The final analysis included 18 patients aged 4-23 years with weight >15 kg. Compared with the reference Fick True , IGR Qp estimates correlated best and had the least systematic bias and narrowest 95% limits of agreement (results presented as mean bias ±95% limits of agreement): IGR −0.2 ± 1.1 L/min, r = 0.90; Fick LaFarge +0.7 ± 2.2 L/min, r = 0.80; Fick Lundell +1.6 ± 2.9 L/min, r = 0.83; Fick Seckeler +0.8 ± 2.5 L/min, r = 0.83. Conclusions IGR estimation of Qp is feasible in mechanically ventilated patients weighing >15 kg, and agreement with Fick True Qp estimates is better for IGR than for other Fick Qp estimates commonly used in pediatric catheterization. IGR is an attractive option for bedside monitoring of Qp in mechanically ventilated children.

beta-Adrenergic antagonism decreases the size of myocardial infarction and provides myocardial protection during hypothermic arrest for cardiac surgery. However, concern regarding the negative inotropic and chronotropic effects of beta-adrenergic antagonism persisting after cardiopulmonary bypass (CPB) has impeded the use of esmolol for this purpose during cardiac surgery. This is a randomized, double-blind prospective study of the effects of esmolol infused during CPB and the effects of hypothermic CPB on esmolol. Patients scheduled for CPB were randomized to receive intravenous esmolol (300.micrograms.kg-1.min-1 during CPB after a bolus of 2 mg/kg prior to CPB) or placebo. Infusion was stopped at 10 min after release of aortic cross-clamp. Hemodynamics were measured, as well as serum esmolol, catecholamines, lactate, and potassium. Postoperative variables measured included electrocardiographic changes, creatine kinase (CK)-MB fractions, post-CPB dysrhythmias and drugs, hospitalization time and cost, and mortality. Esmolol was administered to 16 patients and placebo to 14. Esmolol levels reached a high of 10.5 +/- 0.9 micrograms/mL during CPB, but decreased to 0.1 +/- 0.02 microgram/mL within 30 min after stopping infusion. Cardiac indices (cardiac index, stroke volume index, left cardiac work index, left ventricular stroke work index, right cardiac work index, and right ventricular stroke work index) were higher in the esmolol group for the first hour post-CPB (P < 0.05). Systemic arterial lactate and coronary sinus lactate were lower in the esmolol group after CPB (P < 0.05), but myocardial lactate extraction was not significantly different between groups. After CPB, hemoglobin was lower in the esmolol group (P < 0.05) due to longer CPB and aortic cross-clamp time (P < 0.05), but oxygen consumption was less than in the control group (P < 0.05). Post-CPB serum potassium was higher in the esmolol group (P < 0.05). Results are confounded by more chronically beta-adrenergically blocked patients randomized to the esmolol group (P < 0.05). Esmolol infused during CPB in this series of patients was associated with high concentrations during CPB but did not result in any adverse clinical effects after CPB.

The population of adults with congenital heart disease (ACHD) now exceeds that of children with congenital heart disease. Within this relatively young population of adults in their third decade of life exist a population of patients with heart failure (HF) who, in functional status, closely resemble patients in their fifth and sixth decades of life with HF caused by coronary artery disease and myocardial ischemia. The ACHD patients currently at greatest risk for HF are those with single-ventricle physiology, those with a 2-ventricle circulation with the right ventricle as the systemic ventricle, and those with repaired tetralogy of Fallot and pulmonary valve insufficiency. This article will review the demographics, diagnosis, functional capacity, and treatment of HF in the ACHD population.

Mechanical circulatory support is an important modality in the management of paediatric patients with circulatory failure. The field of ventricular assist device (VAD) is particularly evolving. We review the currently available and developing VADs in the paediatric population, and the anaesthetic management of patients presenting for VAD implantation and with VADs for non-cardiac surgery.