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The Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body's immune response. Altered Starling forces at the capillary level, capillary leak, and lymphatic obstruction contribute to lymphatic dysfunction in patients with Fontan physiology. These lymphatic complications include edema, pleural effusions, plastic bronchitis (PB), and protein-losing enteropathy (PLE). Over the past decade, there have been innovations in lymphatic imaging. These new imaging techniques include noncontrast magnetic resonance (MR) lymphangiography, intranodal lymphangiography (IL), dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), and liver lymphangiography. These imaging techniques help in delineating anatomy and guiding the appropriate therapeutic approach. Lymphatic interventions then may be performed to decompress the lymphatic system or to identify and occlude abnormal lymphatic vessels and drainage pathways. The anesthesiologist should have an understanding of the effects of lymphatic disorders on the Fontan circulation and apply appropriate management techniques for the associated interventions. The Fontan surgery was developed as a palliative intervention for CHD patients with single-ventricle physiology who are not candidates for a biventricular repair. The surgery creates a series systemic and pulmonary circulation with the energy necessary to provide gradient-driven pulmonary blood flow generated by the ventricle.1 In the past decades, improvements in the surgery and medical management of these patients have increased survival, with 30-year survival rates close to 85%.2 Despite these improvements, this population remains at risk for complications and end-organ dysfunction due to Fontan failure, which is characterized by elevated systemic venous pressures and low cardiac output. These complications include arrhythmias, cardiac dysfunction, ascites, liver fibrosis/cirrhosis, renal dysfunction, pulmonary failure, and lymphatic complications such as edema, pleural effusions, PB, and PLE. Complications ultimately contribute to increased risk for hospitalization, death, and need for heart transplantation.3,4 For this reason, there has been increasing interest in the role of abnormal lymphatic circulation in the genesis of Fontan failure. The authors characterize the lymphatic pathophysiology associated with Fontan physiology and review the imaging and interventional strategies used to treat these patients.

Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms.

With advances in surgical and catheter-based interventions and technologies in patients with congenital heart disease (CHD), the practice of pediatric cardiac anesthesiology has evolved in parallel with pediatric cardiac surgery and pediatric cardiology as a distinct subspecialty over the past 80 years. To date, there has not been an analysis of the distribution of pediatric cardiac anesthesiologists relative to cardiac and noncardiac procedures in the pediatric population. The primary aim is to report the results of a survey and its subsequent analysis to describe the distribution of pediatric cardiac anesthesiologists relative to pediatric cardiac procedures that include surgical interventions, cardiac catheterization procedures, imaging studies (echocardiography, magnetic resonance, computed tomography, positron emission tomography), and noncardiac procedures.A survey developed in Research Electronic Data Capture (REDcap) was sent to the identifiable division chiefs/cardiac directors of 113 pediatric cardiac anesthesia programs in the United States. Data regarding cardiac surgical patients and procedures were collected from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHD).This analysis reveals that only 38% (117 of 307) of pediatric cardiac anesthesiologists caring for patients with CHD pursued additional training in pediatric cardiac anesthesiology, while 44% (136 of 307) have gained experience during their clinical practice. Other providers have pursued different training pathways such as adult cardiac anesthesiology or pediatric critical care. Based on this survey, pediatric cardiac anesthesiologists devote 35% (interquartile range [IQR], 20%-50%) of clinical time to the care of patients in the cardiac operating room, 25% (20%-35%) of time to the care of patients in the cardiac catheterization laboratory, 10% (5%-10%) to patient care in imaging locations, and 15% covering general pediatric, adult, or cardiac patients undergoing noncardiac procedures. Attempts to actively recruit pediatric cardiac anesthesiologists were reported by 49.2% (29 of 59) of the institutions surveyed. Impending retirement of staff was anticipated in 17% (10 of 59) of the institutions, while loss of staff to relocation was anticipated in 3.4% (2 of 59) of institutions. Thirty-seven percent of institutions reported that they anticipated no immediate changes in current staffing levels.The majority of currently practicing pediatric cardiac anesthesiologists have not completed a fellowship training in the subspecialty. There is, and will continue to be, a need for subspecialty training to meet increasing demand for services especially with increase survival of this patient population and to replace retiring members of the workforce.

Continuous advances in pediatric cardiology, surgery, and critical care have significantly improved survival rates for children and adults with congenital heart disease. Paradoxically, the resulting increase in longevity has expanded the prevalence of both repaired and unrepaired congenital heart disease and has escalated the need for diagnostic and interventional procedures. Because of this expansion in prevalence, anesthesiologists, pediatricians, and other health care professionals increasingly encounter patients with congenital heart disease or other pediatric cardiac diseases who are presenting for surgical treatment of unrelated, noncardiac disease. Patients with congenital heart disease are at high risk for mortality, complications, and reoperation after noncardiac procedures. Rigorous study of risk factors and outcomes has identified subsets of patients with minor, major, and severe congenital heart disease who may have higher-than-baseline risk when undergoing noncardiac procedures, and this has led to the development of risk prediction scores specific to this population. This scientific statement reviews contemporary data on risk from noncardiac procedures, focusing on pediatric patients with congenital heart disease and describing current knowledge on the subject. This scientific statement also addresses preoperative evaluation and testing, perioperative considerations, and postoperative care in this unique patient population and highlights relevant aspects of the pathophysiology of selected conditions that can influence perioperative care and patient management.

The management of children with a borderline ventricle has been debated for many years. The pursuit of a biventricular repair in these children aims to avoid the long-term sequelae of single ventricle palliation. There is a lack of anesthesia literature relating to the care of this complex heterogenous patient population. Anesthesiologists caring for these patients should have an understanding on the many different forms of physiology and the impact on provision of anesthesia and hemodynamic parameters, the goals of biventricular staging and completion as well as the pre-operative, intra-operative, and post-operative considerations relating to this high-risk group of patients.

Introduction: Patients with Fontan physiology require non-cardiac surgery. Our objectives were to characterise perioperative outcomes of patients with Fontan physiology undergoing non-cardiac surgery and to identify characteristics which predict discharge on the same day. Materials and Method: Children and young adults with Fontan physiology who underwent a non-cardiac surgery or an imaging study under anaesthesia between 2013 and 2019 at a single-centre academic children’s hospital were reviewed in a retrospective observational study. Continuous variables were compared using the Wilcoxon rank sum test, and categorical variables were analysed using the Chi-square test or Fisher’s exact test. Multivariable logistic regression analysis results are presented by adjusted odds ratios with 95% confidence intervals and p values. Results: 182 patients underwent 344 non-cardiac procedures with anaesthesia. The median age was 11 years (IQR 5.2–18), 56.4% were male. General anaesthesia was administered in 289 (84%). 125 patients (36.3%) were discharged on the same day. On multivariable analysis, independent predictors that reduced the odds of same-day discharge included the chronic condition index (OR 0.91 per additional chronic condition, 95% CI 0.76–0.98, p = 0.022), undergoing a major surgical procedure (OR 0.17, 95% CI 0.05–0.64, p = 0.009), the use of intraoperative inotropes (OR 0.48, 95% CI 0.25–0.94, p = 0.031), and preoperative admission (OR = 0.24, 95% CI: 0.1–0.57, p = 0.001). Discussion: In a contemporary cohort of paediatric and young adults with Fontan physiology, 36.3% were able to be discharged on the same day of their non-cardiac procedure. Well selected patients with Fontan physiology can undergo anaesthesia without complications and be discharged same day.

Background The type and location of hospitals where patients with congenital heart disease (CHD) undergo noncardiac procedures have not been investigated. This study aimed to describe (1) the characteristics of these patients, (2) the distribution of procedures among hospitals with and without a cardiac surgical program and travel distances, (3) the characteristics determining the distribution, and (4) mortality rates. Methods and Results This is a retrospective cohort analysis of inpatient data from the Center for Healthcare Information and Analysis of the Commonwealth of Massachusetts, Texas Healthcare Information Collection, and Health Care Cost and Utilization Project State Inpatient Database. Children P Conclusions Patients with CHD are more likely to travel to a hospital with a cardiac program for noncardiac procedures than to a hospital without; especially patients with single ventricle disease, other complex CHD, and with ≥6 chronic conditions.

Cardiopulmonary bypass (CPB) circuits can significantly sequester intravenous anesthetics. Adsorption of medications by our institution's standard circuit (Terumo CAPIOX FX05 oxygenator; noncoated polyvinylchloride tubing) has not been described. We prepared ex vivo CPB circuits with and without oxygenators. Medication combinations studied included midazolam (0.5 mg), fentanyl (50 [micro]g), midazolam (0.5 mg) with morphine (0.5 mg), and midazolam (0.5 mg) with fentanyl (50 [micro]g). Medications were administered after obtaining baseline samples. Samples were drawn at 2, 5, 15, 30, 60, 120, and 180 minutes, and analyzed for concentration of injected medications. Midazolam demonstrated no sequestration after 180 minutes. Fentanyl concentration at 180 minutes was lower with an oxygenator (52.7 +/- 12.5 vs. 110.9 +/- 12.0 ng/ml, P = 0.00432). More fentanyl was found in solution after 180 minutes when given with midazolam compared to fentanyl given alone in the presence of an oxygenator (101 +/- 22.3 vs. 52.7 +/- 12.5 ng/ml, P = 0.044). Less midazolam was present after 180 minutes when given with morphine compared to midazolam given alone in the absence of an oxygenator (1264.9 +/- 425.6 vs. 2124 +/- 254 ng/ml, P = 0.037). We successfully characterized the adsorption of various combinations of midazolam, fentanyl, and morphine to our CPB circuit, showing that fentanyl and midazolam behave differently based on other medications present.

Once a mainstay in the treatment of neonates with d-transposition of the great arteries (d-TGA), the application of balloon atrial septostomy (BAS) in the d-TGA population has become more selective. Currently, there is no clear evidence for or against a selective BAS strategy. The aims of this single-center retrospective study were to determine the incidence of BAS in the neonatal d-TGA population in the current era, to measure the rate of procedural success, and to compare the outcomes and complication rates of patients who underwent BAS to those who underwent neonatal ASO alone. Between 2012 and 2018, 147 patients with d-TGA underwent initial medical management and ASO, 73 of which underwent BAS. The percentage of patients that underwent BAS decreased from 73 to 33% over the study time period. In patients with d-TGA with intact ventricular septum, 33% of patients remained off of PGE1 at the time of surgery regardless of BAS. In d-TGA with ventricular septal defect, 85.7% of those that underwent BAS and 54.1% of those who did not remained off of PGE1 at the time of surgery, however, this difference did not reach statistical significance. In this single institution retrospective cohort of patients with d-TGA, the performance of a technically successful balloon atrial septostomy did not eliminate the need for PGE1 therapy at the time of definitive ASO. This was true regardless of the presence or absence of a ventricular septal defect.

The objective of this study is to estimate the surgical risk of noncardiac procedures on the incidence of 30-day mortality in children with congenital heart disease.Children with congenital heart disease undergoing noncardiac surgery from 2012 to 2016 and included in the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) Pediatric database were included in the derivation cohort, while the 2017 database was used as a validation cohort. Intrinsic surgical risk quartiles were built utilizing 30-day mortality rates for each Current Procedural Terminology code and relative value units to create 2 groups defined as low surgical risk (quartiles 1-3) and high surgical risk procedures (quartile 4). We used multivariable logistic regression to determine the predictors for 30-day mortality including patient comorbidities and intrinsic surgical risk. A partially external validation of the model was performed using the 2017 version of the database.We included 37,658 children with congenital heart disease undergoing noncardiac surgery with an incidence of overall 30-day mortality of 1.7% in the derivation cohort and 1.5% in the validation cohort (n = 13,129). Intrinsic surgical risk of procedures represented by Current Procedural Terminology procedural codes and relative value units risk quartiles was significantly associated with 30-day mortality (unadjusted P.001). Predicted probability of 30-day mortality ranges from 0.2% (95% confidence interval [CI], 0.2-0.2) with no comorbidities to 39.6% (95% CI, 23.2-56.0) when all comorbidities were present among high surgical risk procedures and from 0.3% (95% CI, 0.3-0.3) to 54.8% (95% CI, 39.4-70.1) among low surgical risk procedures. An excellent discrimination was reported for the multivariable model with area under the curve (AUC) of 0.86 (95% CI, 0.85-0.88). High surgical risk was not associated with increased odds of 30-day mortality after adjustment for all other predictors (adjusted odds ratio [OR]: 0.75, 95% CI, 0.62-0.91). We also estimated the discriminative ability of a model that does not include the surgical risk (0.86 [95% CI, 0.84-0.88], with P value for the direct comparison of the AUC of the 2 models = 0.831). The multivariable model obtained from an external validation cohort reported an optimism corrected AUC of 0.88 (95% CI, 0.85-0.91).Our study demonstrates that integration of intrinsic surgical risk to comorbidities and severity of cardiac disease does not improve prediction of 30-day mortality in children undergoing noncardiac surgery. In children with congenital heart disease, patient comorbidities, and severity of the cardiac lesion are the predominant predictors of 30-day mortality.

Regional cerebral oxygenation index (rSO2) based on near-infrared spectroscopy (NIRS) is frequently used to detect low venous oxyhemoglobin saturation (ScvO2). We compared the performance of 2 generations of NIRS devices. Clinically obtained, time-matched cerebral rSO2 and ScvO2 values were compared in infants monitored with the FORE-SIGHT (n = 73) or FORE-SIGHT ELITE (n = 47) by linear regression and Bland-Altman analyses. In both devices, cerebral rSO2 correlated poorly with measured ScvO2 (FORE-SIGHT partial correlation 0.50 [95% confidence interval {CI}, 0.40-0.58]; FORE-SIGHT ELITE partial correlation 0.47 [0.39-0.55]) and mean bias was +8 (standard deviation [SD] 13.2) for FORE-SIGHT and +14 (SD 12.5) for FORE-SIGHT ELITE. When ScvO2 was

To determine the incidence of clinically significant serious adverse events in a contemporary population of pediatric patients with pulmonary hypertension who require anesthesia and identify factors associated with adverse outcomes.A retrospective, cross-sectional study.A single-center quaternary-care freestanding children's hospital in the northeastern United States.Pediatric patients with pulmonary hypertension based on hemodynamic criteria on cardiac catheterization during a 3-year period from 2015 to 2018.Anesthesia care for cardiac catheterization, noncardiac surgery, and diagnostic imaging.Two hundred forty-nine children underwent 862 procedures, 592 for cardiac catheterization and 278 for noncardiac surgery and diagnostic imaging. The median age was 1.6 years, and the weight was 9.5 lbs. On index catheterization, median pulmonary artery pressure was 36 mmHg, and the pulmonary vascular resistance was 5.1 indexed Wood units. Ten percent of anesthetics were performed with a natural airway, and 80% used volatile anesthetics. Serious adverse events occurred in 26% of procedures (confidence interval [CI], 22%-30%). The rate of periprocedural cardiac arrest was 8 per 1,000 anesthetic administrations. In multivariate analysis, younger age (adjusted odds ratio [aOR], 1.4 per year; CI, 1.1-1.9; p = 0.01), location in the catheterization laboratory (aOR, 5.1; CI, 1.7-16; p = 0.004), and longer procedure duration (aOR, 1.3 per 30 minutes; CI, 1.1-1.4; p = 0.001) were associated with serious adverse events. Patients with a tracheostomy in place were less likely to experience an adverse event (aOR, 0.1; CI, 0.04-0.5; p = 0.001). The primary anesthetic technique was not associated with adverse events. Interventional cardiac catheterization was associated with an increased incidence of adverse events compared with diagnostic catheterization (42% v 21%; OR, 2.23; CI, 1.5-3.3; p0.001).Serious adverse events were common in this cohort. Careful planning to minimize anesthesia time in young children with pulmonary hypertension should be undertaken, and these factors considered in designing risk mitigation strategies.

Introduction: Ischemia reperfusion injury (IRI) is central to many critical illnesses, including cardiac arrest, myocardial infarction, and stroke. Inhaled hydrogen gas (H2) has been shown to diminish IRI in a number of preclinical models, in part by the selective reduction of oxyradical species. Methods: Following FDA and IRB approval of this Investigator-Initiated Phase I IND study (NCT04046211), 8 healthy participants underwent hospitalized exposure to 2.4% H2 in medical air via high flow nasal cannula (15 LPM) for either 24 hours (n=2), 48 hours (n=2), or 72 hours (n=4). Screening for adverse effects included periodic nurse-adjudicated assessments using Common Terminology Criteria for Adverse Events (CTCAE, v5.0); regularly-spaced vital signs, pulmonary function testing, and mini-mental state exam (MMSE); EKG and serologic testing prior to versus following exposure. Data were compared over time using Wilcoxon matched-pairs signed rank test. All AE assignments were verified by two clinicians external to the study team and an external DSMB. Results: Eight adult participants (18-30 years; 50% female; 62% non-Caucasian) completed the study without early termination. No symptoms or clinically significant adverse events occurred in any patient. Compared with baseline measures, there were no significant changes over time in blood pressure, heart rate, oxygen saturation, or respiratory rate (A). There were no significant changes in PFTs (B), MMSE scores (C), EKG measurements (D) or serologic tests for hematologic (except for clinically-insignificant increases in hematocrit and platelet counts), renal, hepatic, pancreatic, or cardiac injury (E) associated with H2 inhalation. Conclusion: Inhalation of 2.4% H2 gas does not appear to cause clinically significant adverse effects in healthy adults. These data will be foundational to future interventional studies of inhaled H2 in injury states, including following cardiac arrest.

Supplemental Digital Content is available in the text., BACKGROUND: Ischemia-reperfusion injury is common in critically ill patients, and directed therapies are lacking. Inhaled hydrogen gas diminishes ischemia-reperfusion injury in models of shock, stroke, and cardiac arrest. The purpose of this study was to investigate the safety of inhaled hydrogen gas at doses required for a clinical efficacy study. DESIGN: Prospective, single-arm study. SETTING: Tertiary care hospital. PATIENTS/SUBJECTS: Eight healthy adult participants. INTERVENTIONS: Subjects underwent hospitalized exposure to 2.4% hydrogen gas in medical air via high-flow nasal cannula (15 L/min) for 24 (n = 2), 48 (n = 2), or 72 (n = 4) hours. MEASUREMENTS AND MAIN RESULTS: Endpoints included vital signs, patient- and nurse-reported signs and symptoms (stratified according to clinical significance), pulmonary function testing, 12-lead electrocardiogram, mini-mental state examinations, neurologic examination, and serologic testing prior to and following exposure. All adverse events were verified by two clinicians external to the study team and an external Data and Safety Monitoring Board. All eight participants (18–30 yr; 50% female; 62% non-Caucasian) completed the study without early termination. No clinically significant adverse events occurred in any patient. Compared with baseline measures, there were no clinically significant changes over time in vital signs, pulmonary function testing results, Mini-Mental State Examination scores, neurologic examination findings, electrocardiogram measurements, or serologic tests for hematologic (except for clinically insignificant increases in hematocrit and platelet counts), renal, hepatic, pancreatic, or cardiac injury associated with hydrogen gas inhalation. CONCLUSIONS: Inhalation of 2.4% hydrogen gas does not appear to cause clinically significant adverse effects in healthy adults. Although these data suggest that inhaled hydrogen gas may be well tolerated, future studies need to be powered to further evaluate safety. These data will be foundational to future interventional studies of inhaled hydrogen gas in injury states, including following cardiac arrest.

Point of care ultrasonography (POCUS) is a rapidly evolving diagnostic modality. Equipment to scan various parts of the body with compact handheld devices is readily available. Surface cardiac ultrasound (US) can be used to rapidly assess volume status, cardiac function, and the detection of pericardial fluid. In the case presented here, we describe the use of a novel, hands-free, biplane, transthoracic echocardiography (TTE) probe with a design that provided continuous acquisition of cardiac US images during an operative procedure.

BACKGROUND: Acute kidney injury (AKI) has been well documented in adults after noncardiac surgery and demonstrated to be associated with adverse outcomes. We report the prevalence of AKI after pediatric noncardiac surgery, the perioperative factors associated with postoperative AKI, and the association of AKI with postoperative outcomes in children undergoing noncardiac surgery. METHODS: Patients ≤18 years of age who underwent noncardiac surgery with serum creatinine during the 12 months preceding surgery and no history of end-stage renal disease were included in this retrospective observational study at a single tertiary academic hospital. Patients were evaluated during the first 7 days after surgery for development of any stage of AKI, according to Kidney Disease: Improving Global Outcomes (KDIGO) criteria. Patients were classified into stages of KDIGO AKI for the purposes of describing prevalence. For further analyses, patients were grouped into those who developed any stage of AKI postoperatively and those who did not. Additionally, the time point at which each patient was first diagnosed with stage I AKI or greater was also assessed. Pre-, intra-, and postoperative factors were compared between the 2 groups. A multivariable Cox proportional hazards regression model was created to examine the time to first diagnosis of AKI using all nonredundant covariates. Analysis of the association of AKI with postoperative outcomes, mortality and 30-day readmission, was undertaken utilizing propensity score–matched controls and a multivariable Cox proportional hazards regression model. RESULTS: A total of 25,203 cases between 2013 and 2018 occurred; 8924 met inclusion criteria. Among this cohort, the observed prevalence of postoperative AKI was 3.2% (288 cases; confidence interval [CI], 2.9–3.6). The multivariable Cox model showed American Society of Anesthesiologists (ASA) status to be associated with the development of postoperative AKI. Several other factors, including intraoperative hypotension, were significantly associated with postoperative AKI in univariable models but found not to be significantly associated after adjustment. The multivariable Cox analyses with propensity-matched controls showed an estimated hazard ratio of 3.28 for mortality (CI, 1.71–6.32, P < .001) and 1.55 for 30-day readmission (CI, 1.08–2.23, P = .018) in children who developed AKI versus those who did not. CONCLUSIONS: In children undergoing noncardiac surgery, postoperative AKI occurred in 3.2% of patients. Several factors, including intraoperative hypotension, were significantly associated with postoperative AKI in univariable models. After adjustment, only ASA status was found to be significantly associated with AKI in children after noncardiac surgery. Postoperative AKI was found to be associated with significantly higher rates of mortality and 30-day readmission in multivariable, time-varying models with propensity-matched controls.

To compare outcomes in children with congenital heart disease (CHD) undergoing noncardiac surgery by presence of chronic conditions and identify associated risk factors.Retrospective analysis of 14 031 children with CHD who underwent noncardiac surgery in the 2016 Healthcare Cost and Utilization Project Kid's Inpatient Database. Multivariable regression was used to assess patient and hospital factors associated with in-hospital mortality and length of stay (LOS).Overall, 94% had at least 1 chronic condition. The in-hospital mortality rate was 5.6%. Neonates with CHD only had the highest mortality (15.6%); otherwise, children with CHD and at least 1 chronic condition had higher mortality than patients with CHD only (infant 3.93%, child 1.22%, adolescent 1.04% vs 2.34%, 0%, and 0%). Neonates (OR, 15.5; 95% CI, 7.1-34.1 vs adolescent), number of chronic conditions (OR, 1.34; 95% CI, 1.27-1.42), chronic conditions type (circulatory system; OR 2.46; 95% CI, 2.04-2.98), and low socioeconomic status (OR, 1.36; 95% CI, 1.05-1.77) were associated with increased mortality. The median LOS was 20 days (IQR, 5-66). Those with CHD and at least 1 chronic condition had a greater LOS (21 days; IQR, 5-68) than those with CHD only (9 days; IQR, 3-46). Neonates (adjusted coefficient, 44.3; 95% CI, 40.3-48.3 vs adolescent), Black race (adjusted coefficient, 4.78; 95% CI, 2.27-7.3), chronic condition indicator number (adjusted coefficient, 5.17; 95% CI, 4.56-5.78), and subtype (adjusted coefficient, 23.6; 95% CI, 20.4-26.7) were associated with a prolonged LOS.Most children with CHD who undergo noncardiac surgery have at least 1 chronic condition. Age, chronic conditions type and number, low socioeconomic status, and Black race impart increased risks of in-hospital mortality and prolonged LOS. Further research is needed to evaluate the impact of specific chronic conditions and determine barriers to equitable care.

THIS ARTICLE IS a review of the highlights of pertinent literature published during the 12 months of 2018 that is of interest to the congenital cardiac anesthesiologist. During a search of the US National Library of Medicine PubMed database, several topics that displayed significant contributions to the field in 2018 emerged. The authors of the present review consider the following topics noteworthy: the patient with high-risk congenital heart disease (CHD) presenting for noncardiac surgery, cardiopulmonary resuscitation in infants and children with CHD, dexmedetomidine use in pediatric patients, point-of-care lung ultrasound, and regional anesthesia in pediatric cardiac surgery.

Early identification of children at high risk for perioperative mortality could lead to improved outcomes; however, there is a lack of well-validated risk prediction tools. The Pediatric Risk Assessment (PRAm) score is a new model to prognosticate perioperative risk of mortality in pediatric patients undergoing noncardiac surgery. It was derived from the American College of Surgeons (ACS) National Surgical Quality Improvement Program (NSQIP) Pediatric database. In this study, we aimed to externally validate the PRAm score at 1 large institution.A PRAm score was prospectively assigned by the primary anesthesia team to children ≤18 years of age undergoing noncardiac surgery between July 2017 and July 2018 at a tertiary care pediatric hospital. The primary outcome was the PRAm score's ability to predict 30-day mortality. The area under the receiver operating characteristic (ROC) curve was utilized to determine discriminative ability. Sensitivity and specificity at varying cutoffs were considered. Youden J index and the gray zone approach were applied to determine the optimal PRAm cutoff for predicting 30-day mortality.Among the 13,530 cases included in the external validation cohort, the incidence of 30-day mortality was 0.21% (29/13,530). The PRAm score was found to predict 30-day mortality with an area under the curve (AUC) of 0.956 (95% confidence interval [CI], 0.938-0.974; P.001). Youden J index determined the optimal PRAm score threshold to be ≥5 with a sensitivity of 86% and a specificity of 91%. The gray zone identified an inconclusive risk of mortality in 6.93% (938/13,530) of patients who had PRAm scores of 4 or 5 (sensitivity or specificity90%, respectively), therefore refining the optimal cutoff point to be a PRAm score of ≥6. The incidence of mortality for patients with an American Society of Anesthesiologists Physical Status (ASA PS) ≤3 (0.06%, 8/13,530) increased 8-fold for those with an ASA PS of ≤3 and a PRAm score of ≥6.The PRAm score is a simple and objective tool that has excellent ability to predict perioperative risk of mortality in pediatric patients undergoing noncardiac surgery and can be easily used by clinicians. The application of the PRAm score could have important implications on the safety and quality of care delivered to infants and children and on the resource utilization in the pediatric health care system.

Objective The utility of extracorporeal membrane oxygenation (ECMO) as an elective support modality for high-risk cardiac procedures is extensively described in adults, but its use in children is limited to isolated reports. The objective of this study was to analyze the outcomes of patients who underwent elective cannulation to ECMO for this purpose. Design Single-center, retrospective chart review. Setting Free-standing pediatric tertiary care center. Participants Patients who underwent elective cannulation to ECMO for cardiorespiratory support during a high-risk cardiac catheterization procedure. Interventions Elective ECMO cannulation for high-risk percutaneous cardiac interventions or electrophysiology procedures. Measurements and Main Results Survival to discharge was 71.4% compared with 30% for patients who required extracorporeal cardiopulmonary resuscitation in the cardiac catheterization laboratory. The mean duration of cannulation was 137.43 hours (range 27-615 h, median 55 h). There were no major neurologic sequelae, but ECMO circuit thrombosis (57%) was relatively common. Conclusion The use of elective ECMO support for high-risk pediatric cardiac catheterizations can be accomplished safely and may allow for an improved rate of survival with lower rates of severe adverse events compared with extracorporeal cardiopulmonary resuscitation as rescue therapy.

Editor’s Perspective What We Already Know about This Topic What This Article Tells Us That Is New Background Recently developed risk stratification models for perioperative mortality incorporate patient comorbidities as predictors but fail to consider the intrinsic risk of surgical procedures. In this study, the authors used the American College of Surgeons National Surgical Quality Improvement Program Pediatric database to demonstrate the relationship between the intrinsic surgical risk and 30-day mortality and develop and validate an accessible risk stratification model that includes the surgical procedures in addition to the patient comorbidities and physical status. Methods A retrospective analysis of the American College of Surgeons National Surgical Quality Improvement Program Pediatric database was performed. The incidence of 30-day mortality was the primary outcome. Surgical Current Procedural Terminology codes with at least 25 occurrences were included. Multivariable logistic regression model was used to determine the predictors for mortality including patient comorbidities and intrinsic surgical risk. An internal validation using bootstrap resampling, and an external validation of the model were performed. Results The authors analyzed 367,065 surgical cases encompassing 659 unique Current Procedural Terminology codes with an incidence of overall 30-day mortality of 0.34%. Intrinsic risk of surgical procedures represented by Current Procedural Terminology risk quartiles instead of broad categorization was significantly associated with 30-day mortality (P < 0.001). Predicted risk of 30-day mortality ranges from 0% with no comorbidities to 4.7% when all comorbidities are present among low-risk surgical procedures and from 0.07 to 46.7% among high-risk surgical procedures. Using an external validation cohort of 110,474 observations, the multivariable predictive risk model displayed good calibration and excellent discrimination with area under curve (c-index) equals 0.95 (95% CI, 0.94 to 0.96; P < 0.001). Conclusions Understanding and accurately estimating perioperative risk by accounting for the intrinsic risk of surgical procedures and patient comorbidities will lead to a more comprehensive discussion between patients, families, and providers and could potentially be used to conduct cost analysis and allocate resources.

Bivalirudin is a direct thrombin inhibitor that is used as a procedural anticoagulant during percutaneous coronary interventions and cardiac surgery for patients with heparin-resistant thrombosis or heparin-induced thrombocytopenia. There is a robust literature describing its safety and efficacy in adults; however, its use in the pediatric population is relatively rare, with dosing extrapolated from adult data. In this case report, we describe a 4-year-old with complex congenital heart disease and history of heparin-induced thrombocytopenia who required bivalirudin dose uptitration during cardiac catheterization.

Recent advances in catheterization and imaging technology allow for more complex procedures to be performed in the catheterization laboratory. A number of lesions are now amenable to a percutaneous procedure, eliminating or at least postponing the need for a surgical intervention. Due to the increase in the complexity of the procedures performed, the involvement of anesthesiologists and their close collaboration with the interventional cardiologists have increased. It is important to understand the physiology and pathophysiology of the patients and to anticipate the plans and the potential complications in order to manage them. We are witnessing a rise in the number of complex interventions in newborns and infants, such as balloon valvotomy (critical aortic stenosis, pulmonary stenosis), radio frequency perforation (of pulmonary atresia and intact ventricular septum), right ventricular outflow tract stenting (in Tetralogy of Fallot), ductal stenting (in some ductus-dependent pulmonary circulation), and combined with a surgical procedure (hybrid procedure for hypoplastic left heart syndrome). Multiple registries have been created in order to understand and improve outcomes of patients with congenital heart disease undergoing catheterization procedures and to develop performance and quality metrics, from which data regarding anesthetic-related risks can be extrapolated. Experienced personnel and a multidisciplinary team approach with direct communication among the team members is a must to ensure anticipation and management of critical events when they occur.

Bivalirudin (Angiomax; The Medicines Company, Parsippany, NJ), a direct thrombin inhibitor, has found increasing utilization as a heparin alternative in the pediatric population, most commonly for the treatment of thrombosis secondary to heparin-induced thrombocytopenia. Due to the relative rarity of heparin-induced thrombocytopenia as well as the lack of Food and Drug Administration-approved indications in this age group, much of what is known regarding the pharmacokinetics and pharmacodynamics of bivalirudin in this population has been extrapolated from adult data. This narrative review will present recommendations regarding the use of bivalirudin for procedural anticoagulation in the pediatric population based on the published literature.

Objective To determine whether children with Down syndrome (DS) receive higher doses of opioid medications compared with children without DS for repair of complete atrioventricular canal (CAVC). Design A retrospective chart review of children with and without DS who underwent primary repair of CAVC. The exclusion criteria included unbalanced CAVC and patients undergoing biventricular staging procedures. The primary outcome was oral morphine equivalents (OME) received in the first 24 hours after surgery. The secondary outcomes included intraoperative OME, OME at 48 and 72 hours, nonopioid analgesic and sedative medications received, pain scores, time to extubation, and length of stay. Setting A pediatric academic medical center in the United States. Participants One hundred thirty-one patients with DS and 24 without, all Interventions Not applicable. Measurements and Main Results Patients with DS were older than patients without DS (median 96.3 days [interquartile range {IQR} 70.7-128.2] v 75.9 days [IQR 49.8-107.3], p = 0.033) but otherwise not statistically different in the baseline characteristics. There was no difference in OME received in the first 24 hours postoperatively between groups (3.01 mg/kg [IQR 1.23-5.43] v 3.57 mg/kg [IQR 1.54-7.06], p = 0.202). OME at 48 and 72 hours was lower in the DS group compared with the control group. Similar amounts of opioid and non-opioid analgesics and sedatives were otherwise given to both groups of patients. Median pain scores did not differ between groups. Conclusions These results suggested that patients with DS undergoing CAVC repair do not have increased opioid requirements compared with a similar control group.

Viscoelastic hemostatic assays (VHA) are point of care tests that allow for a global assessment of coagulation using whole blood. The technology to allow this assessment has evolved from the original thromboelastography (TEG, Haemonetic, Boston, MA) to now include thromboelastometry (ROTEM, Instrumentation Laboratory, Bedford, MA), and, most recently, the Quantra Hemostasis Analyzer (Hemosonics, Charlottesville, VA). Diagnosis and treatment algorithms incorporating viscoelastic hemostatic tests for bleeding patients in a variety of clinical situations have now been developed. The original ROTEM and TEG technologies have been updated with emphasis placed on a cartridge-based technologies. Results from the new devices show good correlation with those from the previous versions of the devices, while cartridge-based technology has increased device stability and enhanced portability to the bedside. In this article, we will review recent advances in TEG and ROTEM technology and introduce the Quantra Hemostasis Analyzer device.

BACKGROUND The association between race and perioperative outcomes has been evaluated in adult cardiac surgical and in healthy pediatric patients but has not been evaluated in children with congenital heart disease (CHD) presenting for noncardiac procedures. This study compares the incidence of the primary outcome of 30-day mortality and adverse postoperative outcomes following noncardiac surgery between Black and White children with CHD, stratified by severity. METHODS This is a retrospective study. Comparison of outcomes between Black and White children was performed using the 2012-2018 American College of Surgeons National Surgical Quality Improvement Program Pediatric database and after stratification for severity of CHD and propensity score matching. RESULTS A total of 55,859 patients were included, and divided into 28,601 minor, 23,839 major, and 3419 severe CHD. Black and White children in each category were matched and compared. Following matching in the overall CHD cohort, there were significantly higher rates of the following adverse postoperative outcomes among Black patients as compared to White patients: 30-day mortality (1.84% vs 1.49%; odds ratio [OR], 1.25; 95% confidence interval [CI], 1.05-1.48; P = .014), composite secondary outcomes (19.90% vs 17.88%; OR, 1.14; 95% CI, 1.08-1.21; P < .001), cardiac arrest (1.42% vs 0.98%; OR, 1.46; 95% CI, 1.19-1.79; P < .001), 30-day reoperation (7.59% vs 6.67%; OR, 1.15; 95% CI, 1.05-1.25; P = .002), and reintubation (3.9% vs 2.95%; OR, 1.34; 95% CI, 1.19-1.52; P < .001). No significant statistical interaction between race and CHD severity was found. Following matching and within the minor CHD cohort, Black children had significantly higher rates of composite secondary outcome (17.44% vs 15.60%; OR, 1.15; 95% CI, 1.05-1.25; P = .002), cardiac arrest (1.02% vs 0.53%; OR, 1.94; 95% CI, 1.37-2.76; P < .001), 30-day reoperation (7.19% vs 5.77%; OR, 1.26; 95% CI, 1.11-1.43; P < .001), and thromboembolic complications (0.49% vs 0.23%; OR, 2.17; 95% CI, 1.29-3.63; P = .003) compared to White children. In the major CHD cohort, Black children had significantly higher rates of 30-day mortality (2.75% vs 2.05%; OR, 1.35; 95% CI, 1.08-1.69; P = .008) and reintubation (4.82% vs 3.72%; OR, 1.32; 95% CI, 1.11-1.56; P = .002). There were no statistically significant differences in outcomes in the severe CHD category for 30-day mortality (3.36% vs 3.3%; OR, 1.02; 95% CI, 0.60-1.73; P = .946), composite secondary outcome (22.65% vs 21.36%; OR, 1.08; 95% CI, 0.86-1.36; P = .517) nor the components of the composite secondary outcomes. CONCLUSIONS Race is associated with postoperative mortality and complications in children with minor and major CHD undergoing noncardiac surgery. No significant association was observed between race and postoperative outcomes in patients with severe CHD. This is consistent with previous findings wherein in patients with severe CHD, residual lesion burden and functional status is the leading predictor of outcomes following noncardiac surgery. Nevertheless, there is no evidence that the relationship between race and outcomes differs across the CHD severity categories. Future studies to understand the mechanisms leading to the racial difference, including institutional, clinical, and individual factors are needed.

Management of difficult tracheal intubation during induction of anesthesia in children with congenital heart disease is challenging. The aim of this study is to evaluate the incidence of difficult tracheal intubation in patients with congenital heart disease and compare the incidence of perioperative complications and outcomes in patients with and without difficult tracheal intubation.Retrospective cohort study.Tertiary Children's Hospital.6858 patient-encounters including cardiac diagnostic, interventional or surgical procedures from 2012 to 2018 were reviewed.age 18 years, endotracheal tube or tracheostomy in-situ.Patients' demographics, number and methods of intubation, peri-intubation hemodynamics, intensive care unit and postoperative hospital length of stay were recorded. Multivariable mixed-effects median, logistic, ordinal, and multinomial regression modeling were implemented to analyze outcomes in the matched sets.Of the 6014 encounters examined in the study, the incidence of DTI was 0.96% and all 58 difficult tracheal intubations (DTI) were matched using 1:2 propensity score matching to 116 non-DTI encounters. Number of intubation attempts was significantly higher among patients with difficult tracheal intubation (ordinal logistic regression odds ratio = 2; 95% CI; 1.3, 2.7; P 0.001). No significant differences in peri-intubation hemodynamic stability were noted. Patients with difficult tracheal intubation had longer postoperative hospital length of stay (median = 12.1 vs 7.9 days, coef. = 4; 95% CI: 1.3, 6.8; P = 0.004) than patients without.Despite a higher number of intubation attempts, our study shows no major differences in the peri-intubation hemodynamics in patients with and without difficult tracheal intubation. This risk can be mitigated by a good understanding of cardiac physiology, management of hemodynamics, and early use of an indirect intubation technique to maximize first attempt success.

Objective:To evaluate the discriminative ability of hyperlactataemia for early morbidity and mortality in neonates with CHD following cardiac surgery.Methods:Retrospective, observational study of neonates who underwent cardiac surgery on cardiopulmonary bypass at a tertiary care children’s hospital from June 2015 to June 2019. The primary predictor was lactate. The primary composite outcome was defined as ≥1 of the following: cardiac arrest or extracorporeal membrane oxygenation within 72 hours or 30-day mortality post-operatively. The secondary outcome was the presence of major residual lesions, according to the Technical Performance Score.Results:Of 432 neonates, 28 (6.5%) sustained the composite outcome. On univariate analysis, peak lactate within 48 hours, increase in lactate from ICU admission through 12 hours, and single ventricle physiology were significantly associated with the composite outcome. The peak lactate occurred at a median of 2.9 hours (interquartile range: 1, 35) before the event. Through multi-variable analysis, a multi-variable risk algorithm was created. Predicted probabilities demonstrated an increasing risk based on single ventricle status and delta lactate, ranging from 1.8% (95% CI: 0.9, 3.9) to 52.4% (95% CI: 32.4, 71.7). The model had good discriminative ability for the composite outcome on receiver operating characteristic analysis (area under the curve = 0.79; 95% CI: 0.75, 0.89). Moreover, a peak lactate of 7.3 mmol/l or greater was significantly associated with the presence of a major residual lesion (odds ratios: 5.16, 95% CI: 3.01, 8.87).Conclusions:We present a simple, two-variable model, including delta lactate in the immediate post-operative period and single ventricle status, to prognosticate the risk of early morbidity and mortality in neonates undergoing cardiac surgery for potential intervention.

Advances made in pediatric cardiology, cardiac surgery and critical care have significantly improved the survival rate of patients with congenital heart disease (CHD) leading to an increase in children with CHD presenting for noncardiac surgical procedures. This study aims (1) to describe the trend and perioperative mortality rates in patients with CHD undergoing noncardiac surgical procedures at children’s hospitals over the past 5 years and (2) to describe the patient characteristics and the most common type of surgical procedures. The Pediatric Health Information System (PHIS) is an administrative database that contains inpatient, observation, and outpatient surgical data from 52 freestanding children’s hospitals. Thirty-nine of the 52 hospitals submitted data on all types of patient encounters for the duration of the study from 2015 to 2019. The total numbers of non-cardiac surgical encounters among patients with history of a CHD diagnosis significantly increased each year from 38,272 in 2015 to 45,993 in 2019 (P

A 13-year-old 30 kg female with a dilated cardiomyopathy (DCM) and progressive heart failure is being considered for orthotopic heart transplantation (OHT). Due to a precipitous decline in the patient’s cardiovascular status culminating in intensive care unit admission and initiation of maximal medical therapy, the decision has been made to semi-electively provide mechanical circulatory support (MCS). The chosen device is a HeartWare centrifugal pump as a left ventricular assist device (LVAD).

Perioperative bleeding is one of the major comorbidities associated with surgery. While anesthesia is a critical component to perform surgery, a number of clinical studies supported the contribution of anesthetic drugs to perioperative bleeding. Here, we reviewed the literature on this topic including the underlying mechanism and discussed the future direction on coagulation research in anesthesia.

Context Thyroid function may be assessed in children before cardiac surgery because of concerns that hypothyroidism or thyrotoxicosis might adversely affect cardiac function perioperatively. However, the relationship between preoperative thyroid dysfunction and surgical outcomes is unknown. Objective Determine the relationship between preoperative thyroid dysfunction and outcomes of pediatric cardiac surgery. Methods Retrospective cohort study (January 2005 to July 2019). Setting Academic pediatric hospital. Patients All patients Main Outcome Measures Subjects were stratified by preoperative TSH concentration (mIU/L): low (10). Outcomes were compared among subjects with normal TSH (control) and each group with abnormal TSH concentrations. The primary outcome was 30-day mortality. Secondary outcomes included time to extubation, intensive care unit and hospital length of stay, and operative complications. Results Among 592 patients analyzed, preoperative TSH was low in 15 (2.5%), normal in 347 (58.6%), mildly high in 177 (29.9%), and moderately high in 53 (9.0%). Free thyroxine was measured in 77.4% of patients and was low in 0 to 4.4% of subjects, with no differences among TSH groups. Thirty-day mortality was similar among TSH groups. There were no differences in any secondary outcome between patients with abnormal TSH and patients with normal TSH. Conclusion Preoperative mild to moderate subclinical hypothyroidism was not associated with adverse postoperative outcomes in children undergoing cardiopulmonary bypass procedures.