The Year in Review: Anesthesia for Congenital Heart Disease 2022.

This review focuses on the literature published during the calendar year 2022 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease (CHD). Four major themes are discussed: enhanced recovery after surgery(ERAS); diversity, equity, and inclusion; the state of pediatric cardiac anesthesiology as a subspecialty in the United States; and neuromonitoring for pediatric cardiac surgery.

Racial and Ethnic Disparities in U.S Children Undergoing Surgery for Congenital Heart Disease: A Narrative Literature Review.

Congenital Heart Disease (CHD) is a significant source of pediatric morbidity and mortality. As in other fields of medicine, studies have demonstrated racial and ethnic disparities in congenital heart disease outcomes. The cause of these outcome disparities is multifactorial, involving biological, behavioral, environmental, sociocultural, and systemic medical factors. Potential contributors include differences in preoperative illness severity secondary to coexisting medical conditions, differences in the rate of prenatal and early postnatal detection of CHD, and delayed access to care, as well as discrepancies in socioeconomic and insurance status, and systemic disparities in hospital care. Understanding the factors that contribute to these disparities is an essential step towards developing strategies to address them. As stewards of the perioperative surgical home, anesthesiologists have an important role in developing institutional policies that mitigate racial disparities. Here, we provide a thorough narrative review of recent research concerning perioperative factors contributing to surgical outcomes disparities for children of all ages with CHD, examine potentially modifiable contributing factors, discuss avenues for future research, and suggest strategies to address disparities both locally and nationally.

The Year in Review: Anesthesia for Congenital Heart Disease 2021.

This review focuses on the literature published during the calendar year 2021 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease. Four major themes are discussed, including cardiovascular disease in children with COVID-19, aortic valve repair and replacement, bleeding and coagulation, and enhanced recovery after surgery (ERAS).

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

The Year in Review: Anesthesia for Congenital Heart Disease 2020.

This review focuses on the literature published during the calendar year 2020 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease. Five major themes are discussed, including COVID-19 in children with heart disease, race and outcome disparities in congenital heart disease, Norwood procedure and outcomes, Fontan procedure and outcomes, and neurotoxicity/neurologic outcomes. A total of 59 peer-reviewed articles are discussed.

Associations between anthropometric indices and outcomes of congenital heart operations in infants and young children: An analysis of data from the Society of Thoracic Surgeons Database.

BACKGROUND: Children with congenital heart disease are at risk for growth failure due to inadequate nutrient intake and increased metabolic demands. We examined the relationship between anthropometric indices of nutrition (height-for-age z-score [HAZ], weight-for-age z-score [WAZ], weight-for-height z-score [WHZ]) and outcomes in a large sample of children undergoing surgery for congenital heart disease. METHODS: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having index cardiac surgery at age 1 month to 10 years were included. Indices were calculated by comparing patients' weight and height to population norms from the World Health Organization and Centers for Disease Control and Prevention. Outcomes included operative mortality, composite mortality or major complication, major postoperative infection, and postoperative length of stay. For each outcome and index, the adjusted odds ratio (aOR) (for mortality, composite outcome, and infection) and adjusted relative change in median (for postoperative length of stay) for a 1-unit decrease in index were estimated using mixed-effects logistic and log-linear regression models. RESULTS: Every unit decrease in HAZ was associated with 1.40 aOR of mortality (95% CI 1.32-1.48), and every unit decrease in WAZ was associated with 1.33 aOR for mortality (95% CI 1.25-1.41). The relationship between WHZ and outcome was nonlinear, with aOR of mortality of 0.84 (95% CI 0.76-0.93) for 1-unit decrease when WHZ ≥ 0 and a nonsignificant association for WHZ < 0. Trends for other outcomes were similar. Overall, the incidence of low nutritional indices was similar for 1-ventricle and 2-ventricle patients. Children between the age of 1 month and 1 year and those with lesions associated with pulmonary overcirculation had the highest incidence of low nutritional indices. CONCLUSIONS: Lower HAZ and WAZ, suggestive of malnutrition, are associated with increased mortality and other adverse outcomes after cardiac surgery in infants and young children. Higher WHZ over zero, suggestive of obesity, is also associated with adverse outcomes.

Selected 2019 Highlights in Congenital Cardiac Anesthesia.

This article is a review of the highlights of pertinent literature published in 2019, which is of interest to the pediatric cardiac anesthesiologist. After a search of the United States National Library of Medicine PubMed database, several topics emerged in which significant contributions were made in 2019. The authors of this manuscript considered the following topics noteworthy and were included in this review: advances in pediatric heart transplantation, blood management in pediatric cardiac surgery, the impact of nutrition on outcomes in congenital heart surgery, and the use of vasopressin in patients after Fontan palliation.

Assessment of muscle oxygenation in children with congenital heart disease.

BACKGROUND: Adaptive responses to congenital heart disease result in altered muscle perfusion and muscle metabolism. Such changes may be detectable using noninvasive spectroscopic monitors. AIMS: In this study we aimed to determine if resting muscle oxygen saturation (MOx) is lower in children with acyanotic or cyanotic congenital heart disease than in healthy children and to identify differences in muscle oxygen consumption in children with cyanotic and acyanotic congenital heart disease. METHODS: Using a custom fiber optic spectrometer system, optical measurements were obtained from the calf or forearm of 49 patients (17 with acyanotic congenital heart disease, 18 with cyanotic congenital heart disease, and 14 control). Twenty additional control patients were used to develop the analytic model. Spectra were used to determine MOx at baseline, during arterial occlusion, and during reperfusion. The rate of muscle desaturation during arterial occlusion was also evaluated. Two-sample t-tests were used to compare each heart disease group with the controls. RESULTS: Patients with acyanotic and cyanotic congenital heart disease had lower baseline MOx than controls. Baseline MOx was 91.3% (CI 85.9%, 96.7%) for acyanotic patients, 91.1% (CI 86.3%, 95.9%) for cyanotic patients, and 98.9% (CI 96.7%, 101.1%) for controls. Similarly, MOx was lower in the acyanotic and cyanotic groups than the controls after reperfusion (84.6% [CI 74.1%, 95.1%] and 82.1% [CI 74.5%, 89.7%] vs 98.9% [96.5%, 101.3%]). The rate of decline in oxygenation was significantly greater in cyanotic patients versus controls (0.46%/s (CI 0.30%, 0.62%/s) vs 0.17%/s (0.13%, 0.21%/s)). CONCLUSION: This study demonstrates that muscle oxygenation is abnormal in children with both cyanotic and acyanotic congenital heart disease. This suggests that noninvasive monitoring of muscle oxygenation may provide valuable information in situations where children with congenital heart disease may be at risk of hemodynamic compromise.

Current perspectives on treatment of perioperative hemodynamic instability and hypotension.

Overall, there are numerous causes of hypotension in the perioperative period. The approach to definitive treatment must be tailored to the child's unique anatomy and physiology, as well as the current factors presumed to be eliciting the hypotensive state. It is imperative to consider both routine and lesion-specific etiologies to the current hypotensive episode. Lastly, when employing pharmacologic therapy for hypotension, there are often multiple combinations of medications that can reasonably be used to achieve the desired hemodynamic effects.

Perioperative and Anesthetic Management of Coarctation of the Aorta.

Isolated coarctation of the aorta is a relatively common form of congenital heart disease that is characterized by variable degrees of obstruction to aortic outflow. The clinical presentation varies from asymptomatic arterial hypertension to cardiogenic shock. The treatment options include surgical repair or interventional therapy with aortic balloon dilation and stent placement. This article will summarize the pathophysiology as well as describe the surgical and interventional procedures. The anesthetic management for those interventions will be reviewed.

Perioperative and Anesthetic Considerations in Truncus Arteriosus.

Truncus arteriosus is a congenital cardiac lesion in which failure of embryonic truncal septation results in a single semilunar valve and single arterial trunk providing both pulmonary and systemic circulations. Most patients with this lesion are symptomatic in the neonatal period with cyanosis and/or congestive heart failure and undergo complete repair in the first weeks of life. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with truncus arteriosus.

Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation. Cardiac transplantation is recommended in the most severe cases. This review will discuss the perioperative and anesthetic management of patients with PA/IVS and highlight the challenges in management.

Perioperative and Anesthetic Considerations in Atrioventricular Septal Defect.

Atrioventricular septal defect results from a failure of normal endocardial cushion fusion during embryologic cardiac development. This developmental aberration results in defects in the atrial and/or ventricular septum and malformation of the atrioventricular valves. The pathophysiology of atrioventricular septal defect is variable, and ranges from mild left to right shunting similar to a simple atrial septal defect to complex single-ventricle heart disease. This review focuses on the spectrum of atrioventricular septal defect from partial to complete, without associated cardiac defects.

Assessing the Impact of the Anesthesia Medication Template on Medication Errors During Anesthesia: A Prospective Study.

BACKGROUND: Medication errors continue to be a significant source of patient harm in the operating room with few concrete countermeasures. The organization and identification of medication syringes may have an impact on the commission of medication errors in anesthesia, so a team of physicians and designers at the University of Washington created the Anesthesia Medication Template (AMT) to define a formal way of organizing the anesthesia workspace. The purpose of this study is to assess the ability of the AMT to reduce perioperative medication errors by anesthesia providers. METHODS: This study evaluated the AMT in 2 phases: (1) 41 anesthesia providers administered medications in 2 prospective, randomized operating room simulations with or without the AMT, while medication errors were directly observed; and (2) around 200 providers prospectively self-reported medication errors from all anesthetizing locations during a 2-year period at Seattle Children's Hospital, an academic, pediatric medical center. RESULTS: In simulated emergencies, the odds of medication dosing errors using the AMT were 0.21 times the odds of medication dosing errors without AMT (95% confidence interval [CI], 0.07, 0.66), controlling for scenario, session, training level, and years at training level. During the year after implementation of the AMT, the mean monthly error rate for all reported medication errors that reached patients decreased from 1.24 (95% CI, 0.85-1.79) to 0.65 (95% CI, 0.39-1.09) errors per 1000 anesthetics. The mean monthly error rate of reported swap, preparation, miscalculation, and timing errors decreased from 0.97 (95% CI, 0.64-1.48) to 0.35 (95% CI, 0.17-0.70) errors per 1000 anesthetics. Medication errors that resulted in patient harm did not change after implementation of the AMT. CONCLUSIONS: Standardizing medication organization with the AMT is an intuitive, low-cost strategy with the potential to improve patient safety through reducing medication errors by anesthesia providers.

Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection.

Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions.

Perioperative morbidity in children with elastin arteriopathy.

BACKGROUND: Children with elastin arteriopathy (EA), the majority of whom have Williams-Beuren syndrome, are at high risk for sudden death. Case reports suggest that the risk of perioperative cardiac arrest and death is high, but none have reported the frequency or risk factors for morbidity and mortality in an entire cohort of children with EA undergoing anesthesia. AIM: The aim of this study was to present one institution's rate of morbidity and mortality in all children with EA undergoing anesthesia and to examine patient characteristics that pose the greatest risk. METHODS: We reviewed medical records of children with EA who underwent anesthesia or sedation for any procedure at our institution from 1990 to 2013. Cardiovascular hemodynamic indices from recent cardiac catheterization or echocardiography were tabulated for each child. The incidence, type, and associated factors of complications occurring intraoperatively through 48 h postoperatively were examined. RESULTS: Forty-eight patients with confirmed EA underwent a total of 141 anesthetics. There were seven cardiac arrests (15% of patients, 5% of anesthetics) and nine additional intraoperative cardiovascular complications (15% of patients, 6% of anesthetics). Extracorporeal life support was initiated in five cases. There were no perioperative deaths. All children having a cardiac arrest or complication were <3 years old and had biventricular outflow tract obstruction (BVOTO). Subgroup analysis demonstrated high rates of cardiac arrest in two groups: children with BVOTO (44%) and age <3 years old (21%). CONCLUSIONS: We have confirmed that the rate of cardiac arrest and complications is significantly elevated in children with EA undergoing anesthesia. Children <3 years old and with BVOTO were at the greatest risk in our population.

A first look at the Accreditation Council for Graduate Medical Education anesthesiology milestones: implementation of self-evaluation in a large residency program.

STUDY OBJECTIVE: The objective was to determine if there is a correlation between resident postgraduate year (PGY) of training and self-evaluation of performance using the Accreditation Council for Graduate Medical Education milestones. DESIGN: Survey. SETTING: Residency program at a large academic center. PATIENTS: Residents and Faculty Clinical Competency Committee (CCC). INTERVENTIONS: None. MEASUREMENTS: Resident and CCC milestone scores. MAIN RESULTS: Correlation coefficients for average score for each milestone vs PGY level ranged from 0.80 for receiving and giving feedback to 0.95 for anesthetic choice and conduct. All milestones showed a relatively linear relationship with PGY of training, and none were found to be consistently reached very late or very early in training. When examining variation across the scores for the individual residents, the distributions for PGY-2 and -3 appeared to be wider than those for PGY-1 and -4. The intraclass correlation coefficients ranged from 0.718 to 0.928. CONCLUSIONS: There was a remarkable degree of consistency in the relationship between level of training and resident self-assessment score for every milestone, as well as strong agreement between the resident and CCC faculty scores. Examination of the variance in the scores, when interpreted in light of our particular training program's characteristics, suggests that the milestones accurately reflect the progression in skill across the residency. In addition, given the concordance between the self-evaluation scores and the CCC faculty scores, self-evaluation may be a reasonable starting point as programs begin the daunting task of determining scores for each of the 25 milestones as part of the biannual evaluation process.

Perioperative and Anesthetic Considerations in Ebstein's Anomaly.

Ebstein's anomaly is a complex and heterogeneous form of congenital heart disease characterized by malformation and apical displacement of the tricuspid valve leaflets. Patients may present at any time from the neonatal period to adulthood with symptoms ranging from cardiac failure and cyanosis to paroxysmal arrhythmias. Depending on the timing of presentation, various surgical options are available for the management of symptomatic patients. This review will discuss the perioperative and anesthetic management of patients with Ebstein's anomaly with reference to the more common surgical approaches.