RESUMEN
BACKGROUND: Opioids have been a central component of routine adult and pediatric anesthesia for decades. However, the long-term effects of perioperative opioids are concerning. Recent studies show a 4.8%-6.5% incidence of persistent opioid use after surgery in older children and adults. This means that >2 million of the 50 million patients undergoing elective surgeries in the United States each year are likely to develop persistent opioid use. With this in mind, anesthesiologists at Bellevue Clinic and Surgery Center assembled an interdisciplinary quality improvement team focused on 2 goals: (1) develop effective anesthesia protocols that minimize perioperative opioids and (2) add value to clinical services by maintaining or improving perioperative outcomes while reducing costs. This article describes our project and findings but does not attempt to make inferences or generalizations about populations outside our facility. METHODS: We performed a large-scale implementation of opioid-sparing protocols at our standalone pediatric clinic and ambulatory surgery facility, based in part on the prior success of our previously published tonsillectomy and adenoidectomy protocol. Multiple Plan-Do-Study-Act cycles were performed using data captured from the electronic medical record. The percentage of surgical patients receiving intraoperative opioids and postoperative morphine preintervention and postintervention were compared. The following measures were evaluated using statistical process control charts: maximum postoperative pain score, postoperative morphine rescue rate, total postanesthesia care unit minutes, total anesthesia minutes, and postoperative nausea and vomiting rescue rate. Intraoperative analgesic costs were calculated. RESULTS: Between January 2017 and June 2019, 10,948 surgeries were performed at Bellevue, with 10,733 cases included in the analyses. Between December 2017 and June 2019, intraoperative opioid administration at our institution decreased from 84% to 8%, and postoperative morphine administration declined from 11% to 6% using analgesics such as dexmedetomidine, nonsteroidal anti-inflammatory drugs, and regional anesthesia. Postoperative nausea and vomiting rescue rate decreased, while maximum postoperative pain scores, total anesthesia minutes, and total postanesthesia care unit minutes remained stable per control chart analyses. Costs improved. CONCLUSIONS: By utilizing dexmedetomidine, nonsteroidal anti-inflammatory drugs, and regional anesthesia for pediatric ambulatory surgeries at our facility, perioperative opioids were minimized without compromising patient outcomes or value.
Asunto(s)
Procedimientos Quirúrgicos Ambulatorios , Analgésicos Opioides/administración & dosificación , Anestesia , Hospitales Pediátricos , Dolor Postoperatorio/prevención & control , Mejoramiento de la Calidad , Indicadores de Calidad de la Atención de Salud , Centros Quirúrgicos , Adolescente , Adulto , Factores de Edad , Procedimientos Quirúrgicos Ambulatorios/efectos adversos , Analgésicos Opioides/efectos adversos , Anestesia/efectos adversos , Niño , Preescolar , Esquema de Medicación , Utilización de Medicamentos , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Dolor Postoperatorio/diagnóstico , Dolor Postoperatorio/etiología , Atención Perioperativa , Evaluación de Programas y Proyectos de Salud , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
CHILDREN with cancer and survivors of childhood cancer have an increased risk of cardiovascular disease, and this risk in the perioperative period must be understood. During diagnosis and treatment of pediatric cancer, multiple acute cardiovascular morbidities are possible, including anterior mediastinal mass, tamponade, hypertension, cardiomyopathy,and heart failure. Childhood cancer survivors reaching late childhood and adulthood experience substantially increased rates of cardiomyopathy, heart failure, valvular disease, pericardiac disease, ischemia, and arrhythmias. Despite considerable advances in the understanding and therapeutic options of pediatric malignancies, cardiac disease remains the most common treatment-related, noncancer cause of death in childhood cancer survivors. Increasingly, molecularly targeted agents, including small molecule inhibitors, are being incorporated into pediatric oncology. The acute and chronic risks associated with these newer therapeutic options in children are not yet well-described, which poses challenges for clinicians caring for these patients. In the present review, the unique risks factors, prevention strategies, and treatment of cardiovascular toxicities of the child with cancer and the childhood cancer survivor are examined, with an emphasis on the perioperative period.
Asunto(s)
Antineoplásicos , Insuficiencia Cardíaca , Neoplasias , Adulto , Antraciclinas/uso terapéutico , Antineoplásicos/uso terapéutico , Niño , Insuficiencia Cardíaca/tratamiento farmacológico , Humanos , Neoplasias/tratamiento farmacológico , Neoplasias/epidemiología , Factores de Riesgo , SobrevivientesRESUMEN
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 10â¯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 WHZâ¯≥ 0 and a nonsignificant association for WHZ <â¯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 1â¯month and 1â¯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.
Asunto(s)
Antropometría/métodos , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas/cirugía , Estado Nutricional , Complicaciones Posoperatorias/epidemiología , Sociedades Médicas , Cirugía Torácica/estadística & datos numéricos , Peso Corporal , Niño , Preescolar , Bases de Datos Factuales , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Factores de Riesgo , Tasa de Supervivencia/tendencias , Estados Unidos/epidemiologíaRESUMEN
Pediatric pulmonary hypertension is a complex disease with multiple, diverse etiologies affecting the premature neonate to the young adult. Pediatric pulmonary arterial hypertension, whether idiopathic or associated with congenital heart disease, is the most commonly discussed form of pediatric pulmonary hypertension, as it is progressive and lethal. However, neonatal forms of pulmonary hypertension are vastly more frequent, and while most cases are transient, the risk of morbidity and mortality in this group deserves recognition. Pulmonary hypertension due to left heart disease is another subset increasingly recognized as an important cause of pediatric pulmonary hypertension. One aspect of pediatric pulmonary hypertension is very clear: anesthetizing the child with pulmonary hypertension is associated with a significantly heightened risk of morbidity and mortality. It is therefore imperative that anesthesiologists who care for children with pulmonary hypertension have a firm understanding of the pathophysiology of the various forms of pediatric pulmonary hypertension, the impact of anesthesia and sedation in the setting of pulmonary hypertension, and anesthesiologists' role as perioperative experts from preoperative planning to postoperative disposition. This review summarizes the current understanding of pediatric pulmonary hypertension physiology, preoperative risk stratification, anesthetic risk, and intraoperative considerations relevant to the underlying pathophysiology of various forms of pediatric pulmonary hypertension.
Asunto(s)
Hipertensión Pulmonar/complicaciones , Atención Perioperativa/métodos , Anestesia , Anestésicos/uso terapéutico , Niño , Preescolar , Hipertensión Pulmonar Primaria Familiar/complicaciones , Cardiopatías Congénitas/complicaciones , Humanos , Hipertensión Pulmonar/fisiopatología , Lactante , Recién Nacido , Hipertensión Arterial Pulmonar/complicaciones , Medición de RiesgoRESUMEN
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.
Asunto(s)
Hemodinámica , Hipotensión/terapia , Periodo Perioperatorio , Anestesia/efectos adversos , Niño , Preescolar , Humanos , Hipotensión/diagnóstico , Lactante , Recién NacidoRESUMEN
Pediatric cardiac anesthesiology has evolved as a subspecialty of both pediatric and cardiac anesthesiology and is devoted to caring for individuals with congenital heart disease ranging in age from neonates to adults. Training in pediatric cardiac anesthesia is a second-year fellowship with variability in both training duration and content and is not accredited by the Accreditation Council on Graduate Medical Education. Consequently, in this article and based on the Accreditation Council on Graduate Medical Education Milestones Model, an expert panel of the Congenital Cardiac Anesthesia Society, a section of the Society of Pediatric Anesthesiology, defines 18 milestones as competency-based developmental outcomes for training in the pediatric cardiac anesthesia fellowship.
Asunto(s)
Anestesia en Procedimientos Quirúrgicos Cardíacos/normas , Competencia Clínica/normas , Consenso , Educación de Postgrado en Medicina/normas , Cardiopatías Congénitas/terapia , Sociedades Médicas/normas , Anestesia en Procedimientos Quirúrgicos Cardíacos/métodos , Educación de Postgrado en Medicina/métodos , Becas/métodos , Becas/normas , HumanosRESUMEN
The Institute of Medicine has called for development of strategies to prevent medication errors, which are one important cause of preventable harm. Although the field of anesthesiology is considered a leader in patient safety, recent data suggest high medication error rates in anesthesia practice. Unfortunately, few error prevention strategies for anesthesia providers have been implemented. Using Toyota Production System quality improvement methodology, a multidisciplinary team observed 133 h of medication practice in the operating room at a tertiary care freestanding children's hospital. A failure mode and effects analysis was conducted to systematically deconstruct and evaluate each medication handling process step and score possible failure modes to quantify areas of risk. A bundle of five targeted countermeasures were identified and implemented over 12 months. Improvements in syringe labeling (73 to 96%), standardization of medication organization in the anesthesia workspace (0 to 100%), and two-provider infusion checks (23 to 59%) were observed. Medication error reporting improved during the project and was subsequently maintained. After intervention, the median medication error rate decreased from 1.56 to 0.95 per 1000 anesthetics. The frequency of medication error harm events reaching the patient also decreased. Systematic evaluation and standardization of medication handling processes by anesthesia providers in the operating room can decrease medication errors and improve patient safety.
Asunto(s)
Anestesia/normas , Análisis de Modo y Efecto de Fallas en la Atención de la Salud , Errores de Medicación/prevención & control , Anestesia/efectos adversos , Niño , Etiquetado de Medicamentos/normas , Guías como Asunto , Hospitales Pediátricos , Humanos , Bombas de Infusión/normas , Quirófanos/organización & administración , Seguridad del Paciente , Pediatría , Mejoramiento de la Calidad , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Anestesia , Paro Cardíaco/epidemiología , Complicaciones Intraoperatorias/epidemiología , Complicaciones Posoperatorias/epidemiología , Síndrome de Williams/epidemiología , Preescolar , Comorbilidad , Elastina , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Factores de RiesgoRESUMEN
Myofibrillar myopathy (MFM) is a relatively newly recognized genetic disease that leads to progressive muscle deterioration. MFM has a varied phenotypic presentation and impacts cardiac, skeletal, and smooth muscles. Affected individuals are at increased risk of respiratory failure, significant cardiac conduction abnormalities, cardiomyopathy, and sudden cardiac death. In addition, significant skeletal muscle involvement is common, which may lead to contractures, respiratory insufficiency, and airway compromise as the disease progresses. This study is the first report of anesthetic management of a patient with MFM. We report multiple anesthetic encounters of a child with genetically confirmed BAG3-myopathy, a subtype of MFM with severe childhood disease onset. A review of the anesthetic implications of the disease is provided, with specific exploration of possible susceptibility to malignant hyperthermia, rhabdomyolysis, and sensitivity to other anesthetic agents.
Asunto(s)
Anestesia , Enfermedades Musculares/cirugía , Miofibrillas/patología , Anestésicos , Humanos , Lactante , Masculino , Hipertermia Maligna/terapia , Enfermedades Musculares/genética , Enfermedades Musculares/patología , Rabdomiólisis/terapiaAsunto(s)
Anestesiología/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Pediatría/métodos , Cirugía Torácica/métodos , Cirugía Torácica/normas , Análisis Químico de la Sangre , Análisis de los Gases de la Sangre , Canadá , Niño , Estudios de Seguimiento , Corazón , Hemostasis , Humanos , Pautas de la Práctica en Medicina , Estudios Retrospectivos , Especialidades Quirúrgicas , Encuestas y Cuestionarios , Estados UnidosRESUMEN
The reported incidence of venous thromboembolism (VTE) in children has increased dramatically over the past decade, and the primary risk factor for VTE in neonates and infants is the presence of a central venous catheter (CVC). Although the associated morbidity and mortality are significant, very few trials have been conducted in children to guide clinicians in the prophylaxis, diagnosis, and treatment of CVC-related VTE. Furthermore, pediatric guidelines for prophylaxis and management of VTE are largely extrapolated from adult data. How then should the anesthesiologist approach central access in children of different ages to lessen the risk of CVC-related VTE or in children with prior thrombosis and vessel occlusion? A comprehensive review of the pediatric and adult literature is presented with the goal of assisting anesthesiologists with point-of-care decision-making regarding the risk factors, diagnosis, and treatment of CVC-related VTE. Illustrative cases are also provided to highlight decision-making in varying situations. The only risk factor strongly associated with CVC-related VTE formation in children is the duration of the indwelling CVC. Several other factors show a trend toward altering the incidence of CVC-related VTE formation and may be under the control of the anesthesiologist placing and managing the catheter. In particular, because children with VTE may live decades with its sequelae and chronic vein thrombosis, careful consideration of lessening the risk of VTE is warranted in every child. Further studies are needed to form a clearer understanding of the risk factors, prophylaxis, and management of CVC-related VTE in children and to guide the anesthesiologist in lessening the risk of VTE.
Asunto(s)
Cateterismo Venoso Central/efectos adversos , Catéteres Venosos Centrales , Tromboembolia Venosa/etiología , Femenino , Humanos , Lactante , Masculino , Factores de Riesgo , Tromboembolia Venosa/terapiaRESUMEN
Patients with congenital supravalvar aortic stenosis (SVAS) with associated biventricular outflow tract obstruction and coronary artery abnormalities have a tenuous myocardial oxygen supply/demand relationship. They are at increased risk of acute myocardial ischemia and sudden death, especially during anesthesia. Furthermore, resuscitation during cardiac arrest is frequently unsuccessful. We report a case of perioperative cardiac arrest due to an unexpected cause in a 2 month old with SVAS during a laparoscopic Nissen fundoplication.
Asunto(s)
Estenosis Aórtica Supravalvular/cirugía , Fundoplicación/efectos adversos , Paro Cardíaco/etiología , Laparoscopía/métodos , Neumoperitoneo Artificial/efectos adversos , Estenosis Aórtica Supravalvular/congénito , Soluciones Cristaloides , Epinefrina/uso terapéutico , Fundoplicación/métodos , Paro Cardíaco/tratamiento farmacológico , Humanos , Hipotensión/etiología , Lactante , Soluciones Isotónicas/uso terapéutico , Masculino , Oximetría/métodos , Neumoperitoneo Artificial/métodos , Resucitación/métodos , Simpatomiméticos/uso terapéutico , Resultado del TratamientoRESUMEN
BACKGROUND: Oxymetazoline nasal spray is not FDA approved for use in children less than 6 years; however, its safety and efficacy are widely accepted, and it is in widespread use in children prior to procedures that may lead to epistaxis. We report a case of intraoperative oxymetazoline toxicity in a 4-year-old boy that led to a hypertensive crisis. While examining the possible causes for this problem, we became aware that the method of drug delivery led to an unanticipated overdose. The position in which the bottle is held causes pronounced variation in the quantity of oxymetazoline dispensed. METHODS: To examine the impact that bottle position has on the volume delivered, we measured the volume of oxymetazoline dispensed with the bottle in the upright and inverted position. We also measured the volume of a drop of oxymetazoline dispensed from the bottle. Because an additional source of oxymetazoline exposure is from packing the nares with surgical pledgets, we analyzed the volume of oxymetazoline absorbed by each pledget. RESULTS: Squeezing the bottle in the upright position results in a fine spray of fluid that averaged 28.9 ± 6.8 µl and was largely independent of effort. This volume is nearly identical to the measured volume of a drop of oxymetazoline, which was 30 µl. However, squeezing the bottle in the inverted position resulted in a steady stream of fluid, and the volume administered was completely effort dependent. Multiple tests in the inverted position demonstrated an average volume of 1037 ± 527 µl, with a range of 473-2196 µl. Lastly, the volume of oxymetazoline absorbed by each surgical pledget was 1511 ± 184 µl. DISCUSSION: Our testing indicates that bottle position during oxymetazoline administration can cause up to a 75-fold increase in intended drug administration.
Asunto(s)
Hipertensión/inducido químicamente , Hipertensión/prevención & control , Descongestionantes Nasales/efectos adversos , Oximetazolina/efectos adversos , Administración por Inhalación , Administración Intranasal , Presión Sanguínea/efectos de los fármacos , Niño , Preescolar , Sobredosis de Droga/prevención & control , Falla de Equipo , Humanos , Masculino , Descongestionantes Nasales/administración & dosificación , Oximetazolina/administración & dosificación , Radiografía DentalRESUMEN
BACKGROUND: Cannulation of small arteries and veins in young children can be challenging. Although anesthesiologists frequently use ultrasound for placement of central venous lines and nerve blocks, its use for cannulation of small, peripheral vessels is less helpful. Ultrasound systems (7-15 MHz) currently used in clinical practice focus poorly at the sub-10-mm space and thus lack the resolution to allow accurate ultrasound-guided cannulation of small vessels. High-frequency micro-ultrasound (HFMU) is a new technology that allows higher resolution (15-50 MHz) compared with conventional ultrasound. Limited human studies have been performed thus far with HFMU, and none have been performed in young children or for vascular access. METHODS: This study was conducted to determine the feasibility of using HFMU to visualize and cannulate peripheral arteries and central veins in children under the age of 6 years old. The diameter of radial and ulnar arteries was also measured. RESULTS: The anesthesiologists involved in this study found the 50 MHz HFMU probe useful for cannulation of peripheral arteries, especially in the youngest children. The higher-frequency probes were less helpful for internal jugular vein cannulation because it was not always possible to view the carotid artery while cannulating the vein. CONCLUSIONS: The experience gained in this feasibility study suggests that HFMU could be a valuable addition to our armamentarium for difficult vascular access in the future.
Asunto(s)
Arterias/diagnóstico por imagen , Ultrasonografía Intervencional/métodos , Dispositivos de Acceso Vascular , Venas/diagnóstico por imagen , Factores de Edad , Cateterismo/métodos , Cateterismo Venoso Central , Preescolar , Estudios de Factibilidad , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Palpación , Medición de Riesgo , Programas InformáticosRESUMEN
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.
Asunto(s)
Anestesia , Anestesiología , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Adulto , Humanos , Niño , Cardiopatías Congénitas/cirugía , CorazónRESUMEN
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).
Asunto(s)
Anestesia , Anestesiología , COVID-19 , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Adulto , Niño , Cardiopatías Congénitas/cirugía , HumanosRESUMEN
Anesthesiologists are important components of volunteer teams which perform congenital cardiac surgery in low-resource settings throughout the world, but limited data exist to characterize the nature and breadth of their work. A survey of Congenital Cardiac Anesthesia Society (CCAS) members was conducted with the objective of understanding the type of voluntary care being provided, its geographic reach, the frequency of volunteer activities, and factors which may encourage or limit anesthesiologists' involvement in this work. The survey was completed by 108 participants. Respondents reported a total of 115 volunteer trips during the study period, including work in 41 countries on 5 continents. Frequent motivating factors to begin volunteering included invitations from charitable groups, encouragement from senior colleagues, and direct connections to individual locations. Discouraging factors included familial responsibilities, the need to use vacation time, and a lack of support from home institutions. The year 2020 saw a marked decrease in reported volunteer activity, and respondents reported multiple pandemic-related factors which might discourage future volunteer activities. The results of this study demonstrate the global reach of anesthesiologists in providing care for children having cardiac surgery. It also offers insights into the challenges faced by interested individuals, many of which are related to a lack of institutional support. These challenges have only mounted under the COVID-pandemic, leading to a dramatic downturn in volunteer activities. Finally, the survey reinforces the need for better coordination of volunteer activities to optimize clinical impact.
Asunto(s)
Anestesia en Procedimientos Quirúrgicos Cardíacos , COVID-19 , Procedimientos Quirúrgicos Cardíacos , Anestesiólogos , Niño , Humanos , Encuestas y CuestionariosRESUMEN
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.