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Acute decompensated heart failure (ADHF) in pediatrics is a significant cause for morbidity and mortality in children. Congenital heart disease and cardiomyopathy are the leading etiologies of ADHF. It is common for these children to undergo diagnostic, therapeutic, or surgical procedure under anesthesia, which may be associated with significant morbidity and mortality. The importance of preanesthetic multidisciplinary planning with all involved teams, including anesthesia, cardiology, intensive care, perfusion, and cardiac surgery, cannot be emphasized enough. In order to safely manage these patients, it is imperative for the anesthesiologist to understand the complex pathophysiological interactions between cardiopulmonary systems and anesthesia during these procedures. This review discusses the etiology, pathophysiology, clinical manifestations, and perioperative management of these patients.

Background: Early extubation is performed either in the operating room or in the cardiovascular intensive care unit during the first 24 postoperative hours; however, altitude might possibly affect the process. The aim of this study is the evaluation of early extubation feasibility of patients undergoing congenital heart surgery in a center located at 2,691 m (8,828 ft.) above sea level. Material and Methods: Patients undergoing congenital heart surgery, from August 2012 through December 2018, were considered for early extubation. The following variables were recorded: weight, serum lactate, presence or not of Down syndrome, optimal oxygenation and acid–base status according to individual physiological condition (biventricular or univentricular), age, bypass time, and ventricular function. Standardized anesthetic management with dexmedetomidine–fentanyl–rocuronium and sevoflurane was used. If extubation in the operating room was considered, 0.08 mL/kg of 0.5% ropivacaine was injected into the parasternal intercostal spaces bilaterally before closing the sternum. Results: Four hundred seventy-eight patients were operated and 81% were early extubated. Mean pre- and postoperative SaO2 was 92% and 98%; postoperative SaO2 for Glenn and Fontan procedures patients was 82% and 91%, respectively. Seventy-three percent of patients who underwent Glenn procedure, 89% of those who underwent Fontan procedure (all nonfenestrated), and 85% with Down syndrome were extubated in the operating room. Reintubation rate in early extubated patients was 3.6%. Conclusion: Early extubation is feasible, with low reintubation rates, at 2,691 m (8,828 ft.) above sea level, even in patients with single ventricle physiology.

This article is a review of the highlights of pertinent literature of interest to the congenital cardiac anesthesiologist and was published in 2021. After a search of the United States National Library of Medicine PubMed database, several topics emerged where significant contributions were made in 2021. The authors of this manuscript considered the following topics noteworthy to be included in this review: risk stratification in adult congenital heart disease surgery, physician burnout in pediatric cardiac anesthesia, transfusion practice in pediatric congenital heart surgery, and racial disparity and outcomes in pediatric patients with congenital heart disease.

PURPOSE OF REVIEW The current review focuses on the new development of adult congenital heart disease (ACHD) patients in the areas of imaging, percutaneous interventions, ventricular assist devices and transplantation. RECENT FINDINGS Since the last ACHD publication in the journal, several advances have been made in the evaluation and treatment of these patients. As CHD patients' longevity increases pregnancy, comorbities and acquired heart disease become a concern. Recent data show that the incidence of complications in low-risk CHD is not higher that the regular population. In addition, breakthrough research in percutaneous valve implantation has been published showing good outcomes but needing intensive care recovery in a significant number of patients. In the ACHD heart failure population, assist device and transplant fields mounting evidence shows that these therapies should not be the last resort since low-risk ACHD patient may have similar outcomes to those with acquired heart disease. Finally risk stratification is important in ACHD to define better ways to recover from surgery and anesthesia. SUMMARY The field of anesthesia for ACHD is growing with new indications for diagnostic, interventional and surgical procedures. Tailoring cardiac and noncardiac care to the different risk profile in ACHD patients will be defined in the next few years. VIDEO ABSTRACT Motta summary clip: http://links.lww.com/COAN/A65.

There are currently in North America more adults with congenital heart disease than children. This article discusses the anesthetic considerations in adults with single-ventricle physiology and prior repairs who present for Fontan conversion surgery as a demonstration of the challenges of caring for adults undergoing interventions for the repair of congenital heart defects. The care of these patients requires an understanding of the impact of passive pulmonary blood flow and single systemic ventricular physiology. The perioperative morbidity in this patient population remains high.

Transesophageal echocardiography (TEE) plays an important role in the anatomical, functional, and hemodynamic assessment of patients with congenital heart disease (CHD). This imaging approach has been applied to both children and adults with a wide range of cardiovascular malformations. Extensive clinical experience documents significant contributions, particularly in the perioperative setting. In fact, in the current medical era, many consider this technology to be an essential adjunct to surgical and anesthetic management in CHD. This review focuses on the applications of TEE in patients with tetralogy of Fallot (TOF), the most common form of cyanotic heart disease. Emphasis is given to the perioperative use of this imaging modality and benefits derived during the prebypass and postbypass periods. Limitations and pitfalls relevant to the TEE assessment in patients with this anomaly are also addressed.

To evaluate the hemodynamic effects of the long-acting, alpha-adrenergic blocker, phenoxybenzamine, in children of different age groups.Retrospective chart review.Tertiary-care, congenital cardiac surgery center.The data of 75 pediatric patients who received phenoxybenzamine while undergoing surgical repair of congenital heart defects on cardiopulmonary bypass (CPB) were studied. Patients were selected in three age groups: younger than one month (n = 25), one to 12 months (n = 25), and one to 5 years (n = 25). All patients received a full dose of phenoxybenzamine 1 mg/kg. Demographics, CPB duration, mean arterial pressure on CPB, mean flow on CPB (normalized to body surface area), and central-to-peripheral temperature gradients were recorded. Systemic vascular resistance index (SVRI) was calculated.Cardiopulmonary bypass duration was significantly longer in the age group of younger than 1 mo than in the older groups. Mean CPB flow/body surface area was similar in all children (3.45 +/- 0.9, 3.74 +/- 0.69, and 3.48 +/- 0.59 L/min/m2, respectively; P0.28). However, mean SVRI was significantly lower in children younger than 1 mo (997.3 +/- 233, 1196.9 +/- 394, and 1168.83 +/- 227 dynes/cm2m5; P0.04). Temperature gradient was significantly narrower in patients younger than one month than those who were one to 12 months and one to 5 years at the end of cooling (0.90 degrees C +/- 0.1 degrees C, 1.04 degrees C +/- 3.61 degrees C, 1.4 degrees C +/- 3.07 degrees C; P0.001) at end-rewarming and termination of CPB (4.58 degrees C +/- 2.36 degrees C, 6.23 degrees C +/- 4.17 degrees C, 7.32 degrees C +/- 3.46 degrees C; P0.02). Multivariate analysis showed that patient age was a significant variable affecting response to phenoxybenzamine, after adjusting for duration of CPB (P = 0.31), mean hematocrit on CPB (P = 0.86), and core cooling temperature (P = 0.34).The effect of phenoxybenzamine on SVRI, cooling, and rewarming on CPB varies with age as shown by more profound vasodilatation and narrower temperature gradients.

Regional cerebral oxygenation can be monitored using near-infrared spectroscopy (NIRS). Inadequacy of collateral cerebral circulation and regional cerebral ischemia during cardiac and vascular surgery may be detected by the use of NIRS monitoring. We report a 2-year-old child who underwent surgical repair of vascular ring and subclavian reimplantation, where use of NIRS helped in early detection and timely intervention to prevent prolonged cerebral ischemia.

"Renal dose" dopamine (rDA; 1-3 microg/kg per min) is administered to patients after cardiac surgery to preserve or improve renal function. Many of these patients develop new-onset postoperative atrial fibrillation or atrial flutter (pAF) that could be related to rDA administration. The objective of this investigation was to determine whether there was an association between rDA and new-onset pAF in patients undergoing coronary artery bypass grafting with cardiopulmonary bypass (CABG).Research hospital.The study population consisted of 1,731 patients undergoing CABG.CABG with and without rDA.After approval by the institutional review board, a retrospective study using the Cardiothoracic Anesthesia Patient Registry was undertaken to determine the association between rDA and pAF in patients undergoing CABG. Patients with a documented history of atrial fibrillation, those who required inotrope use during or after surgery, and those having valve surgery were excluded.One-thousand seven-hundred thirty-one patients undergoing CABG during the period of January 1, 2000, through June 30, 2002, were the study population; of these, 15.0% (260/1,731) developed pAF. The incidence of pAF was 23.3 % (41/176) among patients who received rDA and 14.1% (219/1,555) among those who did not receive rDA. In the multivariable logistic regression model, patient age, gender, chronic obstructive pulmonary disease or asthma, and rDA were associated with pAF (p.01). Receipt of rDA increased the odds of developing pAF by 74%, independent of the effect of other variables.Renal-dose dopamine is associated with a 1.74 odds ratio of pAF developing after CABG.

IMPLICATIONS: The authors describe the management of a patient with arrhythmogenic right ventricular dysplasia treated with right ventricular exclusion surgery.

The population of children with end-stage heart failure requiring mechanical circulatory support is growing. These children present for diagnostic imaging studies, various interventions and noncardiac surgical procedures that require anesthetic care. This article is a review of the population demographics of children on mechanical cardiac support, the alternative devices available, and the important concepts for safe perioperative management of these patients. The discussion will be limited to devices for short- and long-term cardiac support, excluding extracorporeal membrane oxygenation (ECMO) for respiratory support.

The purpose of this study was to compare the effects of a direct-acting arterial dilator, sodium nitroprusside, to an alpha-adrenergic receptor blocker, phenoxybenzamine, in infants with congenital heart defects undergoing cardiac repairs on cardiopulmonary bypass.A prospective, multicenter, observational study.Tertiary care center.Sixty infants scheduled for elective congenital cardiac surgery repair requiring cardiopulmonary bypass.Patients received either sodium nitroprusside 2 to 5 microg/kg/min infusion intraoperatively and in the intensive care unit (n=30 patients) or received phenoxybenzamine 1 mg/kg slowly intravenously at the onset of cardiopulmonary bypass (n=30 patients).Despite similar mean arterial pressures during cardiopulmonary bypass in both groups, infants who received phenoxybenzamine had a significantly higher flow compared with those who received sodium nitroprusside (180+/-4.8 v 73+/-5.12 mL/kg/min, p0.0001). Base deficit was significantly larger in the sodium nitroprusside group compared with the phenoxybenzamine group intraoperatively and postoperatively (3.4+/-0.5 v 1.3+/-0.5 mEq/L, p0.05). The core-to-peripheral temperature gradient was significantly larger in the sodium nitroprusside group compared with the phenoxybenzamine group intra- and postoperatively at all points studied. In the intensive care unit, the left atrial pressure was significantly higher in the sodium nitroprusside group compared with the phenoxybenzamine group (9+/-0.4 v 7+/-0.4 mmHg, por=0.0005).The use of phenoxybenzamine can maintain organ perfusion on cardiopulmonary bypass and improve peripheral circulation as shown by less base deficit and smaller temperature gradients intraoperatively and in the intensive care unit better than nitroprusside.

Objective: To evaluate the effectiveness and safety of the ALLON 2001 microprocessor-based thermoregulation system in pediatric patients undergoing cardiac surgery requiring hypothermic cardiopulmonary bypass compared with the routine thermal care. Design: Prospective randomized clinical study. Setting: Single tertiary academic medical center. Participants: Infants (0-1 year) who underwent congenital heart surgery requiring hypothermic cardiopulmonary bypass (n 18). Patients with open wounds and/or patients treated with an investigational drug or device within 30 days of surgery were excluded. Interventions: Randomized use of thermoregulation system (warming garment, n 9) or routine thermal care (control, n 9) after separating from cardiopulmonary bypass until the arrival to the pediatric intensive care unit (PICU). Measurements and Main Results: There were no statistically significant differences in the demographic data, cardiopulmonary bypass time, operating room time, incidence of deep hypothermic circulatory arrest, and cooling temperature between the groups. The nasopharyngeal temperature was significantly higher in the warming garment group after separation from cardiopulmonary bypass. Nasopharyngeal temperature at 20 minutes was 36.5°C versus 35.01°C (p 0.0047), at 40 minutes was 36.98°C versus 35.30°C (p 0.034), and at admission to the PICU was 36.09°C versus 35.31°C (p not significant). There was no difference in the core-to-peripheral temperature gradient (nasopharyngealto-skin temperature) between the 2 study groups at any time point. No adverse events related to the use of the warming garment thermoregulation system were observed. Conclusion: The investigated thermoregulation system was effective in preventing the after-drop of temperature that occurs after cardiopulmonary bypass in small infants compared with routine warming methods. © 2004 Elsevier Inc. All rights reserved.

SPASMODIC dysphonia (SD) is a laryngeal disorder characterized by adductor or abductor dystonic spasms of the vocal cord (VC). It is usually manifested by abnormal phonation but may infrequently be associated with respiratory distress. We describe a patient with SD who developed recurrent severe postoperative stridor that required a tracheostomy.