Selected 2022 Highlights in Congenital Cardiac Anesthesia.

This article is a review of the highlights of pertinent literature of interest to the congenital cardiac anesthesiologist, and was published in 2022. After a search of the United States National Library of Medicine PubMed database, several topics emerged in which significant contributions were made in 2022. The authors of this manuscript considered the following topics noteworthy to be included in this review-intensive care unit admission after congenital cardiac catheterization interventions, antifibrinolytics in pediatric cardiac surgery, the current status of the pediatric cardiac anesthesia workforce in the United States, and kidney injury and renal protection during congenital heart surgery.

Management of routine postoperative pain for children undergoing cardiac surgery: a Paediatric Acute Care Cardiology Collaborative Clinical Practice Guideline.

BACKGROUND: Pain following surgery for cardiac disease is ubiquitous, and optimal management is important. Despite this, there is large practice variation. To address this, the Paediatric Acute Care Cardiology Collaborative undertook the effort to create this clinical practice guideline. METHODS: A panel of experts consisting of paediatric cardiologists, advanced practice practitioners, pharmacists, a paediatric cardiothoracic surgeon, and a paediatric cardiac anaesthesiologist was convened. The literature was searched for relevant articles and Collaborative sites submitted centre-specific protocols for postoperative pain management. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus. RESULTS: 60 recommendations achieved consensus and are included in this guideline. They address guideline use, pain assessment, general considerations, preoperative considerations, intraoperative considerations, regional anaesthesia, opioids, opioid-sparing, non-opioid medications, non-pharmaceutical pain management, and discharge considerations. CONCLUSIONS: Postoperative pain among children following cardiac surgery is currently an area of significant practice variability despite a large body of literature and the presence of centre-specific protocols. Central to the recommendations included in this guideline is the concept that ideal pain management begins with preoperative counselling and continues through to patient discharge. Overall, the quality of evidence supporting recommendations is low. There is ongoing need for research in this area, particularly in paediatric populations.

Twin-twin transfusion syndrome complicated by single ventricle physiology: A case report.

Clinically significant extrauterine twin-twin transfusion syndrome in conjoined twins is rare and carries a high risk of perinatal mortality. The ensuing postnatal imbalance in circulation across connecting vessels results in hypovolemia in the donor and hypervolemia in the recipient. Data on management and treatment are sparse especially in the setting of a single ventricle congenital heart defect. We present a case of a pair of omphalopagus conjoined twins, one with a single ventricle physiology (Twin B), who developed twin-twin transfusion syndrome shortly after birth. The resulting pathophysiology in the setting of a single ventricle congenital heart defect created added layers of complexity to their management and expedited surgical separation. Shunting from Twin B to Twin A-with an anatomically normal heart-resulted in mal-perfusion and rapid deterioration jeopardizing the health of both twins. In the preoperative course, steps taken to medically optimize the twins prior to surgery and the anesthetic considerations are detailed in this report.

3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions.

Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study. Demographic, advanced imaging, and catheterisation data were collected between 1 December, 2016 and 31 January, 2019. We report successful use of this technology in both diagnostic and interventional cases such as placing stents and percutaneous valves, performing angioplasties, occlusion of collaterals, and guidance for lymphatic interventions. In addition, radiation exposure was markedly decreased when comparing our 10-15-year-old coarctation of the aorta stent angioplasty cohort to cases without the use of overlay technology and the most recently published national radiation dose benchmarks. No complications were encountered due to the application of overlay technology. 3D CT or MRI overlay for CHD intervention with rapid registration is feasible and aids decisions regarding access and planned angiographic angles. Operators found intraprocedural overlay fusion registration using placed vessel guidewires to be more accurate than attempts using bony structures.

Congenital heart disease in adults (when kids grow up) pediatric geriatric anesthesia.

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.

Interventional Cardiovascular Magnetic Resonance Imaging (iCMR) in an Adolescent with Pulmonary Hypertension.

The interventional cardiac magnetic resonance imaging (iCMR) catheterization procedure is feasible and safe for children and adults with pulmonary hypertension and congenital heart defects (CHD). With iCMR, the calculation of pulmonary vascular resistance (PVR) in children with complex CHD with multilevel shunt lesions is accurate. In this paper, we describe the role of the MRI-guided right-sided cardiac catheterization procedure to accurately estimate PVR in the setting of multiple shunt lesions (ventricular septal defect and patent ductus arteriosus) and to address the clinical question of operability in an adolescent with trisomy 21 and severe pulmonary hypertension.

Consensus Statement by the Congenital Cardiac Anesthesia Society: Milestones for the Pediatric Cardiac Anesthesia Fellowship.

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.

Rationalising the use of cardiac catheterisation before Glenn completion.

Previous studies have shown that cardiac MRI can be used to evaluate the suitability for infants to undergo the Glenn operation after having undergone the Norwood procedure. We sought to analyse our institutional data retrospectively to identify whether such a policy would be advisable in the current era. We reviewed patients who underwent the Norwood procedure between 1 January, 2006 and 1 January, 2016. All patients undergoing evaluation for the Glenn procedure received clinical evaluation, echocardiography, and cardiac catheterisation. A total of 179 patients were identified; 154 patients (86%) survived to undergo cardiac catheterisation as part of evaluation for the Glenn, and all who were evaluated did not eventually receive the Glenn. Using said algorithm, if cardiac MRI or CT were to be used to rationalise the use of catheterisation, 26 of 154 patients would have required catheterisation after cross-sectional imaging identified vascular obstruction; 83 of 154 patients would have received cross-sectional imaging only; and 45 of 154 would have had catheterisation only. All cases that required intervention, excluding aortopulmonary collaterals, and all cases that were not suitable to progress would have been correctly identified using clinical and echocardiographic criteria in addition to cardiac cross-sectional imaging to rationalise the use of catheterisation. Thus, in cases with acceptable clinical, echocardiographic, and angiographic findings, the additional haemodynamic information from catheterisation is rarely of use for decision-making, and interventions can largely be predicted by angiographic imaging modalities.

The Congenital Cardiac Anesthesia Society: An Update on the First Twelve Years.

The Congenital Cardiac Anesthesia Society is an international body instituted for collaboration among parties with interest in the perioepartive care of patients with congenial heart disease. This report is a review and update on the first 12 years of this society.

Cyanotic congenital heart disease (CCHD): focus on hypoxemia, secondary erythrocytosis, and coagulation alterations.

Children with cyanotic congenital heart disease (CCHD) have complex alterations in their whole blood composition and coagulation profile due to long-standing hypoxemia. Secondary erythrocytosis is an associated physiological response intended to increase circulating red blood cells and oxygen carrying capacity. However, this response is frequently offset by an increase in whole blood viscosity that paradoxically reduces blood flow and tissue perfusion. In addition, the accompanying reduction in plasma volume leads to significant deficiencies in multiple coagulation proteins including platelets, fibrinogen and other clotting factors. On the one hand, these patients may suffer from severe hyperviscosity and subclinical 'sludging' in the peripheral vasculature with an increased risk of thrombosis. On the other hand, they are at an increased risk for postoperative hemorrhage due to a complex derangement in their hemostatic profile. Anesthesiologists caring for children with CCHD and secondary erythrocytosis need to understand the pathophysiology of these alterations and be aware of available strategies that lessen the risk of bleeding and/or thrombosis. The aim of this review is to provide an updated analysis of the systemic effects of long-standing hypoxemia in children with primary congenital heart disease with a specific focus on secondary erythrocytosis and hemostasis.

Impact of protamine dose on activated clotting time and thromboelastography in infants and small children undergoing cardiopulmonary bypass.

OBJECTIVES: To study the effect of two protamine-dosing strategies on activated clotting time (ACT) and thromboelastography (TEG). BACKGROUND: Protamine dosage based on neutralizing heparin present in the combined estimated blood volumes (EBVs) of the patient and cardiopulmonary bypass (CPB) pump may result in excess protamine and contributes toward a coagulopathy that can be detected by ACT and TEG in pediatric patients. METHODS: A total of 100 pediatric patients 1 month to ≤5 years of age undergoing CPB were included in this retrospective before/after design study. Combined-EBV group consisted of 50 consecutive patients whose protamine dose was calculated to neutralize heparin in the combined EBVs of the patient and the pump. Pt-EBV group consisted of the next 50 consecutive patients whose protamine dose was calculated to neutralize heparin in the patient's EBV. RESULTS: Baseline and postprotamine ACTs were similar between groups. Postprotamine heparin assay (Hepcon) showed the absence of residual heparin in both groups. Postprotamine kaolin-heparinase TEG showed that R was prolonged by 7.5 min in the Combined-EBV group compared with the Pt-EBV group (mean R of 20.17 vs. 12.4 min, respectively, P < 0.001). Increasing doses of protamine were associated with a corresponding, but nonlinear increase in R. There was no significant difference in the changes for K, alpha, and MA between the groups. CONCLUSION: Automated protamine titration with a protamine dosage based on Pt-EBV can adequately neutralize heparin as assessed by ACT while minimizing prolonging clot initiation time as measured by TEG.

Transesophageal Doppler measurement of renal arterial blood flow velocities and indices in children.

BACKGROUND: Doppler-derived renal blood flow indices have been used to assess renal pathologies. However, transesophageal ultrasonography (TEE) has not been previously used to assess these renal variables in pediatric patients. In this study, we (a) assessed whether TEE allows adequate visualization of the renal parenchyma and renal artery, and (b) evaluated the concordance of TEE Doppler-derived renal blood flow measurements/indices compared with a standard transabdominal renal ultrasound (TAU) in children. METHODS: This prospective cohort study enrolled 28 healthy children between the ages of 1 and 17 years without known renal dysfunction who were undergoing atrial septal defect device closure in the cardiac catheterization laboratory. TEE was used to obtain Doppler renal artery blood velocities (peak systolic velocity, end-diastolic velocity, mean diastolic velocity, resistive index, and pulsatility index), and these values were compared with measurements obtained by TAU. Concordance correlation coefficient (CCC) was used to determine clinically significant agreement between the 2 methods. The Bland-Altman plots were used to determine whether these 2 methods agree sufficiently to be used interchangeably. Statistical significance was accepted at P ≤ 0.05. RESULTS: Obtaining 2-dimensional images of kidney parenchyma and Doppler-derived measurements using TEE in children is feasible. There was statistically significant agreement between the 2 methods for all measurements. The CCC between the 2 imaging techniques was 0.91 for the pulsatility index and 0.66 for the resistive index. These coefficients were sensitive to outliers. When the highest and lowest data points were removed from the analysis, the CCC between the 2 imaging techniques was 0.62 for the pulsatility index and 0.50 for the resistive index. The 95% confidence interval (CI) for pulsatility index was 0.35 to 0.98 and for resistive index was 0.21 to 0.89. The Bland-Altman plots indicate good agreement between the 2 methods; for the pulsatility index, the limits of agreement were -0.80 to 0.53. The correlation of the size of the measurement and the mean difference in methods (-0.14; 95% CI = -0.28, 0.01) was not statistically significant (r = 0.31, P = 0.17). For the resistive index, the limits of agreement were -0.22 to 0.12. The correlation of the size of the measurement and the mean difference in methods (-0.05; 95% CI = -0.09, -0.01) was not statistically significant (r = 0.10, P = 0.65). CONCLUSION: This study confirms the feasibility of obtaining 2-dimensional images of kidney parenchyma and Doppler-derived measurements using TEE in children. Angle-independent TEE Doppler-derived indices show significant concordance with those derived by TAU. Further studies are required to assess whether this correlation holds true in the presence of renal pathology. This technique has the potential to help modulate intraoperative interventions based on their impact on renal variables and may prove helpful in the perioperative period for children at risk of acute kidney injury.

Anesthetic Considerations in Pediatric Patients With Acute Decompensated Heart Failure.

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.

The Anesthesiologist's Perspective and Experience in Global Congenital Cardiac Surgery: Results of a Survey of the Congenital Cardiac Anesthesia Society Membership.

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.

Use of institutional criteria for transcatheter device closure of Fontan fenestration - Midterm outcomes.

BACKGROUND: There are no established criteria to decide suitability for Fontan fenestration closure. Our institution has the following criteria: an unobstructed Fontan pathway with no significant decompressing venovenous collaterals, baseline Fontan pressure ≤15 mmHg, baseline cardiac index ≥2 L/min/m2, and a decrease in cardiac index ≤20% with test occlusion of the fenestration. OBJECTIVE: The objective of the study was to review midterm outcomes following device closure of Fontan fenestration using institutional criteria. MATERIALS AND METHODS: A retrospective review was performed of patients who underwent catheterization with prior fenestrated Fontan procedure between May 2005 and January 2015. Patients were classified as those who underwent successful closure (A), had closure deferred due to failure to meet criteria (B), or were not referred for closure (C). RESULTS: There were 42 patients in Group A, 10 in Group B, and 150 in Group C. The mean Fontan pressure increased from 13.1 ± 2.1 to 14.5 ± 2.1mmHg in Group A and 14.6 ± 1.5 to 15.7 ± 2.2 mmHg in Group B (P = not significant). With test occlusion, cardiac index fell by 18.12% ± 15.68% in Group A and 33.75% ± 14.98% in Group B (P = 0.019). At a median of 46 month follow-up, oxygen saturation increased significantly from 85.15% ± 6.29% at baseline to 94.6% ± 4.43% (P < 0.001) in Group A but with no statistically significant difference in the rates of plastic bronchitis, protein-losing enteropathy, stroke, or heart transplantation between the three groups. CONCLUSIONS: Using institutional criteria, transcatheter device closure of Fontan fenestration was followed by significant increase in oxygen saturations and no statistically significant difference in morbidity or mortality between closure and nonclosure groups.