Deep Sedation in Pediatric Patients With Single Ventricle Physiology Outside of the Operating Room.

Background: Advancements in palliative surgery of patients with single ventricle physiology have led to an increase in the need for deep sedation protocols for painful procedures. However, positive pressure ventilation during anesthesia can result in unfavorable cardiopulmonary interactions. This patient population may benefit from sedation from these painful procedures. Methods: This study aims to demonstrate the safety and efficacy of deep sedation by pediatric intensivists outside the operating room for children with single ventricle physiology. This is a single-center, retrospective chart review on consecutive pediatric patients with single ventricle physiology who received deep sedation performed by pediatric intensivists between 2013 and 2020. Results: Thirty-three sedations were performed on 27 unique patients. The median age was 3.7 years (25th%-75th%: 2.1-15.6). The majority of the sedations, 88% (29/33), were done on children with Fontan physiology and 12% (4/33) were status-post superior cavopulmonary anastomosis. The primary cardiac defect was hypoplastic left heart in 63% (17/27) of all sedation procedures. There were 24 chest tube placements and 9 cardioversions. Ketamine alone [median dose 1.5 mg/kg (range 0.8-3.7)], ketamine [median dose 1 mg/kg (range 0.1-2.1)] with propofol [median dose 2.3 mg/kg (range 0.7-3.8)], and ketamine [median dose 1.5 mg/kg (range 0.4-3.0)] with morphine [median dose 0.06 mg/kg (range 0.03-0.20)] were the most common sedation regimens used. Adverse events (AEs) occurred in 4 patients (15%), three of which were transient AEs. All sedation encounters were successfully completed. Conclusion: Procedural deep sedation can be safely and effectively administered to single ventricle patients by intensivist-led sedation teams in selective case.

Bivalirudin or heparin for systemic anticoagulation during pediatric extracorporeal membrane oxygenation: Multicenter retrospective study.

BACKGROUND: The objective of this study is to evaluate the outcomes of unfractionated heparin (UFH) compared to bivalirudin anticoagulation in pediatric ExtraCorporeal Membrane Oxygenation (ECMO). METHODS: A multicenter retrospective study, that included pediatric patients <18 years of age, who were supported on ECMO between June 2017 and May 2020. Patients treated with UFH were matched 2:1 by age and type of ECMO support to the bivalirudin group. RESULTS: The bivalirudin group (75 patients) were matched to 150 patients treated with UFH. Baseline characteristics and comorbidities of the two groups were similar. Veno-Arterial ECMO was the most common mode (141/225 [63 %]) followed by extracorporeal cardiopulmonary resuscitation (48/225 [21 %]). Bivalirudin treatment was associated with lower odds of bleeding events (aOR 0.23, 95%CI 0.12-0.45, p < 0.001) and lower odds of thrombotic events (aOR 0.48, 95%CI 0.23-0.98, p = 0.045). Patients who received bivalirudin had lesser odds for transfusion with fresh frozen plasma, and platelets (aOR 0.26, CI 0.12-0.57, p ≤0.001 and aOR 0.28, CI 0.15-0.53, p < 0.001, respectively). After adjusting for the type of ECMO support and adjusting for age, bivalirudin was associated with a decrease in hospital mortality by 50 % compared to the UFH group (aOR 0.50, 95%CI 0.27-0.93, p = 0.028). Similarly, for neurological disability at time of discharge, bivalirudin was associated with higher odds of intact neurological outcomes compared to UFH (OR 1.99 [95%CI 1.13-3.51], p = 0.017). CONCLUSIONS: This study demonstrated that effective anticoagulation can be achieved with bivalirudin, which was associated with lesser odds of bleeding events and utilization of blood products. Bivalirudin, in comparison with UFH, was associated with greater odds of hospital survival and intact neurological function at the time of discharge. A prospective randomized trial is required to validate the results of this study.

Safety and Efficacy of Propofol- and Ketamine-Based Procedural Sedation Regimen in Pediatric Patients During Burn Repetitive Dressing Change: 10 Years Single Center Experience.

It is crucial to provide an adequate level of sedation and analgesia during burn dressing changes in the pediatric population due to the amount of pain and anxiety patients experience during the procedure. To evaluate the safety and efficacy of an intensivist-based deep sedation regimen using a combination of propofol and ketamine to provide procedural sedation to pediatric burn patients. This is a retrospective chart review of pediatric patients who underwent inpatient burn wound dressing changes from 2011 through 2021. Demographic and clinical data, including age, length of the procedure, recovery time, medication doses, and adverse events, were collected. A total of 104 patients aged between 45 and 135 months with a median total burn body surface area (TBSA) of 11.5 percent (interquartile range [IQR] 4.0, 25.0) underwent 378 procedural sedation encounters with propofol- and ketamine-based sedation. The median total dose of propofol was 7 mg/kg (IRQ 5.3, 9.2). Of these sedations, 64 (17 percent) had minor adverse events, of which 50 (13 percent) were transient hypoxemia, 12 (3 percent) were upper airway obstruction, and 2 (0.5 percent) were hypotension. There were no serious adverse events. Hypoxemia was not related to age, weight, gender, burn TBSA, or total dose of propofol. There were 35 (33.6 percent) patients who had repetitive sedation encounters with no statistically significant changes in propofol dose or adverse events with the repeated encounters. Children can be effectively sedated for repetitive inpatient burn dressing changes. Given the high-risk patient populations, this procedure should be performed under the vigilance of highly trained providers.

Evaluation of Bivalirudin Dosing in Pediatric Extracorporeal Membrane Oxygenation with Renal Insufficiency or Renal Replacement Therapy.

Bivalirudin offers several important advantages of relevance to the management of extracorporeal membrane oxygenation (ECMO) patients. This multicenter retrospective analysis evaluated the bivalirudin dosing in pediatric ECMO and correlated these doses with the severity of renal dysfunction. A total of 75 patients were included in this analyses: estimated glomerular filtration rate (eGFR) > 60 ml/min/1.73 m 2 (n = 29), eGFR 30-60 (n = 18), eGFR < 30 (n = 28), and of those 23 were on renal replacement therapy (RRT). The initial bivalirudin dose used to reach therapeutic anticoagulation in patients with eGFR > 60 was significantly higher than the dose required in those with renal impairment (0.25 mg/kg/hr in patients with eGFR > 60 and 0.19 mg/kg/hr in patients on RRT, 0.18 mg/kg/hr in patients with eGFR 30-60 and 0.13 mg/kg/hr in patients with eGFR < 30 with no RRT). Progressive dose escalations (two to threefold increase) were required to maintain therapeutic range over the initial 4 days of ECMO that coincided with improving renal creatinine clearance during that same time period. Establishing an initial starting dose of bivalirudin contingent upon eGFR is essential for the rapid achievement of target anticoagulation intensity. Further dose adjustments guided by laboratory monitoring is necessary given the dynamic changes in creatinine clearance following ECMO initiation.

Prophylactic Enoxaparin Against Catheter-Associated Thrombosis in Postoperative Cardiac Children: An Interrupted Time Series Analysis.

OBJECTIVES: The effectiveness of pharmacologic prophylaxis against catheter-associated thrombosis in children is unclear. We evaluated the compliance and outcomes associated with a prophylactic enoxaparin protocol in postoperative cardiac children. DESIGN: The protocol was implemented as a quality improvement initiative and then analyzed using interrupted time series method. Data collected from November 2014 to December 2018 were divided into preprotocol (period 1), protocol implementation (period 2), and protocol revision (period 3). SETTING: A 12-bed academic pediatric cardiac ICU. PATIENTS: Children less than or equal to 18 years old with congenital heart disease admitted postoperatively with central venous catheter in situ for greater than or equal to 1 day. INTERVENTIONS: Before 2016, prophylactic enoxaparin was administered according to physician preference. In January 2016, an enoxaparin protocol was implemented with a goal anti-Xa range of 0.25-0.49 international units/mL. Protocol was revised in February 2017 to increase the starting dose by 25% for infants less than 1 year old. MEASUREMENTS AND MAIN RESULTS: We analyzed 780 hospitalizations from 636 children. Median percentage of catheter-days on prophylactic enoxaparin was 33% (interquartile range [IQR], 23-47%), 42% (IQR, 30-51%), and 38% (IQR, 35-52%) in periods 1-3, respectively. Percentage of catheter-days on enoxaparin showed immediate increase of 90% (95% CI, 17-210%) between periods 1 and 2 and sustained increase of 2% (95% CI, 0.3-4%) between periods 2 and 3. Median rates of thrombosis per 1,000 catheter-days were 5.8 (IQR, 0-9.3), 3.8 (IQR, 0-12), and 0 (IQR, 0-5.3) in periods 1-3, respectively. Rate of thrombosis showed immediate decrease of 67% (95% CI, 12-87%) between periods 1 and 2 and sustained decrease of 11% (95% CI, 2-18%) between periods 1 and 3. CONCLUSIONS: The temporal association between increase in percentage of catheter-days on enoxaparin and decrease in rate of thrombosis suggests the effectiveness of prophylactic enoxaparin.

Measures of anticoagulation and coagulopathy in pediatric cardiac extracorporeal membrane oxygenation patients.

INTRODUCTION: Pediatric cardiac Extracorporeal Membrane Oxygenation (ECMO) is effective, however, bleeding and clotting issues continue to cause significant morbidity and mortality. The objective of this study was to assess the correlation between measures of anticoagulation, the heparin dose in pediatric cardiac ECMO patients as well as to assess covert coagulopathy as measured by thromboelastography (TEG). METHODS: Retrospective study of cardiac ECMO patients in a large, academic referral center using anticoagulation data during the ECMO support. RESULTS: Five hundred and eighty-four sets of anticoagulation tests and 343 TEG from 100 patients with median age of 26 days were reviewed. ECMO was post-surgical for congenital heart disease in 94% with resuscitation (ECPR) in 38% of the cases. Mean duration of support was 6.3 days. Overall survival to discharge was 35%. There was low but statistically significant correlation between individual anticoagulation measures and low correlation between Anti-Xa levels and heparin dose. There was no correlation between PTT and heparin dose. 343 TEG with Heparinase were reviewed to assess covert coagulopathy which was present in 25% of these. The coagulopathy noted was pro-hemorrhagic in almost all of the cases with high values of reaction time and kinetics and low values for angle and maximum amplitude. CONCLUSION: Coagulation monitoring on ECMO may benefit from addition of Heparinase TEG to diagnose covert coagulopathy which can contribute to significant hemorrhagic complications. There is a need for a prospective, thromboelastography guided intervention trial to reduce coagulopathy related morbidity and mortality in ECMO.

Adjuvant lidocaine to a propofol-ketamine-based sedation regimen for bone marrow aspirates and biopsy in the pediatric population.

Pediatric patients with hematological malignancies repeatedly undergo painful bone marrow aspirates and biopsies (BMABs) in routine care. No standard sedation regimen has been established. This study evaluated the addition of injected local lidocaine to a propofol-ketamine sedation for BMAB and its effects on propofol dosing, safety, and efficacy. A retrospective analysis of children undergoing BMAB with propofol-ketamine with (PK+L) and without (PK-only) the injection of local lidocaine. Patients were matched through propensity probability scores. To measure efficacy, dosing, procedure length, and recovery time were evaluated. To assess safety, adverse and serious events were recorded. As an indirect measurement of analgesia, changes in heart rate and blood pressure were analyzed. Of the 420 encounters included, 188 matched pairs (376 patients) were analyzed. Patient demographics were comparable. The median dose of propofol was not significantly different between both groups. The incidence of adverse events was similar. There were no significant differences in the changes in heart rate and blood pressure with sedation between groups.Conclusion: This study suggests that the addition of local lidocaine injection to a propofol-ketamine sedation for BMAB pediatric patients does not affect the propofol dose, safety, or efficacy properties of the regimen. What is Known: •Although propofol is commonly used, there is no standard sedation regimen for pediatric patients undergoing bone marrow aspiration and biopsy. •Local lidocaine is used in analgesia in the adults undergoing the same procedure. What is New: •Local lidocaine adjuvant to propofol-ketamine sedation does not affect propofol dosing, the safety of efficacy properties of the regimen in the pediatric population.

Analysis of factors associated with prolonged post-operative course after surgical repair of aortic coarctation.

OBJECTIVE: We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation. METHODS: We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay. RESULTS: We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay. CONCLUSIONS: In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.

Upper body peripherally inserted central catheter in pediatric single ventricle patients.

BACKGROUND: There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement. This complication is more serious among patients with single ventricle physiology, as it might preclude them from undergoing further life-sustaining palliative surgery. AIM: To describe complications associated with the use of upper extremity percutaneous intravenous central catheters (PICCs) in children with single ventricle physiology. METHODS: A single institution retrospective review of univentricular patients who underwent superior cavopulmonary anastomoses as their stage 2 palliation procedure from January 2014 until December 2018 and had upper body PICCs placed at any point prior to this procedure. Clinical data including ultrasonography, cardiac catheterization, echocardiogram reports and patient notes were used to determine the presence of thrombus or stenosis of the upper extremity and cervical vessels. Data regarding the presence and duration of upper extremity PICCs and upper extremity central venous catheter (CVC), and use of anticoagulation were recorded. RESULTS: Seventy-six patients underwent superior cavopulmonary anastomoses, of which 56 (73%) had an upper extremity PICC at some point prior to this procedure. Median duration of PICC usage was 24 d (25%, 75%: 12, 39). Seventeen patients (30%) with PICCs also had internal jugular or subclavian central venous catheters (CVCs) in place at some point prior to their superior cavopulmonary anastomoses, median duration 10 d (25%, 75%: 8, 14). Thrombus was detected in association with 2 of the 56 PICCs (4%) and 3 of the 17 CVCs (18%). All five patients were placed on therapeutic dose of low molecular weight heparin at the time of thrombus detection and subsequent cardiac catheterization demonstrated resolution in three of the five patients. No patients developed clinically significant venous stenosis. CONCLUSION: Use of upper extremity PICCs in patients with single ventricle physiology prior to super cavopulmonary anastomosis is associated with a low rate of catheter-associated thrombosis.

Improving Adherence to a Pediatric Advanced Life Support Supraventricular Tachycardia Algorithm in Community Emergency Departments Following in Situ Simulation.

BACKGROUND: Recognition and management of pediatric dysrhythmias is challenging for community emergency department (CED) providers, given their infrequent exposure to these cases. METHOD: A prospective, interventional study measured adherence of CEDs to pediatric supraventricular tachycardia (SVT) algorithm pre- and postimplementation of an in situ simulation-based collaborative program. CED teams' adherence was scored using a composite adherence score (CAS) based on the number of actions scored correctly on the performance checklist. RESULTS: A total of 74 multiprofessional teams from nine CEDs participated in simulated sessions. Of 367 participants, 12.3% were physicians, 62.1% were RNs, and 25.6% were other providers. The mean CAS improved from 57% to 71%. The ability to identify an SVT rhythm, stable versus unstable SVT, and the correct performance of synchronized cardioversion significantly improved. CONCLUSION: This study demonstrated improvement in overall adherence of CEDs to pediatric SVT algorithm following a collaborative program in simulated setting. This approach could be adapted to improve the quality of care provided to children. [J Contin Educ Nurs. 2019;50(9):404-410.].

Minimization of Surgical Site Infections in Patients With Delayed Sternal Closure After Pediatric Cardiac Surgery.

BACKGROUND: Delayed sternal closure (DSC) following pediatric cardiac surgery is commonly implemented at many centers. Infectious complications occur in 18.7% of these patients based on recent multicenter data. We aimed to describe our experience with DSC, hypothesizing that our practices surrounding the implementation and maintenance of the open sternum during DSC minimize the risk of infectious complications. METHODS: We reviewed patients less than 365 days who underwent DSC between 2012 and 2016 at our institution. Infectious complications as defined by the Society of Thoracic Surgeons Congenital Heart Surgery Database were recorded. Patients with and without infectious complications were compared using Wilcoxon rank sum tests or Fisher exact tests as appropriate. RESULTS: We identified 165 patients less than 365 days old who underwent DSC, 135 (82%) of whom had their skin closed over their open sternum. Median duration of open sternum was 3 days (range: 1-32 days). Infectious complications occurred in 15 (9.1%) patients-13 developed clinical sepsis with positive blood cultures, one patient developed ventilator-associated pneumonia, and one patient developed wound infection (0.6%). No cases of mediastinitis occurred. No statistical differences in characteristics between patients with and without infectious complications could be identified. CONCLUSION: Infectious complications after DSC at our institution were notably less than reported in recent literature, primarily due to minimization of surgical site infections. Practices described in the article, including closing skin over the open sternum whenever possible, could potentially aid other institutions aiming to reduce infectious complications associated with DSC.

Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy.

We describe two pediatric patients with Duchenne muscular dystrophy that presented with acute neurologic deterioration and hypoxic respiratory failure requiring mechanical ventilation. These cases fulfill the clinical criteria for Fat Embolism Syndrome. Early recognition and aggressive supportive therapy with mechanical ventilation, right ventricular afterload reduction, and blood transfusion led to survival without any residual effects from the event. Fat Embolism Syndrome needs to be considered early in the course of patients with Duchenne muscular dystrophy who present with respiratory and neurological symptoms.

Management of Complications Caused by a Massive Left Ventricle Tumor in a Neonate.

We report the case of a neonate born with a giant fibroma occupying the entirety of her left ventricle. Owing to the extensive resection, her postoperative course was complicated by severely diminished left ventricular function and complete heart block necessitating extracorporeal support. Ultimately, cardiac resynchronization therapy was used, after which the infant's ventricular function gradually improved and she was successfully discharged to home.

Utility and correlation of known anticoagulation parameters in the management of pediatric ventricular assist devices.

AIM: To assess utility and correlation of known anticoagulation parameters in the management of pediatric ventricular assist device (VAD). METHODS: Retrospective study of pediatric patients supported with a Berlin EXCOR VAD at a single pediatric tertiary care center during a single year. RESULTS: We demonstrated associations between activated thromboplastin time (aPTT) and R-thromboelastography (R-TEG) values (rs = 0.65, P < 0.001) and between anti-Xa assay and R-TEG values (rs = 0.54, P < 0.001). The strongest correlation was seen between aPTT and anti-Xa assays (rs = 0.71, P < 0.001). There was also a statistically significant correlation between platelet counts and the maximum amplitude of TEG (rs = 0.71, P < 0.001). Importantly, there was no association between dose of unfractionated heparin and either measure of anticoagulation (aPTT, anti-Xa or R-TEG value). CONCLUSION: This study suggests that while there is strong correlation between aPTT, anti-Xa assay and R-TEG values for patients requiring VAD support, there is a lack of relevant correlation between heparin dose and degree of effect. This raises concern as various guidelines continue to recommend using these parameters to titrate heparin therapy.

Hypoplastic left heart syndrome: from comfort care to long-term survival.

The management of hypoplastic left heart syndrome (HLHS) has changed substantially over the past four decades. In the 1970s, children with HLHS could only be provided with supportive care. As a result, most of these unfortunate children died within the neonatal period. The advent of the Norwood procedure in the early 1980s has changed the prognosis for these children, and the majority now undergoing a series of three surgical stages that can support survival beyond the neonatal period and into early adulthood. This review will focus on the Norwood procedure and the other important innovations of the last half century that have improved our outlook toward children born with HLHS.

Recognition and successful treatment of priapism and suspected black widow spider bite with antivenin.

Priapism, although uncommon in preadolescent children, is considered a true emergency. Envenomation by a black widow spider bite has been reported to induce priapism as a manifestation of its toxicity. Early recognition and timely administration of antivenin have been reported to be effective in relieving priapism. Clinicians who care for children need to be aware of this unusual presentation. The diagnosis is traditionally from either direct observation of a spider bite or capture of a spider. We report a case of a previously healthy 2-year-old boy who presented with severe irritability, leg cramps, and stomach ache. The diagnosis of a likely black widow spider envenomation was made on the basis of clinical suspicion and suggestive physical findings in absence of demonstrated exposure. This case highlights the importance of early recognition and successful resolution of symptoms with administration of antivenin and supportive care.