From Surgical to Total Transcatheter Stage I Palliation: Exploring Evidence and Perspectives.

Neonates with single ventricle physiology and ductal-dependent systemic circulation, such as those with hypoplastic left heart syndrome, undergo palliation in the first days of life. Over the past decades, variations on the traditional Stage 1 palliation, also known as Norwood operation, have emerged. These include the hybrid palliation and the total transcatheter approach. Here, we review the current evidence and data on different Stage 1 approaches, with a focus on their advantages, challenges, and future perspectives. Overall, although controversy remains regarding the superiority or inferiority of one approach to another, outcomes after the Norwood and the hybrid palliation have improved over time. However, both procedures still represent high-risk approaches that entail exposure to sternotomy, surgery, and potential cardiopulmonary bypass. The total transcatheter Stage 1 palliation spares patients the surgical and cardiopulmonary bypass insults and has proven to be an effective strategy to bridge even high-risk infants to a later palliative surgery, complete repair, or transplant. As the most recently proposed approach, data are still limited but promising. Future studies will be needed to better define the advantages, challenges, outcomes, and overall potential of this novel approach.

Administration of Milrinone Following Tetralogy of Fallot Repair Increases Postoperative Volume Administration Without Improving Cardiac Output.

BACKGROUND: Phosphodiesterase inhibitors are known to relieve symptoms in the setting of heart failure, although their effects in restrictive ventricular physiology have been poorly characterized. We explored the association between the use of milrinone and volume administration during the first 72 hours following surgical repair of tetralogy of Fallot (TOF). METHODS: We reviewed all cases of primary surgical repair of TOF with pulmonary stenosis or atresia at Boston Children's Hospital between 2011 and 2020. To adjust for baseline differences between patients who did and did not receive milrinone, we matched patients with similar propensity scores in a 1:1 ratio (use of milrinone versus not). We then compared the need for volume administration during the first 72 hours postoperatively, vital signs, and measures of cardiac output between the matched cohorts. Additionally, in the group of patients receiving milrinone, linear regression modeling was used to explore the relationship between total dose of milrinone and total volume administration. RESULTS: Among 351 included patients, 134 received perioperative milrinone. A total of 212 patients (106 per group) were matched based on anatomic and surgical risks using a propensity score. After propensity matching, compared with nonmilrinone-treated patients, milrinone-treated patients were given postoperative volume more frequently (66% vs 52%; difference 14% [95% confidence interval, CI, 1%-27%]; P = .036). Milrinone-treated patients had a slower recovery of tachycardia during the first 12 hours (difference in slope 0.30 [95% CI, 0.14-0.47] beats per minute [BPM]/h; P < .001), and the intergroup difference peaked at 12 hours postoperatively (8 [95% CI, 5-12] BPM). Milrinone administration was not associated with improved cardiac output, including arteriovenous oxyhemoglobin saturation difference. In propensity-matched patients receiving milrinone, the total volume administered during the first 72 postoperative hours was significantly associated with the cumulative dose of postoperative milrinone ( r = 0.20; 95% CI, 0.01-0.38; P = .036). Based on the slope of the regression line, for every 1000 µg/kg of milrinone (equivalent to ~0.25 µg/kg/min for 72 hours) administered in the first 72 postoperative hours, an estimated 11.0 (95% CI, 0.6-21.4) mL/kg additional volume was administered. CONCLUSIONS: The use of milrinone within the first 72 hours following TOF repair is associated with more frequent administration of volume, a positive association between a higher total dose of postoperative milrinone and the amount of postoperative volume administered, a higher heart rate, and a lower blood pressure, but is not associated with improved cardiac output.

Analgesia and sedation in critically ill pediatric patients: an update from the recent guidelines and point of view.

In the last decades, the advancement of knowledge in analgesia and sedation for critically ill pediatric patients has been conspicuous and relevant. Many recommendations have changed to ensure patients' comfort during their intensive care unit (ICU) stay and prevent and treat sedation-related complications, as well as improve functional recovery and clinical outcomes. The key aspects of the analgosedation management in pediatrics have been recently reviewed in two consensus-based documents. However, there remains a lot to be researched and understood. With this narrative review and authors' point of view, we aimed to summarize the new insights presented in these two documents to facilitate their interpretation and application in clinical practice, as well as to outline research priorities in the field.    Conclusion: With this narrative review and authors' point of view, we aimed to summarize the new insights presented in these two documents to facilitate their interpretation and application in clinical practice, as well as to outline research priorities in the field. What is Known: • Critically ill pediatric patients receiving intensive care required analgesia and sedation to attenuate painful and stressful stimuli. •Optimal management of analgosedation is a challenge often burdened with complications such as tolerance, iatrogenic withdrawal syndrome, delirium, and possible adverse outcomes. What is New: •The new insights on the analgosedation treatment for critically ill pediatric patients delineated in the recent guidelines are summarized to identify strategies for changes in clinical practice. •Research gaps and potential for quality improvement projects are also highlighted.

Cardiac arrest and cardiopulmonary resuscitation in pediatric patients with cardiac disease: a narrative review.

Children with cardiac disease are at a higher risk of cardiac arrest as compared to healthy children. Delivering adequate cardiopulmonary resuscitation (CPR) can be challenging due to anatomic characteristics, risk profiles, and physiologies. We aimed to review the physiological aspects of resuscitation in different cardiac physiologies, summarize the current recommendations, provide un update of current literature, and highlight knowledge gaps to guide research efforts. We specifically reviewed current knowledge on resuscitation strategies for high-risk categories of patients including patients with single-ventricle physiology, right-sided lesions, right ventricle restrictive physiology, left-sided lesions, myocarditis, cardiomyopathy, pulmonary arterial hypertension, and arrhythmias. Cardiac arrest occurs in about 1% of hospitalized children with cardiac disease, and in 5% of those admitted to an intensive care unit. Mortality after cardiac arrest in this population remains high, ranging from 30 to 65%. The neurologic outcome varies widely among studies, with a favorable neurologic outcome at discharge observed in 64%-95% of the survivors. Risk factors for cardiac arrest and associated mortality include younger age, lower weight, prematurity, genetic syndrome, single-ventricle physiology, arrhythmias, pulmonary arterial hypertension, comorbidities, mechanical ventilation preceding cardiac arrest, surgical complexity, higher vasoactive-inotropic score, and factors related to resources and institutional characteristics. Recent data suggest that Extracorporeal membrane oxygenation CPR (ECPR) may be a valid strategy in centers with expertise. Overall, knowledge on resuscitation strategies based on physiology remains limited, with a crucial need for further research in this field. Collaborative and interprofessional studies are highly needed to improve care and outcomes for this high-risk population. What is Known: • Children with cardiac disease are at high risk of cardiac arrest, and cardiopulmonary resuscitation may be challenging due to unique characteristics and different physiologies. • Mortality after cardiac arrest remains high and neurologic outcomes suboptimal. What is New: • We reviewed the unique resuscitation challenges, current knowledge, and recommendations for different cardiac physiologies. • We highlighted knowledge gaps to guide research efforts aimed to improve care and outcomes in this high-risk population.

MIS-C across three SARS-CoV-2 variants: Changes in COVID-19 testing and clinical characteristics in a cohort of U.S. children.

As new variants of SARS-Co-V 2 have emerged over time and Omicron sub-variants have become dominant, the severity of illness from COVID-19 has declined despite greater transmissibility. There are fewer data on how the history, diagnosis, and clinical characteristics of multisystem inflammatory syndrome in children (MIS-C) have changed with evolution in SARS-CoV-2 variants. We conducted a retrospective cohort study of patients hospitalized with MIS-C between April 2020 and July 2022 in a tertiary referral center. Patients were sorted into Alpha, Delta, and Omicron variant cohorts by date of admission and using national and regional data on variant prevalence. Among 108 patients with MIS-C, significantly more patients had a documented history of COVID-19 in the two months before MIS-C during Omicron (74%) than during Alpha (42%) (p = 0.03). Platelet count and absolute lymphocyte count were lowest during Omicron, without significant differences in other laboratory tests. However, markers of clinical severity, including percentage with ICU admission, length of ICU stay, use of inotropes, or left ventricular dysfunction, did not differ across variants. This study is limited by its small, single-center case series design and by classification of patients into era of variant by admission date rather than genomic testing of SARS- CoV-2 samples.     Conclusion: Antecedent COVID-19 was more often documented in the Omicron than Alpha or Delta eras, but clinical severity of MIS-C was similar across variant eras. What is Known: • There has been a decrease in incidence of MIS-C in children despite widespread infection with new variants of COVID-19. • Data has varied on if the severity of MIS-C has changed over time across different variant infections. What is New: • MIS-C patients were significantly more likely to report a known prior infection with SARS-CoV-2 during Omicron than during Alpha. • There was no difference in severity of MIS-C between the Alpha, Delta, and Omicron cohorts in our patient population.

Nucleated Red Blood Cells Are Predictive of In-Hospital Mortality for Pediatric Patients.

PURPOSE: We sought to establish whether nucleated red blood cells (NRBCs) are predictive of disposition, morbidity, and mortality for pediatric patients presenting to the emergency department (ED). METHODS: A single-center retrospective cohort study examining all ED encounters from patients aged younger than 19 years between January 2016 and March 2020, during which a complete blood count was obtained. Univariate analysis and multivariable logistic regression were used to test the presence of NRBCs as an independent predictor of patient-related outcomes. RESULTS: The prevalence of NRBCs was 8.9% (4195/46,991 patient encounters). Patient with NRBCs were younger (median age 4.58 vs 8.23 years; P < 0.001). Those with NRBCs had higher rates of in-hospital mortality (30/2465 [1.22%] vs 65/21,741 [0.30%]; P < 0.001), sepsis (19% vs 12%; P < 0.001), shock (7% vs 4%; P < 0.001), and cardiopulmonary resuscitation (CPR) (0.62% vs 0.09%; P < 0.001). They were more likely to be admitted (59% vs 51%; P < 0.001), have longer median hospital length of stay {1.3 (interquartile range [IQR], 0.22-4.14) vs 0.8 days (IQR, 0.23-2.64); P < 0.001}, and median intensive care unit (ICU) length of stay (3.9 [IQR, 1.87-8.72] vs 2.6 days [IQR, 1.27-5.83]; P < 0.001). Multivariable regression revealed presence of NRBCs as an independent predictor for in-hospital mortality (adjusted odds ratio [aOR], 2.21; 95% confidence interval [CI], 1.38-3.53; P < 0.001), ICU admission (aOR, 1.30; 95% CI, 1.11-1.51; P < 0.001), CPR (aOR, 3.83; 95% CI, 2.33-6.30; P < 0.001), and 30-day return to the ED (aOR, 1.15; 95% CI, 1.15-1.26; P < 0.001). CONCLUSIONS: The presence of NRBCs is an independent predictor for mortality, including in-hospital mortality, ICU admission, CPR, and readmission within 30 days for children presenting to the ED.

Neuromuscular Blocker Use in Critically Ill Children: Assessing Mortality Risk by Propensity Score-Weighted Analysis.

OBJECTIVES: We aim to describe the use of continuous infusion of neuromuscular blocking agents in mechanically ventilated critically ill children and to test its association with in-hospital mortality. DESIGN: Multicenter, registry-based, observational, two-cohort-comparison retrospective study using prospectively collected data from a web-based national registry. SETTING: Seventeen PICUs in Italy. PATIENTS: We included children less than 18 years who received mechanical ventilation and a neuromuscular blocking agent infusion from January 2010 to October 2017. A propensity score-weighted Cox regression analysis was used to assess the relationship between the use of neuromuscular blocking agents and in-hospital mortality. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 23,227 patients admitted to the PICUs during the study period, 3,823 patients were included. Patients who received a continuous infusion of neuromuscular blocking agent were more likely to be younger (p < 0.001), ex-premature (p < 0.001), and presenting with less chronic respiratory insufficiency requiring home mechanical ventilation (p < 0.001). Reasons for mechanical ventilation significantly differed between patients who received a continuous infusion of neuromuscular blocking agent and patients who did not receive a continuous infusion of neuromuscular blocking agent, with a higher frequency of respiratory and cardiac diagnosis among patients who received neuromuscular blocking agents compared with other diagnoses (all p < 0.001). The covariates were well balanced in the propensity-weighted cohort. The mortality rate significantly differed among the two cohorts (patients who received a continuous infusion of neuromuscular blocking agent 21% vs patients who did not receive a continuous infusion of neuromuscular blocking agent 11%; p < 0.001 by weighted logistic regression). Patients who received a continuous infusion of neuromuscular blocking agent experienced longer mechanical ventilation and PICU stay (both p < 0.001 by weighted logistic regression). A weighted Cox regression analysis found the use of neuromuscular blocking agents to be a significant predictor of in-hospital mortality both in the unadjusted analysis (hazard ratio, 1.7; 95% CI, 1.3-2.2) and in the adjusted one (hazard ratio, 1.6; 95% CI, 1.2-2.1). CONCLUSIONS: Thirteen percent of mechanically ventilated children in PICUs received neuromuscular blocking agents. When adjusting for selection bias with a propensity score approach, the use of neuromuscular blocking agent was found to be a significant predictor of in-hospital mortality.

Pain and sedation management and monitoring in pediatric intensive care units across Europe: an ESPNIC survey.

BACKGROUND: Management and monitoring of pain and sedation to reduce discomfort as well as side effects, such as over- and under-sedation, withdrawal syndrome and delirium, is an integral part of pediatric intensive care practice. However, the current state of management and monitoring of analgosedation across European pediatric intensive care units (PICUs) remains unknown. The aim of this survey was to describe current practices across European PICUs regarding the management and monitoring of pain and sedation. METHODS: An online survey was distributed among 357 European PICUs assessing demographic features, drug choices and dosing, as well as usage of instruments for monitoring pain and sedation. We also compared low- and high-volume PICUs practices. Responses were collected from January to April 2021. RESULTS: A total of 215 (60% response rate) PICUs from 27 European countries responded. Seventy-one percent of PICUs stated to use protocols for analgosedation management, more frequently in high-volume PICUs (77% vs 63%, p = 0.028). First-choice drug combination was an opioid with a benzodiazepine, namely fentanyl (51%) and midazolam (71%) being the preferred drugs. The starting doses differed between PICUs from 0.1 to 5 mcg/kg/h for fentanyl, and 0.01 to 0.5 mg/kg/h for midazolam. Daily assessment and documentation for pain (81%) and sedation (87%) was reported by most of the PICUs, using the preferred validated FLACC scale (54%) and the COMFORT Behavioural scale (48%), respectively. Both analgesia and sedation were mainly monitored by nurses (92% and 84%, respectively). Eighty-six percent of the responding PICUs stated to use neuromuscular blocking agents in some scenarios. Monitoring of paralysed patients was preferably done by observation of vital signs with electronic devices support. CONCLUSIONS: This survey provides an overview of current analgosedation practices among European PICUs. Drugs of choice, dosing and assessment strategies were shown to differ widely. Further research and development of evidence-based guidelines for optimal drug dosing and analgosedation assessment are needed.

Diagnosis and management of urinary tract infections in children aged 2 months to 3 years in the Italian emergency units: the ItaUTI study.

Urinary tract infections (UTIs) are among the most frequent bacterial diseases in infants and children. Physician adherence to recommendations is notoriously often poor, but no data are available on UTIs management in the emergency setting. In this multicenter national study, we investigated the policies regarding UTIs management in children aged 2 months to 3 years in Italian emergency units. Between April and June 2021, directors of the emergency units were invited to answer an online survey on the following items: diagnostic approach to children with fever without an apparent source, therapeutic approach to UTIs, the use of kidney and urinary tract ultrasound, and the criteria for hospitalization. A total of 121 (89%) out of 139 of invited units participated in the study. Overall, units manage children with a suspected or confirmed UTI according to available recommendations for most of the items. However, in almost 80% (n = 94) of units, a sterile perineal bag is used to collect urine for culture. When urine is collected by cathether, heterogeneity exists on the threshold of bacterial load considered for UTI diagnosis. Conclusions: Available recommendations on UTIs in children are followed by Italian emergency units for most of the items. However, the methods to collect urine specimens for culture, one of the crucial steps of the diagnostic work-up, often do not align with current recommendations and CFU thresholds considered for diagnosis largely vary among centers. Efforts should be addressed to validate and implement new child and family friendly urine collection techniques. What is Known: • Several guidelines are published on the management of children with suspected or confirmed urinary tract infection. • No data are available on the management of pediatric urinary tract infections in the emergency setting. What is New: • Almost 80% of the Italian emergency units employ a sterile perineal bag to collect urine for culture. • Diagnostic CFU thresholds largely vary among centers.

Consensus-based recommendations for the management of juvenile systemic sclerosis.

Juvenile systemic sclerosis (JSSc) is a rare disease of childhood and currently no international consensus exists with regard to its assessment and treatment. This SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) initiative, based on expert opinion informed by the best available evidence, provides recommendations for the assessment and treatment of patients with JSSc with a view to improving their outcome. Experts focused attention not only on the skin assessment but also on the early signs of internal organ involvement whose proper treatment can significantly affect the long-term outcome. A score for disease severity is proposed in order to perform a structured assessment of outcome over time but a validation in a wider patient population is recommended. Finally, a stepwise treatment approach is proposed in order to unify the standard of care throughout Europe with the aim to reduce morbidity and mortality in this disease.

Unplanned and medical admissions to pediatric intensive care units significantly decreased during COVID-19 outbreak in Northern Italy.

Northern Italy has been the first European area affected by the COVID-19 pandemic and related social restrictive measures. We sought to evaluate the impact of the COVID-19 outbreak on PICU admissions in Northern Italy, using data from the Italian Network of Pediatric Intensive Care Units Registry. We included all patients admitted to 4 PICUs from 8-weeks-before to 8-weeks-after February 24th, 2020, and those admitted in the same period in 2019. Incidence rate ratios (IRR) evaluating incidence rate differences between pre- and post-COVID-19 periods in 2020 (IRR-1), as well as between the post-COVID-19-period with the same period in 2019 (IRR-2), were computed using zero-inflated negative binomial or Poisson regression modeling. A total of 1001 admissions were included. The number of PICU admissions significantly decreased during the COVID-19 outbreak compared to pre-COVID-19 and compared to the same period in 2020 (IRR-1 0.63 [95%CI 0.50-0.79]; IRR-2 0.70 [CI 0.57-0.91]). Unplanned and medical admissions significantly decreased (IRR-1 0.60 [CI 0.46-0.70]; IRR-2 0.67 [CI 0.51-0.89]; and IRR-1 0.52, [CI 0.40-0.67]; IRR-2 0.77 [CI 0.58-1.00], respectively). Intra-hospital, planned (potentially delayed by at least 12 h), and surgical admissions did not significantly change. Patients admitted for respiratory failure significantly decreased (IRR-1 0.55 [CI 0.37-0.77]; IRR-2 0.48 [CI 0.33-0.69]).Conclusions: Unplanned and medical PICU admissions significantly decreased during COVID-19 outbreak, especially those for respiratory failure. What is Known: • Northern Italy has been the first European area affected by the COVID-19 pandemic. • Although children are relatively spared from the severe COVID-19 disease, the pediatric care system has been affected by social restrictive measures, with a reported 73-88% reduction in pediatric emergency department admissions. What is New: • Unplanned and medical PICU admissions significantly decreased during the COVID-19 outbreak compared to pre-COVID-19 and to the same period in 2019, especially those for respiratory failure. Further studies are needed to identify associated factors and new prevention strategies.

Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach.

Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2-6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as SARS-Cov-2-associated multisystem inflammatory syndrome in children and showed similarities to Kawasaki disease. Here, we present a narrative review of cases reported in literature and we discuss the clinical acute and follow-up management of these patients. Patients with SARS-Cov-2-associated multisystem inflammatory syndrome frequently presented with persistent fever, gastrointestinal symptoms, polymorphic rash, conjunctivitis, and mucosal changes. Elevated inflammatory markers and evidence of cytokine storm were frequently observed. A subset of these patients also presented with hypotension and shock (20-100%) from either acute myocardial dysfunction or systemic hyperinflammation/vasodilation. Coronary artery dilation or aneurysms have been described in 6-24%, and arrhythmias in 7-60%. Cardiac support, immunomodulation, and anticoagulation are the key aspects for the management of the acute phase. Long-term structured follow-up of these patients is required due to the unclear prognosis and risk of progression of cardiac manifestations.Conclusion: Multisystem inflammatory syndrome is a novel syndrome related to SARS-CoV-2 infection. Evidence is still scarce but rapidly emerging in the literature. Cardiac manifestations are frequent, including myocardial and coronary involvement, and need to be carefully identified and monitored over time. What is Known: • Multisystem inflammatory syndrome in children (MIS-C) has been described associated with SARS-CoV-2. What is New: • Patients with MIS-C often present with fever, gastrointestinal symptoms, and shock. • Cardiac involvement is found in a high proportion of these patients, including ventricular dysfunction, coronary artery dilation or aneurysm, and arrhythmias. • Management is based on expert consensus and includes cardiac support, immunomodulatory agents, and anticoagulation. • Long-term follow-up is required due to the unclear prognosis and risk of progression of cardiac manifestation.

Shock Index, Coronary Perfusion Pressure, and Rate Pressure Product As Predictors of Adverse Outcome After Pediatric Cardiac Surgery.

OBJECTIVES: To determine whether shock index, coronary perfusion pressure, or rate pressure product in the first 24 hours after congenital heart surgery are independent predictors of subsequent clinically significant adverse outcomes. DESIGN: A retrospective cohort study. SETTING: A tertiary care center. PATIENTS: All patients less than 18 years old who underwent cardiac surgery at Boston Children's Hospital between January 1, 2010, and December 31, 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Shock index (heart rate/systolic blood pressure), coronary perfusion pressure (diastolic blood pressure-right atrial pressure), and rate pressure product (heart rate × systolic blood pressure) were calculated every 5 seconds, and the median value for the first 24 hours of cardiac ICU admission for each was used as a predictor. The composite, primary outcome was the occurrence of any of the following adverse events in the first 7 days following cardiac ICU admission: cardiopulmonary resuscitation, extracorporeal cardiopulmonary resuscitation, mechanical circulatory support, unplanned surgery, heart transplant, or death. The association of each variable of interest with this outcome was tested in a multivariate logistic regression model. Of the 4,161 patients included, 296 (7%) met the outcome within the specified timeframe. In a multivariate regression model adjusted for age, surgical complexity, inotropic and respiratory support, and organ dysfunction, shock index greater than 1.83 was significantly associated with the primary outcome (odds ratio, 6.6; 95% CI, 4.4-10.0), and coronary perfusion pressure greater than 35 mm Hg was protective against the outcome (odds ratio, 0.5; 0.4-0.7). Rate pressure product was not found to be associated with the outcome. However, the predictive ability of the shock index and coronary perfusion pressure models were not superior to their component hemodynamic variables alone. CONCLUSIONS: Both shock index and coronary perfusion pressure may offer predictive value for adverse outcomes following cardiac surgery in children, although they are not superior to the primary hemodynamic variables.

Avoidable Serum Potassium Testing in the Cardiac ICU: Development and Testing of a Machine-Learning Model.

OBJECTIVES: To create a machine-learning model identifying potentially avoidable blood draws for serum potassium among pediatric patients following cardiac surgery. DESIGN: Retrospective cohort study. SETTING: Tertiary-care center. PATIENTS: All patients admitted to the cardiac ICU at Boston Children's Hospital between January 2010 and December 2018 with a length of stay greater than or equal to 4 days and greater than or equal to two recorded serum potassium measurements. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We collected variables related to potassium homeostasis, including serum chemistry, hourly potassium intake, diuretics, and urine output. Using established machine-learning techniques, including random forest classifiers, and hyperparameter tuning, we created models predicting whether a patient's potassium would be normal or abnormal based on the most recent potassium level, medications administered, urine output, and markers of renal function. We developed multiple models based on different age-categories and temporal proximity of the most recent potassium measurement. We assessed the predictive performance of the models using an independent test set. Of the 7,269 admissions (6,196 patients) included, serum potassium was measured on average of 1 (interquartile range, 0-1) time per day. Approximately 96% of patients received at least one dose of IV diuretic and 83% received a form of potassium supplementation. Our models predicted a normal potassium value with a median positive predictive value of 0.900. A median percentage of 2.1% measurements (mean 2.5%; interquartile range, 1.3-3.7%) was incorrectly predicted as normal when they were abnormal. A median percentage of 0.0% (interquartile range, 0.0-0.4%) critically low or high measurements was incorrectly predicted as normal. A median of 27.2% (interquartile range, 7.8-32.4%) of samples was correctly predicted to be normal and could have been potentially avoided. CONCLUSIONS: Machine-learning methods can be used to predict avoidable blood tests accurately for serum potassium in critically ill pediatric patients. A median of 27.2% of samples could have been saved, with decreased costs and risk of infection or anemia.

Central Venous Pressure Estimation by Ultrasound Measurement of Inferior Vena Cava and Aorta Diameters in Pediatric Critical Patients: An Observational Study.

OBJECTIVES: To evaluate the ability to predict central venous pressure by ultrasound measured inferior vena cava and aortic diameters in a PICU population and to assess interoperator concordance. DESIGN: Noninterventional observational study. SETTING: PICU of a tertiary-care academic center. PATIENTS: Eighty-eight pediatric patients (0-16 yr old) with a central venous catheter in place were studied. Sixty-nine percent of the patients received positive-pressure ventilation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: An experienced and a nonexperienced operator used ultrasound to measure the maximal diameter of inferior vena cava and minimal diameter of the inferior vena cava and the maximum diameter of the abdominal aorta from the subxiphoid window. The inferior vena cava collapsibility index and the ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta were then derived. The central venous pressure was measured using a central venous catheter and recorded. Twenty-three patients had low central venous pressure values (≤ 4 mm Hg), 35 patients a value in the range of 5-9 mm Hg, and 30 patients high values (≥ 10 mm Hg). Both inferior vena cava collapsibility index and ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta were predictive of high (≥ 10 mm Hg) or low (≤ 4 mm Hg) central venous pressure. The test accuracy showed the best results in predicting low central venous pressure with an inferior vena cava collapsibility index greater than or equal to 35% and ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta less than or equal to 0.8, and in predicting high central venous pressure with an inferior vena cava collapsibility index less than or equal to 20% and ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta greater than or equal to 1.3. Inferior vena cava collapsibility index returned generally higher accuracy values than ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta. Lin's coefficient of concordance between the operators was 0.78 for inferior vena cava collapsibility index and 0.86 for ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta. CONCLUSIONS: Inferior vena cava collapsibility index and ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta correlate well with central venous pressure measurements in this PICU population, and specific inferior vena cava collapsibility index or ratio of maximal diameter of inferior vena cava/maximum diameter of the abdominal aorta thresholds appear to be able to differentiate children with high or low central venous pressure. However, the actual clinical application of these statistically significant results remains limited, especially by the intrinsic flaws of the procedure.

The snared wire technique for Sapien valve implantation in the pulmonary position.

OBJECTIVES: Description of the snared wire technique (SWT) to facilitate the delivery of the Sapien valve in pulmonary position, and comparison with standard delivery technique. BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Sapien delivery system has proven to be challenging. Therefore, alternative strategies for facilitating its delivery in this position are needed. METHODS: Retrospective analysis of patients who underwent TPVR with or without the new SWT. The SWT was chosen as an elective strategy when the anatomy was judged to be challenging for TPVR (planned SWT) or as a rescue strategy when a standard delivery failed (rescue SWT). RESULTS: From February 2018 to January 2020, 84 patients underwent TPVR with a Sapien S3 valve using either a standard delivery (n = 63, 75%) or a SWT (n = 21, 25%). Fifteen patients underwent a planned SWT, six patients underwent a rescue SWT after failure of a standard delivery. All planned SWT cases were successful and, compared to the standard delivery group, no significant differences were found in terms of time to valve-deployment, fluoroscopy time, procedure time, or frequency of complications. Rescue SWT cases had longer fluoroscopy time (p = .05), longer time to valve-deployment (p = .0001), and higher frequency of complications (p = .002) including tricuspid valve injury (p = .0004), but allowed the operator to successfully implant the valve into the desired location. CONCLUSIONS: Even in the most challenging anatomies, the SWT represents a feasible and effective alternative strategy for TPVR with the Sapien valve that should be considered when other techniques have failed.

Dexmedetomidine for Prolonged Sedation in the PICU: A Systematic Review and Meta-Analysis.

OBJECTIVES: We aimed to systematically describe the use of dexmedetomidine as a treatment regimen for prolonged sedation in children and perform a meta-analysis of its safety profile. DATA SOURCES: PubMed, EMBASE, Cochrane Library, Scopus, Web of Science, ClinicalTrials.gov, and CINAHL were searched from inception to November 30, 2018. STUDY SELECTION: We included studies involving hospitalized critically ill patients less than or equal to 18 years old receiving dexmedetomidine for prolonged infusion (≥ 24 hr). DATA EXTRACTION: Data extraction included study characteristics, patient demographics, modality of dexmedetomidine use, associated analgesia and sedation details, comfort and withdrawal evaluation scales, withdrawal symptoms, and side effects. DATA SYNTHESIS: Literature search identified 32 studies, including a total of 3,267 patients. Most of the studies were monocentric (91%) and retrospective (88%); one was a randomized trial. Minimum and maximum infusion dosages varied from 0.1-0.5 µg/kg/hr to 0.3-2.5 µg/kg/hr, respectively. The mean/median duration range was 25-540 hours. The use of a loading bolus was reported in eight studies (25%) (range, 0.5-1 µg/kg), the mode of weaning in 11 (34%), and the weaning time in six of 11 (55%; range, 9-96 hr). The pooled prevalence of bradycardia was 2.6% (n = 10 studies; 14/387 patients; 95% CI, 0.3-7.3; I = 75%), the pooled prevalence incidence of bradycardia was 2.6% (n = 10 studies; 14/387 patients; 95% CI, 0.3-7.3; I = 75%), the pooled incidence of hypotension was 6.1% (n = 8 studies; 19/304 patients; 95% CI, 0.8-15.9; I = 84%). Three studies (9%) reported side effects' onset time which in all cases was within 12 hours of the infusion starting. CONCLUSIONS: High-quality data on dexmedetomidine use for prolonged sedation and a consensus on correct dosing and weaning protocols in children are currently missing. Infusion of dexmedetomidine can be considered relatively safe in pediatrics even when longer than 24 hours.

Efficacy and Safety of Dexmedetomidine for Prolonged Sedation in the PICU: A Prospective Multicenter Study (PROSDEX).

OBJECTIVES: We sought to evaluate dexmedetomidine efficacy in assuring comfort and sparing conventional drugs when used for prolonged sedation (≥24 hr) in critically ill patients, by using validated clinical scores while systematically collecting drug dosages. We also evaluated the safety profile of dexmedetomidine and the risk factors associated with adverse events. DESIGN: Observational prospective study. SETTING: Nine tertiary-care PICUs. PATIENTS: Patients less than 18 years who received dexmedetomidine for greater than or equal to 24 hours between January 2016 and December 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One-hundred sixty-three patients (median age, 13 mo; interquartile range, 4-71 mo) were enrolled. The main indication for dexmedetomidine use was as an adjuvant for drug-sparing (42%). Twenty-three patients (14%) received dexmedetomidine as monotherapy. Seven percent of patients received a loading dose. The median infusion duration was 108 hours (interquartile range, 60-168 hr), with dosages between 0.4 (interquartile range, 0.3-0.5) and 0.8 µg/kg/hr (interquartile range, 0.6-1.2 µg/kg/hr). At 24 hours of dexmedetomidine infusion, values of COMFORT-B Scale (n = 114), Withdrawal Assessment Tool-1 (n = 43) and Cornell Assessment of Pediatric Delirum (n = 6) were significantly decreased compared with values registered immediately pre dexmedetomidine (p < 0.001, p < 0.001, p = 0.027). Dosages/kg/hr of benzodiazepines, opioids, propofol, and ketamine were also significantly decreased (p < 0.001, p < 0.001, p = 0.001, p = 0.027). The infusion was weaned off in 85% of patients, over a median time of 36 hours (interquartile range, 12-48 hr), and abruptly discontinued in 15% of them. Thirty-seven percent of patients showed hemodynamic changes, and 9% displayed hemodynamic adverse events that required intervention (dose reduction in 79% of cases). A multivariate logistic regression model showed that a loading dose (odds ratio, 4.8; CI, 1.2-18.7) and dosages greater than 1.2 µg/kg/hr (odds ratio, 5.4; CI, 1.9-15.2) increased the odds of hemodynamic changes. CONCLUSIONS: Dexmedetomidine used for prolonged sedation assures comfort, spares use of other sedation drugs, and helps to attenuate withdrawal syndrome and delirium symptoms. Adverse events are mainly hemodynamic and are reversible following dose reduction. A loading dose and higher infusion dosages are independent risk factors for hemodynamic adverse events.

Consensus-based recommendations for the management of juvenile localised scleroderma.

In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.