Dexmedetomidine Withdrawal Syndrome in Children in the PICU: Systematic Review and Meta-Analysis.

OBJECTIVES: To systematically review literature describing the clinical presentation, risk factors, and treatment for dexmedetomidine withdrawal in the PICU (PROSPERO: CRD42022307178). DATA SOURCES: MEDLINE/PubMed, Cochrane, Web of Science, and Scopus databases were searched. STUDY SELECTION: Eligible studies were published from January 2000 to January 2022 and reported clinical data for patients younger than 21 years old following discontinuation of dexmedetomidine after greater than or equal to 24 hours of infusion. DATA EXTRACTION: Abstracts identified during an initial search were screened and data were manually abstracted after full-text review of eligible articles. The Newcastle-Ottawa Scale was used to assess study quality. Summary statistics were provided and Spearman rank correlation coefficient was used to identify relationships between covariates and withdrawal signs. A weighted prevalence for each withdrawal sign was generated using a random-effects model. DATA SYNTHESIS: Twenty-three studies (22 of which were retrospective cohort studies) containing 28 distinct cohorts were included. Median cumulative dexmedetomidine exposure by dose was 105.95 µg/kg (range, 30-232.7 µg/kg), median dexmedetomidine infusion duration was 131.75 hours (range, 20.5-525.6 hr). Weighted estimates for proportion (95% CI) of subjects experiencing withdrawal signs across all cohorts were: hypertension 0.34 (range, 0.0-0.92), tachycardia 0.26 (range, 0.0-0.87), and agitation 0.26 (range, 0.09-0.77). Meta-analysis revealed no correlation between dexmedetomidine exposure variables and withdrawal signs. A moderate negative monotonic relationship existed between the proportion of patients who had undergone cardiac surgery and the proportion experiencing hypertension (correlation coefficient, -0.47; p = 0.048) and tachycardia (correlation coefficient, -0.57; p = 0.008), indicating that in cohorts with a higher proportion of patients who were postcardiac surgery, there were fewer occurrences of hypertension and or tachycardia. CONCLUSIONS: On review of the 2000-2022 literature, dexmedetomidine withdrawal may be characterized by tachycardia, hypertension, or agitation, particularly with higher cumulative doses or prolonged durations. Since most studies included in the review were retrospective, prospective studies are needed to further clarify risk factors, establish diagnostic criteria, and identify optimal management strategies.

Acute vital signs changes are underrepresented by a conventional electronic health record when compared with automatically acquired data in a single-center tertiary pediatric cardiac intensive care unit.

OBJECTIVE: We sought to evaluate the fidelity with which the patient's clinical state is represented by the electronic health record (EHR) flow sheet vital signs data compared to a commercially available automated data aggregation platform in a pediatric cardiac intensive care unit (CICU). METHODS: This is a retrospective observational study of heart rate (HR), systolic blood pressure (SBP), respiratory rate (RR), and pulse oximetry (SpO2) data archived in a conventional EHR and an automated data platform for 857 pediatric patients admitted postoperatively to a tertiary pediatric CICU. Automated data captured for 72 h after admission were analyzed for significant HR, SBP, RR, and SpO2 deviations from baseline (events). Missed events were identified when the EHR failed to reflect the events reflected in the automated platform. RESULTS: Analysis of 132 054 622 data entries, including 264 966 (0.2%) EHR entries and 131 789 656 (99.8%) automated entries, identified 15 839 HR events, 5851 SBP events, 9648 RR events, and 2768 SpO2 events lasting 3-60 min; these events were missing in the EHR 48%, 58%, 50%, and 54% of the time, respectively. Subanalysis identified 329 physiologically implausible events (eg, likely operator or device error), of which 104 (32%) were nonetheless documented in the EHR. CONCLUSION: In this single-center retrospective study of CICU patients, EHR vital sign documentation was incomplete compared to an automated data aggregation platform. Significant events were underrepresented by the conventional EHR, regardless of event duration. Enrichment of the EHR with automated data aggregation capabilities may improve representation of patient condition.

A Minimal Opioid Postoperative Management Protocol in Congenital Cardiac Surgery: Safe and Effective.

There is evidence that reducing opioid exposure in children undergoing cardiac surgery may enhance postoperative recovery. We aimed to describe a minimal opioid postoperative management protocol in children undergoing cardiac surgery and our early outcomes with this strategy. We reviewed the medical records of children (6 months-18 years) who underwent elective cardiac surgery through a median sternotomy with cardiopulmonary bypass at our institution between 2016 and 2018. All patients were managed postoperatively using a standardized protocol. 101 children (median age 5 years) were included and 85% were extubated in the operating room. Although most patients (96%) received opioids postoperatively, opioid requirements decreased steadily over time, with 88%, 58%, and 18% of children receiving opioids on postoperative day 1, 2, and 3, respectively; 41% received no opioids after postoperative day 1. The median cumulative opioid exposure was 0.25 morphine milligram equivalents per kg (interquartile range, 0.10-0.75). Greater than mild pain was rare (<10%) at each time point. The rates of operative mortality and major complication were 0% and 3%, respectively. The median postoperative length of stay was 3 days, and 13% required readmission within 30 days. Age, cardiopulmonary bypass time, and number of benzodiazepine doses were independently associated with cumulative opioid exposure. Any complication, chest tube time, and higher STAT Category were independently associated with prolonged postoperative length of stay. A minimal opioid postoperative management protocol can be safe and effective in children undergoing cardiac surgery. Future prospective studies are needed to determine optimal practice and patient selection.

Inadequate oxygen delivery index dose is associated with cardiac arrest risk in neonates following cardiopulmonary bypass surgery.

AIM: To evaluate the Inadequate oxygen delivery (IDO2) index dose as a predictor of cardiac arrest (CA) in neonates following congenital heart surgery. METHODS: Retrospective cohort study in 3 US pediatric cardiac intensive units (1/2011- 8/2016). Calculated IDO2 index values were blinded to bedside clinicians and generated from data collected up to 30 days postoperatively, or until death or ECMO initiation. Control event data was collected from patients who did not experience CA or require ECMO. IDO2 dose was computed over a 120-min window up to 30 min prior to the CA and control events. A multivariate logistic regression prediction model including the IDO2 dose and presence or absence of a single ventricle (SV) was used. Model performance metrics were the odds ratio for each regression coefficient and receiver operating characteristic area under the curve (ROC AUC). RESULTS: Of 897 patients monitored during the study period, 601 met inclusion criteria: 29 patients had CA (33 events) and 572 patients were used for control events. Seventeen (59%) CA and 125 (26%) control events occurred in SV patients. Median age/weight at surgery and level of monitoring were similar in both groups. Median postoperative event time was 0.73 days [0.05-22.39] in CA patients and 0.82 days [0.08 25.11] in control patients. Odds ratio of the IDO2 dose coefficient was 1.008 (95% CI: 1.006-1.012, p = 0.0445), and 2.952 (95% CI: 2.952-3.258, p = 0.0079) in SV. The ROC AUC using both coefficients was 0.74 (95% CI: 0.73-0.75). These associations of IDO2 dose with CA risk remained robust, even when censored periods prior to arrest were 10 and 20 min. CONCLUSION: In neonates post-CPB surgery, higher IDO2 index dose over a 120-min monitoring period is associated with increased risk of cardiac arrest, even when censoring data 10, 20 or 30 min prior to the CA event.

Uhl's anomaly: A one and a half ventricular repair in a patient presenting with cardiac arrest.

Uhl's anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis ("Glenn" anastomosis) with right atrial reduction and right ventricular free wall plication.

Obesity and Diabetes Mellitus Adversely Affect Outcomes after Cardiac Surgery in Children's Hospitals.

OBJECTIVE: To assess how obesity or diabetes mellitus impacts outcomes in patients undergoing cardiac surgery in pediatric hospitals. DESIGN: A multi-institutional, matched case-control study of the Pediatric Health Information System database was performed. SETTING: Tertiary children's hospitals in the United States. PATIENTS: All cardiac surgical cases in patients with obesity or diabetes mellitus between 2004 and 2012 were included. Cases were matched to controls by age, sex, race, and Risk Adjustment for Congenital Heart Surgery score. OUTCOME MEASURES: Mortality, surgical complications, and hospital utilization. Differences in outcome measures were assessed by chi-square and Mann-Whitney tests. P value < .05 was significant. RESULTS: Six hundred twenty-nine cardiac surgical cases (median age 17 years [IQR 12-32]) with obesity or diabetes mellitus were matched to 629 controls. Cases demonstrated lower median household income than those in the control group ($38,031 [IQR $31,900-$48,844] vs. ($41,896 [IQR $32,854-$56,020], P < .001). Mortality was similar between cases and controls (22% vs. 1.9%, P =.692). Surgical complications occurred similarly between cases and controls (13.5% vs. 12.4%, P = .535). Cases had longer intensive care unit length of stay than controls (3 vs. 2 days, P = .001), resulting in longer overall hospital length of stay (5 vs. 4 days, P < .001). Cases also had a higher odds of undergoing mechanical ventilation for >96 hours (OR 2.0, 95% CI 1.1-3.7) and higher rate of total parenteral nutrition use (7.2% vs. 4.5%, P = .040). Median hospital charges were higher in cases (clinical: $6,696 vs. $5,872; laboratory: $14,168 vs. $12,251; pharmacy: $12,971 vs. $10,426; imaging: $6,259 vs. $5,660; P ≤ .030 for all). CONCLUSIONS: The presence of obesity or diabetes mellitus was associated with increased postoperative morbidity, hospital utilization, and cost in patients undergoing cardiac surgery in pediatric hospitals.

Coronary artery anatomy in congenital heart disease.

Expanded surgical options and improved outcomes for children born with structural heart defects have ushered a greater clinical interest in the normal and abnormal development of the coronary circulation. Anatomic variations of the coronary system may impact surgical candidacy or operative technique during neonatal life, while others may impact long-term clinical management and planning for subsequent interventions. This review aims to characterize coronary artery anatomy in symptomatic congenital heart disease, emphasizing the clinical consequence of these variations and anomalies.

Characterization of extracorporeal membrane oxygenation for pediatric cardiac arrest in the United States: analysis of the kids' inpatient database.

To characterize the overall use, cost, and outcomes of extracorporeal membrane oxygenation (ECMO) as an adjunct to cardiopulmonary resuscitation (CPR) among hospitalized infants and children in the United States, retrospective analysis of the 2000, 2003, and 2006 Kids' Inpatient Database (KID) was performed. All CPR episodes were identified; E-CPR was defined as ECMO used on the same day as CPR. Channeling bias was decreased by developing propensity scores representing the likelihood of requiring E-CPR. Univariable, multivariable, and propensity-matched analyses were performed to characterize the influence of E-CPR on survival. There were 8.6 million pediatric hospitalizations and 9,000 CPR events identified in the database. ECMO was used in 82 (0.9 %) of the CPR events. Median hospital charges for E-CPR survivors were $310,824 [interquartile range (IQR) 263,344-477,239] compared with $147,817 (IQR 62,943-317,553) for propensity-matched conventional CPR (C-CPR) survivors. Median LOS for E-CPR survivors (31 days) was considerably greater than that of propensity-matched C-CPR survivors (18 days). Unadjusted E-CPR mortality was higher relative to C-CPR (65.9 vs. 50.9 %; OR 1.9, 95 % confidence interval 1.2-2.9). Neither multivariable analysis nor propensity-matched analysis identified a significant difference in survival between groups. E-CPR is infrequently used for pediatric in-hospital cardiac arrest. Median LOS and charges are considerably greater for E-CPR survivors with C-CPR survivors. In this retrospective administrative database analysis, E-CPR did not significantly influence survival. Further study is needed to improve outcomes and to identify patients most likely to benefit from this resource-intensive therapy.

Cardiopulmonary resuscitation in hospitalized children with cardiovascular disease: estimated prevalence and outcomes from the kids' inpatient database.

OBJECTIVE: Hospitalized children with cardiovascular disease may be at increased risk of cardiac arrest; however, little data exist regarding prevalence, risk factors, or outcomes of cardiopulmonary resuscitation in these patients. We sought to characterize national estimates of cardiopulmonary resuscitation and death after cardiopulmonary resuscitation for hospitalized children with cardiovascular disease. SETTING: A total of 3,739 hospitals in 38 states participating in Kids' Inpatient Database. DESIGN: Retrospective analysis of the 2000, 2003, and 2006 Healthcare Cost and Utilization Project Kids' Inpatient Database was performed. Sample weighting was employed to produce national estimates. MEASUREMENTS AND MAIN RESULTS: Cardiovascular disease was identified in 2.2% of the estimated 22,175,468 (95% confidence interval 21,391,343-22,959,592) hospitalizations. Cardiopulmonary resuscitation occurred in 0.74% (3,698; 95% confidence interval 3,205-4,191) of hospitalizations of children with cardiovascular disease, compared with 0.05% (11,726; 95% confidence interval 10,647-12,805) without cardiovascular disease (odds ratio 13.8, 95% confidence interval 12.8-15.0). The highest frequency of cardiopulmonary resuscitation occurred with myocarditis (3.0% of admissions), heart failure (2.0%), and coronary pathology (2.0%). Compared with other forms of cardiovascular disease identified in this study, single-ventricle patients were the only subgroup who exhibited a higher mortality after cardiopulmonary resuscitation (mortality 65% vs. 55%; odds ratio 1.7 [95% confidence interval 1.2-2.6]), while those who had undergone cardiac surgery exhibited a lower mortality rate (mortality 48% vs. 57%; odds ratio 0.6 [95% confidence interval 0.5-0.8]). CONCLUSIONS: Cardiopulmonary resuscitation occurs in approximately 7 per 1,000 hospitalizations of children with cardiovascular disease, a rate greater than ten-fold that observed in hospitalizations of children without cardiovascular disease. Single-ventricle patients demonstrated increased mortality after cardiopulmonary resuscitation, while recent cardiac surgery was associated with a reduced odds of death after cardiopulmonary resuscitation. Further studies are needed to confirm these findings and develop techniques to prevent cardiac arrest in this high-risk population.

Response to intravenous potassium chloride supplementation in pediatric cardiac intensive care patients.

Potassium chloride (KCl) supplementation is common among critically ill children. Intravenous (IV) KCl supplementation for pediatric patients is poorly characterized. This study aimed to examine the efficacy and safety of IV KCL and to determine factors affecting patient responses to IV KCL in the pediatric cardiac intensive care unit (CICU). A retrospective review of 211 children (794 KCl doses) undergoing cardiac surgery or a hospital stay for heart failure in the CICU of a tertiary care teaching and referral children's hospital in 2011 was performed. Demographic data, weight, height, creatinine, and concomitant medications during each KCl dose were recorded and analyzed. Body surface area (BSA), creatinine clearance, and change in [K(+)] were calculated. The median age of the children was 4 months (range, 10 days-18 years). In this study, 151 KCl doses were administered to neonates (19 %), 307 doses (39 %) to females, and 510 doses (64 %) to patients with a BSA smaller than 0.33 m(2) (a group with relative renal insufficiency). The mean KCl dose was 0.97 ± 0.006 mEq/kg. No adverse events were associated with IV KCl administration. Blood/plasma [K(+)] increased 0.8 ± 0.02 mEq/L. The responses to KCl did not differ significantly between males and females, between neonates and children, or between patients with a BSA smaller than 0.33 m(2) and those with a BSA of 0.33 m(2) or larger. The responses to IV KCl were attenuated by concomitant furosemide (p = 0.01), amphotericin B (p < 0.01), and KCl in parenteral nutrition (p < 0.01). The responses were augmented by concomitant enalapril (p = 0.03), ethacrynic acid (p < 0.001), and hemodialysis (p < 0.01). Intravenous KCl can be administered safely for CICU patients. Responses to KCl are altered when it is given with certain medications. Intravenous KCl should be used cautiously in children receiving angiotensin-converting enzyme inhibitors. Future studies are needed for further characterization of factors affecting responses to IV KCl in children.

Resuscitation and perioperative management of the high-risk single ventricle patient: first-stage palliation.

Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. Nonetheless, these infants remain at a high risk of morbidity and mortality, and an appreciation of single ventricle physiology is fundamental to the care of these high-risk patients. Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first-stage clinical management strategies are reviewed.

Prevalence and outcomes of pediatric in-hospital cardiopulmonary resuscitation in the United States: an analysis of the Kids' Inpatient Database*.

OBJECTIVE: Population-based data on pediatric in-hospital cardiopulmonary resuscitation in the United States are scarce. Single-center studies and voluntary registries may skew the estimated prevalence and outcomes. This study aimed to determine the prevalence and outcomes of pediatric cardiopulmonary resuscitation on a national scale. DESIGN: A retrospective analysis of the Healthcare Cost and Utilization Project 2006 Kids' Inpatient Database was performed. Sample weighting was employed to produce national estimates. SETTING: Three thousand seven hundred thirty-nine hospitals in 38 states participating with the Kids' Inpatient Database. PATIENTS: All patients <20 yrs of age hospitalized in participating institutions in 2006. MEASUREMENTS AND MAIN RESULTS: Cardiopulmonary resuscitation was performed in 5,807 (95% confidence interval 5259-6355) children with prevalence of 0.77 per 1,000 admissions. Most patients (68%) were <1 yr old, and 44% were female. On multivariable analysis, cardiopulmonary resuscitation was associated with respiratory failure (odds ratio 41.5, 95% confidence interval 35.4-48.8), myocarditis (odds ratio 36.6, 95% confidence interval 21.9-61.0), acute renal failure (odds ratio 21.6, 95% confidence interval 17.5-26.7), heart failure (odds ratio 3.8, 95% confidence interval 3.0-4.8), and cardiomyopathy (odds ratio 3.8, 95% confidence interval 3.2-4.7). Overall mortality was 51.8% and greater among patients ≥1 yr (68%) vs. <1 yr (44%) (odds ratio 2.7, 95% confidence interval 2.3-3.2). Factors associated with mortality among patients receiving cardiopulmonary resuscitation on multivariable analysis included acute renal failure (odds ratio 1.5, 95% confidence interval 1.1-1.9), hepatic insufficiency (odds ratio 1.5, 95% confidence interval 1.01-2.4), sepsis (odds ratio 1.2, 95% confidence interval 1.01-1.4), and congenital heart disease (odds ratio 1.2, 95% confidence interval 1.01-1.5). CONCLUSIONS: Cardiopulmonary resuscitation is performed in approximately one in 1,300 pediatric hospitalizations. Approximately half of patients receiving cardiopulmonary resuscitation do not survive to discharge. Independent risk factors for mortality after receiving cardiopulmonary resuscitation included congenital heart disease, age ≥1 yr, acute renal failure, hepatic insufficiency, and sepsis.

Variability in delivery of care and echocardiogram surveillance of Kawasaki disease.

OBJECTIVE: The objective of this study is to characterize variability in the acute management of Kawasaki disease and compliance of echocardiogram surveillance with published American Heart Association recommendations. DESIGN: Retrospective review. SETTING: Tertiary care children's hospital. PATIENTS: All patients discharged from our institution with Kawasaki Disease between 1999 and 2007 were identified. Patients meeting any of the following were excluded: presence of a comorbidity that necessitated echocardiogram follow-up independent of Kawasaki disease diagnosis, previous history of Kawasaki disease, or magnetic resonance imaging of the coronary arteries performed in place of echocardiography. Preexisting or comorbid conditions resulting in study exclusion included structural heart disease, arrhythmia, and concomitant severe multiorgan disease at presentation (e.g., sepsis). OUTCOME MEASURES: The time course of echocardiogram surveillance among those with a normal echocardiogram at diagnosis was evaluated for compliance with published American Heart Association recommendations. Coronary artery involvement at presentation was characterized using standardized values. Additional characterization of national care practices for children with Kawasaki disease was obtained via distribution of an internet-based survey to pediatric hospitalists. RESULTS: Overall, only 11 (4%) of 302 patients with a normal study at diagnosis received a total of three studies at recommended intervals. Using standardized values for coronary artery dimensions in place of Japanese Ministry of Health aneurysm criteria, 59 (13%) of patients with Kawasaki disease experienced coronary artery involvement at diagnosis. The majority of the early coronary artery abnormalities detected in these patients using standardized definitions persisted on short-term follow-up. Pediatric hospitalist survey results revealed significant interinstitutional variability in the management of these patients. CONCLUSIONS: Lack of optimal surveillance after a diagnosis of Kawasaki disease may result in the underdiagnosis of coronary artery pathology or other complications. Considerable variability in surveillance and acute management exists, and additional research is needed to determine optimal screening and care delivery models for this population.

Warfarin anticoagulation after congenital heart surgery at a large children's hospital.

Management of warfarin in pediatric patients remains a clinical challenge. Warfarin may be administered after congenital heart surgery, and the risks of morbidity can be high. Currently, no data exist to describe the initiation of warfarin and the risk factors for morbidity in post-congenital heart surgery patients. This study aimed to characterize the time required to reach anticoagulation for patients administered warfarin therapy after cardiac surgery and to identify and characterize the risk factors for supratherapeutic anticoagulation and adverse events after warfarin initiation. This retrospective study reviewed all patients between 2006 and 2011 who received warfarin anticoagulation after cardiac surgery at our institution. Factors associated with a prolonged time required to reach an international normalized ratio (INR) of 2 and factors related to supratherapeutic anticoagulation (INR ≥ 4) were identified. The inclusion criteria were met by 59 patients. The median time required to reach an INR of at least 2 after initiation of warfarin was 2 days (interquartile range (IQR), 2-4). The only groups that required a significantly longer time to reach an INR of 2 were those with a postoperative delay in initiation of warfarin and those receiving heparin anticoagulation before and during warfarin initiation. Nine patients experienced an INR of 4 or more. However, no thrombotic events occurred, and significant bleeding was uncommon. In the largest reported group of patients undergoing anticoagulation after cardiac surgery, warfarin was well tolerated across all age groups. The median time required to reach an INR of 2 after loading with warfarin was 2 days, and adverse events were uncommon.

The potential to avoid heart transplantation in children: outpatient bridge to recovery with an intracorporeal continuous-flow left ventricular assist device in a 14-year-old.

Pediatric mechanical circulatory support has evolved considerably in the past decade. Improvements in device design and availability have led to increased short-, medium-, and long-term support options for pediatric patients with heart failure. Most pediatric mechanical circulatory support is utilized as a bridge to transplant and as a bridge to recovery in patients with temporary etiologies of heart failure (i.e., myocarditis). Described herein is our recovery program, and we report our experience as an independent pediatric ventricular assist device program with an intracorporeal continuous-flow device employed as an out-of-hospital bridge to recovery for a child with end-stage chronic heart failure.

Percutaneous atrial septal defect closure in a child with interrupted inferior vena cava: successful femoral venous approach.

Interrupted inferior vena cava (IVC) with azygous continuation to the superior vena cava (SVC) is a relatively common systemic venous anomaly. This anomaly can occasionally complicate transcatheter intervention by rendering more difficult the usual direct route to the systemic venous atrium afforded by femoral venous access. We report our experience with successful transcatheter closure of a large residual atrial septal defect (ASD) using the femoral venous route in a 3-year-old patient with heterotaxy syndrome of left isomerism type, dextrocardia, partial atrioventricular canal defect, and interrupted IVC with azygous continuation to the SVC.