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.
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.
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.
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 m2 (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.
The effectiveness of pharmacologic prophylaxis against catheterassociated 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. KEY WORDS: anticoagulation agents; central venous catheter; intensive care units; pediatric; venous thrombosis C hildren with congenital heart disease (CHD) admitted postoperatively to the cardiovascular ICU (CVICU) are at high risk of venous thromboembolism (VTE) (1, 2). Placement of central venous catheter (CVC) constitutes an important risk factor for VTE (3). Peripherally inserted central catheter (PICC)
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.
AIMTo assess utility and correlation of known anticoagulation parameters in the management of pediatric ventricular assist device (VAD).METHODSRetrospective study of pediatric patients supported with a Berlin EXCOR VAD at a single pediatric tertiary care center during a single year.RESULTSWe 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).CONCLUSIONThis 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.
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.]
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