Ventricular Arrhythmic Events After Transcatheter Pulmonary Valve Replacement in Adults with Repaired Tetralogy of Fallot.

Arrhythmias are a major cause of morbidity and mortality in repaired Tetralogy of Fallot (rTOF). However, predicting those at risk for life-threatening ventricular arrhythmias (VA) remains difficult. Many centers approach risk assessment at the time of surgical pulmonary valve intervention. Increasing numbers of patients have undergone transcatheter pulmonary valve replacement (TPVR), yet there are no studies evaluating VA in rTOF undergoing TPVR and the approach to risk assessment for these patients. A single center retrospective study was performed. The institutional interventional database was queried to identify all adults ≥ 18 years of age with rTOF status who underwent TPVR from 2010 to 2019. A total of 81 patients with rTOF underwent TPVR from 2010 to 2019. Mean age at time of TPVR was 27 ± 13 years; follow up after TPVR was 6.4 ± 3.1 years. VA events occurred in 4 patients (5%). There was no significant difference in current era VA risk factors in rTOF patients between the VA event group and the non-VA event group. VA risk in this cohort of rTOF with TPVR was 5%, comparable to that reported in current era surgical cohort with similar follow up. Multi-center agreement on risk assessment protocol is needed for future studies.

First-in-paediatric uses of a mechanical aspiration system for percutaneous removal of right atrial masses.

We present the first-in-paediatric uses of a mechanical aspiration system for percutaneous removal of right atrial masses in three patients, including central line-related thrombus and metastatic tumour. Percutaneous mechanical removal of right atrial masses can be performed safely and effectively.

Pediatric extracorporeal cardiopulmonary resuscitation for yew cardiotoxicity.

INTRODUCTION: English yew is an evergreen conifer frequently planted in household gardens and, when ingested in large doses, results in severe cardiotoxicity characterized by difficult to control ventricular arrhythmias with high rates of mortality. CASE REPORT: A previously healthy teenage female presented as an out-of-hospital cardiac arrest with refractory ventricular arrhythmias and severe biventricular dysfunction. Due to rapid deterioration in her clinical status, she was cannulated onto venoarterial extracorporeal membrane oxygenation (ECMO) which resulted in rapid normalization of her rhythm and ventricular function. DISCUSSION: Our case highlights the importance of keeping a broad differential diagnosis when considering etiologies of ventricular arrhythmias in the pediatric population. The final diagnosis was not made until after discharge and implantable cardiac defibrillator (ICD) placement. CONCLUSION: The delayed diagnosis of this intentional English yew ingestion ultimately resulted subsequent ICD removal. Early ECMO activation in cases of English yew toxicity can be essential for patient survival.

High-Dose Midazolam for Pediatric Refractory Status Epilepticus: A Single-Center Retrospective Study.

OBJECTIVES: We sought to describe the prevalence of midazolam treatment failure in children with refractory status epilepticus (RSE) and define a threshold dose associated with diminishing frequency of seizure cessation. DESIGN: Single center retrospective cohort study. SETTING: Single-center, quaternary-care PICU. PATIENTS: Children younger than 18 years old admitted to the PICU from 2009 to 2018 who had RSE requiring a continuous midazolam infusion. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We identified individuals with RSE through a data analytics inquiry. Receiver operating characteristic (ROC) curve analysis and Youden's index were used to assess the midazolam dose threshold associated with the highest sensitivity and specificity in identifying seizure cessation. A logistic regression model was used to determine if there was an association between maximum midazolam dose and seizure cessation. Of the 45 patients who met inclusion criteria for this study, 27 (60%) had seizure cessation with a midazolam infusion, whereas 18 (40%) required an additional pentobarbital infusion for seizure cessation. There was an association between maximum midazolam dose and seizure cessation, with patients more likely to fail treatment when midazolam was administered at higher doses. The maximum midazolam dose displayed high area under the ROC curve value for seizure cessation, and the Youden's J index cut-off point was 525 µg/kg/hr. Treatment above this dose was associated with diminishing frequency of seizure cessation. The median time spent titrating midazolam above 500 µg/kg/hr for those patients who required pentobarbital for seizure cessation was 3.83 hours (interquartile range, 2.28-5.58 hr). CONCLUSIONS: In pediatric patients with RSE requiring high dose midazolam, considerable time is spent titrating doses in a range (above 500 µg/kg/hr) that is associated with diminishing frequency of seizure cessation.

Alterations in cerebral ventricle size in children with congenital heart disease.

PURPOSE: Children with tetralogy of Fallot (TOF) and superior cavopulmonary anastomoses (SCPA) can have chronically elevated central venous pressure (CVP), which has been postulated to put patients at risk for cerebral ventriculomegaly. We aimed to examine cerebral ventricle size in children with these congenital heart lesions before and after surgery to determine how changes in CVP affect ventricle size. METHODS: We reviewed the records of patients who underwent SCPA or TOF repair between 2006 and 2015. Patients with pre- or post-operative cranial imaging were included. Frontal-occipital (FO) horn ratios were calculated as measures of cerebral ventricle volume. Reported normal mean FO ratio is 0.37 ± 0.03. Patient characteristics including occipito-fronto circumference (OFC) and available CVP measurements were recorded. CVP, FO ratios, and OFC percentiles were compared using paired and unpaired t tests and Wilcoxon matched pairs signed-rank test as appropriate. RESULTS: We reviewed 44 patients who underwent SCPA and 31 patients who underwent TOF repair who had cranial imaging studies available. In the 22 patients who underwent SCPA and had pre- and post-operative imaging, mean FO ratios significantly increased from 0.37 ± 0.03 to 0.40 ± 0.04 (P < 0.001). In contrast, in the seven patients with TOF with pre- and post-operative imaging, FO ratio was elevated at baseline and remains so after surgical repair, 0.43 ± 0.08 to 0.42 ± 0.08 (P = 0.65). Similar patterns were noted with OFC percentiles, which were significantly increased as compared to baseline after SCPA (P < 0.001) but were not significantly changed after TOF repair (P = 0.58). Finally, when available, preoperative and postoperative CVP measurements of all patients were examined, CVP increased in patients who underwent SCPA, from 6.5 ± 2 mmHg preoperatively to 9.1 ± 2.3 mmHg postoperatively (P < 0.001), while CVP remained statistically unchanged in patients who underwent TOF repair, 12.9 ± 3.3 mmHg preoperatively to 14.4 ± 3.1 mmHg postoperatively (P = 0.2). CONCLUSION: Cerebral ventriculomegaly was observed in patients with SCPA and TOF, and the observed changes in FO ratio and OFC may be related, at least in part, to CVP.

A domain-agnostic approach for characterization of lifelong learning systems.

Despite the advancement of machine learning techniques in recent years, state-of-the-art systems lack robustness to "real world" events, where the input distributions and tasks encountered by the deployed systems will not be limited to the original training context, and systems will instead need to adapt to novel distributions and tasks while deployed. This critical gap may be addressed through the development of "Lifelong Learning" systems that are capable of (1) Continuous Learning, (2) Transfer and Adaptation, and (3) Scalability. Unfortunately, efforts to improve these capabilities are typically treated as distinct areas of research that are assessed independently, without regard to the impact of each separate capability on other aspects of the system. We instead propose a holistic approach, using a suite of metrics and an evaluation framework to assess Lifelong Learning in a principled way that is agnostic to specific domains or system techniques. Through five case studies, we show that this suite of metrics can inform the development of varied and complex Lifelong Learning systems. We highlight how the proposed suite of metrics quantifies performance trade-offs present during Lifelong Learning system development - both the widely discussed Stability-Plasticity dilemma and the newly proposed relationship between Sample Efficient and Robust Learning. Further, we make recommendations for the formulation and use of metrics to guide the continuing development of Lifelong Learning systems and assess their progress in the future.

Traumatic Facial Tattoo Injuries From Gunpowder and Ammunition: A Case Series.

BACKGROUND AND OVERVIEW: Gunpowder inclusion injuries are rare occurrences in the civilian sector but are more frequently encountered in the military setting. The authors report a case series of 3 active duty military service members treated by an Army hospital's Oral & Maxillofacial Surgery service for the removal of embedded gunpowder particles so as to avoid traumatic tattooing. CASE DESCRIPTION: Three otherwise healthy active duty military service members were treated for gunpowder inclusion injuries incurred while conducting live fire training exercises at a state-side military installation between 2018 and 2019. All 3 males presented with injuries of the same etiology: Their weapons malfunctioned, and while visually inspecting the action, a round exploded close to the face. This peppered the face with gunpowder particles that were both superficially and deeply embedded. Treatment focused on individual removal using fine forceps. The patients were followed up and healed quickly without any complications, specifically without traumatic tattooing from the gunpowder injuries. CONCLUSION AND PRACTICAL IMPLICATIONS: Gunpowder inclusion injuries should be addressed quickly to remove the particles before epidermal healing occurs, thus avoiding the complication of traumatic tattooing. This surgical team recommends meticulous fine forceps removal as the treatment of choice for larger particles.

Reduction Expansion Synthesis of Sintered Metal.

Described herein is a novel method, Reduction Expansion Synthesis-Sintered Metal (RES-SM), to create a sintered metal body of a designed shape at ambient pressure, hundreds of degrees below the metal melting temperature. The precursor to the metal part is a mixture of metal oxide particles and activated metal particles, and in this study specifically nickel oxide and activated nickel metal particles. It is postulated that the metal oxide component is reduced via exposure to chemical radical species produced via thermal decomposition of urea or other organic compounds. In the study performed, the highest temperature required was 950 °C, the longest duration of high temperature treatment was 1200 s, and in all cases, the atmosphere was inert gas at ambient pressure. As discovered using scanning electron microscopy (SEM), energy dispersive spectroscopy (EDS) and x-ray diffraction (XRD), the metal that forms via the RES process presents necks of completely reduced metal between existing metal particles. The 'as produced' parts are similar in properties to 'brown' metal parts created using more standard methods and require 'post processing' to full densify. Parts treated by hot isostatic pressing show fully self-supporting, robust structures, with hardness values like others reported in literature for traditional fabrication methods. This novel method uses affordable and environmentally friendly precursors to join metallic parts at moderate temperatures, produces fully reduced metals in a very short time and has potential to make many parts simultaneously in a standard laboratory furnace.

Interferon-Stimulated Gene 15 Upregulation Precedes the Development of Blood-Brain Barrier Disruption and Cerebral Edema after Traumatic Brain Injury in Young Mice.

Recent studies show that myosin light chain kinase (MLCK) plays a pivotal role in development of cerebral edema, a known complication following traumatic brain injury (TBI) in children and a contributing factor to worsened neurologic recovery. Interferon-stimulated gene 15 (ISG15) is upregulated after cerebral ischemia and is neuroprotective. The significant role of ISG15 after TBI has not been studied. Postnatal Day (PND) 21 and PND24 mice were subjected to lateral closed-skull injury with impact depth of 2.0 or 2.25 mm. Behavior was examined at 7 d using two-object novel recognition and Wire Hang tests. Mice were sacrificed at 6 h, 12 h, 24 h, 48 h, 72 h, and 7 d. ISG15 and MLCK were analyzed by Western blot and immunohistochemistry, blood-brain barrier (BBB) disruption with Evans Blue (EB), and cerebral edema with wet/dry weights. EB extravasation and edema peaked at 72 h in both ages. PND21 mice had more severe neurological deficits, compared with PND24 mice. PND24 mice showed peak ISG15 expression at 6 h, and PND21 mice at 72 h. MLCK peaked in both age groups at 12 h and co-localized with ISG15 on immunohistochemistry and co-immunoprecipitation. These studies provide evidence, ISG15 is elevated following TBI in mice, preceding MLCK elevation, development of BBB disruption, and cerebral edema.

Drug-metabolizing enzyme genotypes and aggressive behavior treatment response in hospitalized pediatric psychiatric patients.

OBJECTIVE: The aim of this study was to examine the association between the CYP2D6 and CYP2C19 genotype-predicted combined phenotypes and short-term measures of psychotropic efficacy and toxicity. METHODS: A rater-blinded, retrospective genotype association design examined a cohort of hospitalized pediatric psychiatric patients genotyped for CYP2D6 and CYP2C19 as part of clinical care. These combined genotypes were used to predict a combined phenotype. The primary efficacy outcome measure was the behavior intervention score (BIS), a function of the number of recorded timeouts/seclusions, therapeutic holds, and physical restraints. Drug tolerability was defined as the total number of recorded adverse drug reactions. RESULTS: Primary analysis was performed on 279 pediatric patients taking CYP2D6- or CYP2C19- dependent psychotropics. Combined phenotype was associated with BIS (p = 0.01) and number of adverse drug reactions (p = 0.03). Combined poor metabolizers treated with psychotropics had the lowest BIS (highest efficacy) and the highest number of adverse drug reactions. Combined ultrarapid metabolizers had the highest BIS (lowest efficacy) and the lowest number of adverse drug reactions. CONCLUSION: Common variants in CYP2D6 and CYP2C19 are associated with the short-term efficacy and tolerability of psychotropic medications in hospitalized pediatric patients.