Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction.

Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit. Objective: To deliver the first heart valve implant that grows. Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury. Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves. Main Outcomes and Measures: Transplanted valve growth and hemodynamic function. Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves. Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

School age and adolescent heart failure following the Norwood procedure.

BACKGROUND: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. METHODS: We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure. The primary outcome was heart failure events, defined as heart transplant listing or death attributable to heart failure. Symptomatic heart failure for participants surviving 10 or more years was also assessed utilizing the Pediatric Quality of Life Inventory (PedsQL). RESULTS: Of the 345 participants who underwent a Fontan operation or survived to 6 years without Fontan, 25 (7.2%) had a heart failure event before the age of 12 years. Among these, 21 were listed for heart transplant, and 4 died from heart failure. Nineteen participants underwent heart transplant, all of whom survived to age 12 years. Factors associated with a heart failure event included longer Norwood hospital length of stay, aortic atresia, and no Fontan operation by age 6 years. Assessment of heart failure symptoms at 12 years of age revealed that 24 (12.2%) of 196 PedsQL respondents "often" or "almost always" had difficulty walking more than one block. CONCLUSIONS: Heart failure events occur in over 5% of children with palliated HLHS between preschool age and adolescence. Outcomes for children listed for transplant are excellent. However, a substantial portion of palliated HLHS children have significant symptoms of heart failure at 12 years of age.

Controlling polyHIPE Surface Properties by Tuning the Hydrophobicity of MOF Particles Stabilizing a Pickering Emulsion.

Metal-organic frameworks (MOFs) show promise for the capture of greenhouse gases. To be used at a large scale in fixed-bed processes, their shaping under a hierarchical structure is mandatory and remains a major challenge, while keeping available their high specific surface area. For that purpose, we propose herein an original method based on the stabilization of a paraffin-in-water Pickering emulsion by a fluorinated Zr MOF (UiO-66(F4)) with polyHIPEs (polymers from high internal phase emulsions) strategy consisting of the polymerization of monomers in the external phase. After polymerization of the continuous phase and elimination of the paraffin, a hierarchically structured monolith is obtained with the UiO-66(F4) particles embedded in the polymer wall and covering the internal porosity. To avoid the pore blocking induced by the embedment of the MOF particles, our strategy was to modify their hydrophilic/hydrophobic balance with a controlled adsorption of hydrophobic molecules (perfluorooctanoic acid, PFOA) on the UiO-66(F4) particles. This will induce a displacement of the MOF position at the paraffin-water interface in the emulsion and then make the particles less embedded into the polymer wall. This leads to the formation of hierarchically structured monoliths integrating UiO-66(F4) particles with higher accessibility, maintaining their original properties and allowing their application in fixed-bed processes. This strategy was demonstrated by N2 and CO2 capture, and we believe that such original strategy could be applied to other MOF materials.

Racial and socioeconomic disparities in pediatric heart transplant outcomes in the era of anti-thymocyte globulin induction.

BACKGROUND: Black race is associated with worse outcomes across solid organ transplantation. Augmenting immunosuppression through antithymocyte globulin (ATG) induction may mitigate organ rejection and graft loss. We investigated whether racial and socioeconomic outcome disparities persist in children receiving ATG induction. METHODS: Using the Pediatric Heart Transplant Society registry, we compared outcomes in Black and White children who underwent heart transplant with ATG induction between 2000 and 2020. The primary outcomes of treated rejection, rejection with hemodynamic compromise (HC), and graft loss (death or re-transplant). We explored the association of these outcomes with race and socioeconomic disparity, assessed using a neighborhood deprivation index [NDI] score at 1-year post-transplant (high NDI score implies more socioeconomic disadvantage). RESULTS: The study cohort included 1,719 ATG-induced pediatric heart transplant recipients (22% Black, 78% White). There was no difference in first year treated rejection (Black 24.5%, White 28.1%, p = 0.2). During 10 year follow up, the risk of treated rejection was similar; however, Black recipients were at higher risk of HC rejection (p = 0.009) and graft loss (p = 0.02). Black recipients had a higher mean NDI score (p < 0.001). Graft loss conditional on 1-year survival was associated with high NDI score in both White and Black recipients (p < 0.0001). In a multivariable Cox model, both high NDI score (HR 1.97, 95% CI 1.23-3.17) and Black race (HR 2.22, 95% CI 1.40-3.53) were associated with graft loss. CONCLUSION: Black race and socioeconomic disadvantage remain associated with late HC rejection and graft loss in children with ATG induction. These disparities represent important opportunities to improve long term transplant outcomes.

Green Recycling Methods to Treat Lithium-Ion Batteries E-Waste: A Circular Approach to Sustainability.

E-waste generated from end-of-life spent lithium-ion batteries (LIBs) is increasing at a rapid rate owing to the increasing consumption of these batteries in portable electronics, electric vehicles, and renewable energy storage worldwide. On the one hand, landfilling and incinerating LIBs e-waste poses environmental and safety concerns owing to their constituent materials. On the other hand, scarcity of metal resources used in manufacturing LIBs and potential value creation through the recovery of these metal resources from spent LIBs has triggered increased interest in recycling spent LIBs from e-waste. State of the art recycling of spent LIBs involving pyrometallurgy and hydrometallurgy processes generates considerable unwanted environmental concerns. Hence, alternative innovative approaches toward the green recycling process of spent LIBs are essential to tackle large volumes of spent LIBs in an environmentally friendly way. Such evolving techniques for spent LIBs recycling based on green approaches, including bioleaching, waste for waste approach, and electrodeposition, are discussed here. Furthermore, the ways to regenerate strategic metals post leaching, efficiently reprocess extracted high-value materials, and reuse them in applications including electrode materials for new LIBs. The concept of "circular economy" is highlighted through closed-loop recycling of spent LIBs achieved through green-sustainable approaches.

Relationship of ventricular assist device support duration with pediatric heart transplant outcomes.

BACKGROUND: There is wide variability in the timing of heart transplant (HTx) after pediatric VAD implant. While some centers wait months before listing for HTx, others accept donor heart offers within days of VAD surgery. We sought to determine if HTx within 30 days versus ≥ 30 after VAD impacts post-HTx outcomes. METHODS: Children on VAD pre-HTx were extracted from the Pediatric Heart Transplant Study database. The primary endpoints were post-HTx length of hospital stay (LOS) and one-year survival. Confounding was addressed by propensity score weighting using inverse probability of treatment. Propensity scores were calculated based on age, blood type, primary cardiac diagnosis, decade, VAD type, and allosensitization status. RESULTS: A total of 1064 children underwent VAD prior to HTx between 2000 to 2018. Most underwent HTx ≥ 30 days post-VAD (70%). Infants made up 22% of both groups. Patients ≥ 12 years old were 42% of the < 30 days group and children 1 to 11 years comprised 47% of the ≥ 30 days group (p < 0.001). There was no difference in the prevalence of congenital heart disease vs. cardiomyopathy (p = 0.8) or high allosensitization status (p = 0.9) between groups. Post-HTx LOS was similar between groups (p = 0.11). One-year survival was lower in the < 30 days group (adjusted mortality HR 1.76, 95% CI 1.11-2.78, p = 0.016). CONCLUSIONS: A longer duration of VAD support prior to HTx is associated with a one-year survival benefit in children, although questions of patient complexity, post-VAD complications and the impact on causality remain. Additional studies using linked databases to understand these factors will be needed to fully assess the optimal timing for post-VAD HTx.

ATP1A3-Encoded Sodium-Potassium ATPase Subunit Alpha 3 D801N Variant Is Associated With Shortened QT Interval and Predisposition to Ventricular Fibrillation Preceded by Bradycardia.

Background Pathogenic variation in the ATP1A3-encoded sodium-potassium ATPase, ATP1A3, is responsible for alternating hemiplegia of childhood (AHC). Although these patients experience a high rate of sudden unexpected death in epilepsy, the pathophysiologic basis for this risk remains unknown. The objective was to determine the role of ATP1A3 genetic variants on cardiac outcomes as determined by QT and corrected QT (QTc) measurements. Methods and Results We analyzed 12-lead ECG recordings from 62 patients (male subjects=31, female subjects=31) referred for AHC evaluation. Patients were grouped according to AHC presentation (typical versus atypical), ATP1A3 variant status (positive versus negative), and ATP1A3 variant (D801N versus other variants). Manual remeasurements of QT intervals and QTc calculations were performed by 2 pediatric electrophysiologists. QTc measurements were significantly shorter in patients with positive ATP1A3 variant status (P<0.001) than in patients with genotype-negative status, and significantly shorter in patients with the ATP1A3-D801N variant than patients with other variants (P<0.001). The mean QTc for ATP1A3-D801N was 344.9 milliseconds, which varied little with age, and remained <370 milliseconds throughout adulthood. ATP1A3 genotype status was significantly associated with shortened QTc by multivariant regression analysis. Two patients with the ATP1A3-D801N variant experienced ventricular fibrillation, resulting in death in 1 patient. Rare variants in ATP1A3 were identified in a large cohort of genotype-negative patients referred for arrhythmia and sudden unexplained death. Conclusions Patients with AHC who carry the ATP1A3-D801N variant have significantly shorter QTc intervals and an increased likelihood of experiencing bradycardia associated with life-threatening arrhythmias. ATP1A3 variants may represent an independent cause of sudden unexplained death. Patients with AHC should be evaluated to identify risk of sudden death.

Microstructural and rheological investigation of upcycled metal-organic frameworks stabilized Pickering emulsions.

HYPOTHESIS: Stabilizing Pickering emulsions with metal-organic frameworks (MOFs) is a known way to incorporate them into hierarchically porous materials. Studies generally focus on their final properties and emulsion microstructures are rarely precisely described. Our hypothesis was that characterizing the microstructural and rheological properties of Pickering emulsions stabilized solely by Al-based MOFs (MIL-96) particles would provide insights into how to control their stability and workability for potential industrial applications. EXPERIMENTS: MIL-96(Al) particles, obtained from Li-ion battery waste were used to stabilize paraffin-in-water Pickering emulsions. The influence of the formulation parameters (paraffin/water volume ratio and MIL-96(Al) content) were investigated and the emulsions were analysed using optical microscopy, cryo-scanning electron microscopy and rheological measurements. FINDINGS: MIL-96(Al) efficiently stabilized paraffin-in-water emulsions with up to 80% of internal phase. The emulsions with a low paraffin volume fraction had large droplets and a fluid gel-like texture. The emulsions with higher paraffin volume fractions were more compact and had two-step flow curves. In this system, excess MIL-96(Al) particles aggregated in the continuous phase as flocs interact with particles adsorbed at the paraffin-water interface, creating a secondary network that has to be broken for flow to resume. This behaviour may be interesting to investigate in other MOF-stabilized emulsions.

Cardiac phenotype in ATP1A3-related syndromes: A multicenter cohort study.

OBJECTIVE: To define the risks and consequences of cardiac abnormalities in ATP1A3-related syndromes. METHODS: Patients meeting clinical diagnostic criteria for rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) with ATP1A3 genetic analysis and at least 1 cardiac assessment were included. We evaluated the cardiac phenotype in an Atp1a3 knock-in mouse (Mashl+/-) to determine the sequence of events in seizure-related cardiac death. RESULTS: Ninety-eight patients with AHC, 9 with RDP, and 3 with CAPOS (63 female, mean age 17 years) were included. Resting ECG abnormalities were found in 52 of 87 (60%) with AHC, 2 of 3 (67%) with CAPOS, and 6 of 9 (67%) with RDP. Serial ECGs showed dynamic changes in 10 of 18 patients with AHC. The first Holter ECG was abnormal in 24 of 65 (37%) cases with AHC and RDP with either repolarization or conduction abnormalities. Echocardiography was normal. Cardiac intervention was required in 3 of 98 (≈3%) patients with AHC. In the mouse model, resting ECGs showed intracardiac conduction delay; during induced seizures, heart block or complete sinus arrest led to death. CONCLUSIONS: We found increased prevalence of ECG dynamic abnormalities in all ATP1A3-related syndromes, with a risk of life-threatening cardiac rhythm abnormalities equivalent to that in established cardiac channelopathies (≈3%). Sudden cardiac death due to conduction abnormality emerged as a seizure-related outcome in murine Atp1a3-related disease. ATP1A3-related syndromes are cardiac diseases and neurologic diseases. We provide guidance to identify patients potentially at higher risk of sudden cardiac death who may benefit from insertion of a pacemaker or implantable cardioverter-defibrillator.

Combining Organic and Inorganic Wastes to Form Metal-Organic Frameworks.

This paper reports a simple method to recycle plastic-bottle and Li-ion-battery waste in one process by forming valuable coordination polymers (metal-organic frameworks, MOFs). Poly(ethylene terephthalate) from plastic bottles was depolymerized to produce an organic ligand source (terephthalate), and Li-ion batteries were dissolved as a source of metals. By mixing both dissolution solutions together, selective precipitation of an Al-based MOF, known as MIL-53 in the literature, was observed. This material can be recovered in large quantities from waste and presents similar properties of purity and porosity to as-synthesis MIL-53. This work illustrates the opportunity to form hybrid porous materials by combining different waste streams, laying the foundations for an achievable integrated circular economy from different waste cycle treatments (for batteries and plastics).

An original recycling method for Li-ion batteries through large scale production of Metal Organic Frameworks.

A concept is proposed for the recycling of Li-ion batteries with an open-loop method that allows to reduce the volume of wastes and simultaneously to produce valuable materials in large amounts (Metal-Organic Frameworks, MOFs). After dissolution of Nickel, Manganese, Cobalt (NMC) batteries in acidic solution (HCl, HNO3 or H2SO4/H2O2), addition of organic moieties and a heat treatment, different MOFs are obtained. Solutions after precipitation are analyzed by inductively coupled plasma and materials are characterized by powder X-Ray diffraction, N2 adsorption, thermogravimetric analysis and Scanning electron microscope. With the use of Benzene-Tri-Carboxylic Acid as ligand, it has been possible to form selectively a MOF, based on Al metallic nodes, called MIL-96 in the literature, and known for its interesting properties in gas storage applications. The supernatant is then used again to precipitate other metals as MOFs after addition of a second batch of ligands. These two other MOFs are based on Cu (known as HKUST-1 in the literature) or Ni-Mn (with a new crystalline structure) depending of conditions. This method shows promising results at the lab scale (15 g of wastes can be converted in 10 g of MOFs), and opens interesting perspectives for the scaled-up production of MOFs.

New-onset diabetes after pediatric heart transplantation: A review of the Pediatric Heart Transplant Study.

NDT is a well-defined complication after solid organ transplantation. Little has been published describing the incidence, risk factors, and effect on outcome after pediatric heart transplantation. We performed a retrospective evaluation of pediatric patients from the PHTS registry from 2004 to 2014. Group comparison, associated factors, incidence using Kaplan-Meier method, and risk factor and outcome analysis for NDT at 1 year post-transplant. Of the 2185 recipients, 1756 were alive and followed at 1 year. Overall freedom from NDT was 98.9%, 94.7%, and 92.6% at 1, 5, and 10 years, respectively. Patients with NDT were more likely to be black (non-Hispanic; P = 0.002), older at time of transplant (P < 0.0001), and have a higher BMI percentile at time of transplant (P < 0.0001). Adjusted risk factors for NDT at 1 year were older age at transplant (years; >12 years, OR: 8.8 and 5-12 years, HR: 8.0), obese BMI percentile at time of transplant (OR: 3.8), and steroid use at 30 days after transplant (OR: 4.7). Though uncommon, NDT occurs with a constant hazard after pediatric heart transplant; it occurs more often in older patients at transplant, those who are of black race, those who are obese, and those who use steroids. Therefore, targeted weight reduction and selective steroid use in at-risk populations could reduce the incidence of early NDT. Further data are needed to determine the risk imparted by transplantation, factors that predict late-onset NDT, and whether NDT alters the outcome after transplant.

Variability in radiation dose and image quality: A comparison across fluoroscopy-system vendors, generations of equipment and institutions.

OBJECTIVES: To evaluate differences in radiation dose and image quality across institutions, fluoroscope vendors and generations of fluoroscopes for pediatric cardiac catheterization. BACKGROUND: Increased recognition of the potentially harmful effects of ionizing radiation has spurred technological advances in fluoroscopes, as well as increased focus on optimizing fluoroscope performance. There is currently little understanding of variability in the dose-image quality relationship across institutions, fluoroscope vendor and/or generation of equipment. METHODS: We evaluated latest generation fluoroscopes from Phillips, Siemens, GE, and Toshiba, and an older generation Phillips fluoroscope (release date 2003) at three different institutions. Radiation dose was measured using an anthropomorphic dose-assessment phantom with effective dose in mSv estimated from Monte Carlo simulations. Image quality phantom images were scored on a 12-point scale by three blinded reviewers. RESULTS: Fluoroscope effective doses ranged from 0.04 to 0.14 mSv/1,000 pulses for fluoroscopy with associated composite image quality scores ranging from 8.0 ± 0.6 to 10.4 ± 1.3. For cineangiography, effective doses ranged from 0.17 to 0.57 mSv/1,000 frames with image quality scores ranging from 10.1 ± 0.3 to 11.1 ± 0.3. There was modest correlation between effective dose and image quality (r = 0.67, P = 0.006). The older generation fluoroscope delivered consistently higher doses than the newer generation systems (2.3- to 3.5-fold higher for fluoroscopy; 1.1- to 3.4-fold higher for cineangiography) without appreciable differences in image quality. CONCLUSION: Technological advances have markedly improved fluoroscope performance. Comparing latest generation systems across vendors and institutions, we found variability in the dose-IQ relationship and speculate that this reflects both equipment and institutional optimization practices.

BTEX removal from aqueous solution with hydrophobic Zr metal organic frameworks.

The Zr based metal organic framework, UiO-66, has been synthesized along with its isostructural equivalence tetrafluorinated, the UiO-66-F4. The corresponding ligands to these MOFs are the widely used BDC (1,4-benzenedicarboxylic acid) and the TFBDC (2,3,5,6-Tetrafluoro-1,4-benzenedicarboxylic acid). These coordination materials have been tested towards the sorption of the organic pollutants (Benzene, Toluene, Ethylbenzene and Xylene) in aqueous phase and both materials showed capacities to adsorb all the pollutants. It has been possible to compare the efficiency in the adsorption of two iso-structural MOFs, which will behave differently due to the hydrophobic behavior of the UiO-66-F4 MOF. The addition of F in the structure of the ligand of the MOF allows to obtain a hydrophobic material by changing the nature of the interactions between the adsorbent and the adsorbate from π-π stacking in the pristine UiO-66 to hydrophobic interactions in the UiO-66-F4. However, size of the pores has also revealed an important effect, since steric impediments will decrease the capacity of the fluorinated MOF towards the sorption of bigger molecules.

Efficacy of Flecainide in the Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia: A Randomized Clinical Trial.

Importance: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a potentially lethal genetic arrhythmia syndrome characterized by polymorphic ventricular tachycardia with physical or emotional stress, for which current therapy with ß-blockers is incompletely effective. Flecainide acetate directly suppresses sarcoplasmic reticulum calcium release-the cellular mechanism responsible for triggering ventricular arrhythmias in CPVT-but has never been assessed prospectively. Objective: To determine whether flecainide dosed to therapeutic levels and added to ß-blocker therapy is superior to ß-blocker therapy alone for the prevention of exercise-induced arrhythmias in CPVT. Design, Setting, and Participants: This investigator-initiated, multicenter, single-blind, placebo-controlled crossover clinical trial was conducted from December 19, 2011, through December 29, 2015, with a midtrial protocol change at 10 US sites. Patients with a clinical diagnosis of CPVT and an implantable cardioverter-defibrillator underwent a baseline exercise test while receiving maximally tolerated ß-blocker therapy that was continued throughout the trial. Patients were then randomized to treatment A (flecainide or placebo) for 3 months, followed by exercise testing. After a 1-week washout period, patients crossed over to treatment B (placebo or flecainide) for 3 months, followed by exercise testing. Interventions: Patients received oral flecainide or placebo twice daily, with the dosage guided by trough serum levels. Main Outcomes and Measures: The primary end point of ventricular arrhythmias during exercise was compared between the flecainide and placebo arms. Exercise tests were scored on an ordinal scale of worst ventricular arrhythmia observed (0 indicates no ectopy; 1, isolated premature ventricular beats; 2, bigeminy; 3, couplets; and 4, nonsustained ventricular tachycardia). Results: Of 14 patients (7 males and 7 females; median age, 16 years [interquartile range, 15.0-22.5 years]) randomized, 13 completed the study. The median baseline exercise test score was 3.0 (range, 0-4), with no difference noted between the baseline and placebo (median, 2.5; range, 0-4) exercise scores. The median ventricular arrhythmia score during exercise was significantly reduced by flecainide (0 [range, 0-2] vs 2.5 [range, 0-4] for placebo; P < .01), with complete suppression observed in 11 of 13 patients (85%). Overall and serious adverse events did not differ between the flecainide and placebo arms. Conclusions and Relevance: In this randomized clinical trial of patients with CPVT, flecainide plus ß-blocker significantly reduced ventricular ectopy during exercise compared with placebo plus ß-blocker and ß-blocker alone. Trial Registration: clinicaltrials.gov Identifier: NCT01117454.

Recovery of metals from simulant spent lithium-ion battery as organophosphonate coordination polymers in aqueous media.

An innovative approach is proposed for the recycling of metals from a simulant lithium-ion battery (LIBs) waste aqueous solution. Phosphonate organic linkers are introduced as precipitating agents to selectively react with the metals to form coordination polymers from an aqueous solution containing Ni, Mn and Co in a hydrothermal process. The supernatant is analyzed by ICP-AES to quantify the efficiency and the selectivity of the precipitation and the materials are characterized by Scanning Electron Microscopy (SEM), Powder X-Ray Diffraction (PXRD), Thermogravimetric Analyses (TGA) and nitrogen gas sorption (BET). Conditions have been achieved to selectively precipitate Manganese or Manganese/Cobalt from this solution with a high efficiency. This work describes a novel method to obtain potentially valuable coordination polymers from a waste metal solution that can be generalized on any waste solution.

Abstract 12670: Variability in Radiation Dose and Image Quality: A Comparison Across Fluoroscopy-system Vendors and Generations of Equipment.

INTRODUCTION: Increased recognition of the potentially harmful effects of ionizing radiation has spurred technological advances to reduce exposure during fluoroscopy. However there is currently little understanding of the dose-image quality (IQ) relationship between fluoroscopy vendors and across generations of equipment used for imaging during pediatric catheterization. METHODS: We evaluated latest generation fluoroscopy systems from Phillips, Siemens, GE and Toshiba, and an older generation Phillips system (2004 release). Fluoroscopy and cineangiography were performed on a tissue simulation anthropomorphic phantom using a standardized imaging approach. Phantom surface exposures were used for Monte Carlo simulations to calculate radiation effective dose, accounting for differences in beam parameters. We also imaged a fluoroscopy IQ phantom to assess contrast-detail and line-per-inch visualization. IQ images were scored by 3 blinded reviewers with scores averaged to produce a composite rating (scale 0-18). To assess the impact of imaging approach we then simulated a neonatal cardiac catheterization incorporating "typical" imaging protocols provided by institutions using the various systems. RESULTS: Effective doses and IQ scores are summarized in the table. Effective doses varied by >400% with the older generation system consistently delivering markedly higher doses. The associated figure summarizes dose and IQ for a simulated neonatal cardiac catheterization which accounts for measured doses as well as the reported institutional imaging parameters summarized in the figure legend. CONCLUSION: These data demonstrate substantial technological improvements in fluoroscopy equipment and may be useful to justify institutional "upgrades". Comparing latest generation systems across vendors and institutions, we found variability in the dose-IQ relationship that reflects both equipment and imaging approach.

Quinidine in the treatment of KCNT1-positive epilepsies.

We report 2 patients with drug-resistant epilepsy caused by KCNT1 mutations who were treated with quinidine. Both mutations manifested gain of function in vitro, showing increased current that was reduced by quinidine. One, who had epilepsy of infancy with migrating focal seizures, had 80% reduction in seizure frequency as recorded in seizure diaries, and partially validated by objective seizure evaluation on EEG. The other, who had a novel phenotype, with severe nocturnal focal and secondary generalized seizures starting in early childhood with developmental regression, did not improve. Although quinidine represents an encouraging opportunity for therapeutic benefits, our experience suggests caution in its application and supports the need to identify more targeted drugs for KCNT1 epilepsies.