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Importance: In clinical trials, the early or accelerated continuous renal replacement therapy (CRRT) initiation strategy among adults with acute kidney injury or volume overload has not demonstrated a survival benefit. Whether the timing of initiation of CRRT is associated with outcomes among children and young adults is unknown. Objective: To determine whether timing of CRRT initiation, with and without consideration of volume overload (VO; <10% vs ≥10%), is associated with major adverse kidney events at 90 days (MAKE-90). Design, Setting, and Participants: This multinational retrospective cohort study was conducted using data from the Worldwide Exploration of Renal Replacement Outcome Collaborative in Kidney Disease (WE-ROCK) registry from 2015 to 2021. Participants included children and young adults (birth to 25 years) receiving CRRT for acute kidney injury or VO at 32 centers across 7 countries. Statistical analysis was performed from February to July 2023. Exposure: The primary exposure was time to CRRT initiation from intensive care unit admission. Main Outcomes and measures: The primary outcome was MAKE-90 (death, dialysis dependence, or persistent kidney dysfunction [>25% decline in estimated glomerular filtration rate from baseline]). Results: Data from 996 patients were entered into the registry. After exclusions (n = 27), 969 patients (440 [45.4%] female; 16 (1.9%) American Indian or Alaska Native, 40 (4.7%) Asian or Pacific Islander, 127 (14.9%) Black, 652 (76.4%) White, 18 (2.1%) more than 1 race; median [IQR] patient age, 8.8 [1.7-15.0] years) with data for the primary outcome (MAKE-90) were included. Median (IQR) time to CRRT initiation was 2 (1-6) days. MAKE-90 occurred in 630 patients (65.0%), of which 368 (58.4%) died. Among the 601 patients who survived, 262 (43.6%) had persistent kidney dysfunction. Of patients with persistent dysfunction, 91 (34.7%) were dependent on dialysis. Time to CRRT initiation was approximately 1 day longer among those with MAKE-90 (median [IQR], 3 [1-8] days vs 2 [1-4] days; P = .002). In the generalized propensity score-weighted regression, there were approximately 3% higher odds of MAKE-90 for each 1-day delay in CRRT initiation (odds ratio, 1.03 [95% CI, 1.02-1.04]). Conclusions and Relevance: In this cohort study of children and young adults receiving CRRT, longer time to CRRT initiation was associated with greater risk of MAKE-90 outcomes, in particular, mortality. These findings suggest that prospective multicenter studies are needed to further delineate the appropriate time to initiate CRRT and the interaction between CRRT initiation timing and VO to continue to improve survival and reduce morbidity in this population.
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Injúria Renal Aguda , Terapia de Substituição Renal Contínua , Criança , Humanos , Feminino , Adulto Jovem , Masculino , Diálise Renal , Terapia de Substituição Renal , Estudos de Coortes , Estudos Retrospectivos , Estudos Prospectivos , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/terapia , RimRESUMO
BACKGROUND: Pathogenic GATA6 variants have been associated with congenital heart disease (CHD) and a spectrum of extracardiac abnormalities, including pancreatic agenesis, congenital diaphragmatic hernia, and developmental delay. However, the comprehensive genotype-phenotype correlation of pathogenic GATA6 variation in humans remains to be fully understood. METHODS: Exome sequencing was performed in a family where four members had CHD. In vitro functional analysis of the GATA6 variant was performed using immunofluorescence, western blot, and dual-luciferase reporter assay. RESULTS: A novel, heterozygous missense variant in GATA6 (c.1403 G > A; p.Cys468Tyr) segregated with affected members in a family with CHD, including three with persistent truncus arteriosus. In addition, one member had childhood onset diabetes mellitus (DM), and another had necrotizing enterocolitis (NEC) with intestinal perforation. The p.Cys468Tyr variant was located in the c-terminal zinc finger domain encoded by exon 4. The mutant protein demonstrated an abnormal nuclear localization pattern with protein aggregation and decreased transcriptional activity. CONCLUSIONS: We report a novel, familial GATA6 likely pathogenic variant associated with CHD, DM, and NEC with intestinal perforation. These findings expand the phenotypic spectrum of pathologic GATA6 variation to include intestinal abnormalities. IMPACT: Exome sequencing identified a novel heterozygous GATA6 variant (p.Cys468Tyr) that segregated in a family with CHD including persistent truncus arteriosus, atrial septal defects and bicuspid aortic valve. Additionally, affected members displayed extracardiac findings including childhood-onset diabetes mellitus, and uniquely, necrotizing enterocolitis with intestinal perforation in the first four days of life. In vitro functional assays demonstrated that GATA6 p.Cys468Tyr variant leads to cellular localization defects and decreased transactivation activity. This work supports the importance of GATA6 as a causative gene for CHD and expands the phenotypic spectrum of pathogenic GATA6 variation, highlighting neonatal intestinal perforation as a novel extracardiac phenotype.
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Diabetes Mellitus , Enterocolite Necrosante , Doenças Fetais , Cardiopatias Congênitas , Perfuração Intestinal , Persistência do Tronco Arterial , Feminino , Recém-Nascido , Humanos , Criança , Cardiopatias Congênitas/genética , Fator de Transcrição GATA6/genéticaRESUMO
The Crescent dual lumen right atrial (RA) cannula has recently been introduced for the support of pediatric patients in need of venovenous extracorporeal membrane oxygenation (VV ECMO) support. We present the first pediatric case series illustrating utility of the Crescent RA cannula in the pediatric patient population at a single institution over a 10 month period. From December 2021 to August 2022, six pediatric patients were adequately supported on seven VV ECMO runs at our institution with the Crescent RA cannula. ECMO cannulation, circuit design, anticoagulation management, ECMO circuit pressures, flow rates, and recirculation were similar to our standard of care for VV ECMO. The Crescent RA cannula can be used safely and effectively to provide adequate support for pediatric patients requiring VV ECMO.
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Fibrilação Atrial , Oxigenação por Membrana Extracorpórea , Humanos , Criança , Catéteres , Cateterismo , Átrios do CoraçãoRESUMO
NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008-2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden's J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden's J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.
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Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Recém-Nascido , Humanos , Lactente , Estudos Retrospectivos , Resultado do Tratamento , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Peso ao Nascer , Fatores de Risco , Cuidados PaliativosRESUMO
BACKGROUND: Neonates and infants undergoing cardiac surgery tend to receive high volumes of blood products. The use of rotational thromboelastometry (ROTEM®) has been shown to reduce the administration of blood products in adults after cardiac surgery. We sought to develop a targeted administration of blood products based on ROTEM® to reduce blood product utilization during and after neonatal and infant cardiac surgery. METHODS: We conducted a retrospective review of data from a single center for neonates and infants undergoing congenital cardiac surgery using cardiopulmonary bypass (CPB) from September 2018-April 2019 (control group). Then, using a ROTEM® algorithm, we collected data prospectively between April-November 2021 (ROTEM group). Data collected included age, weight, gender, procedure, STAT score, CPB time, aortic cross-clamp time, volume, and type of blood products administered in the operating room and cardiothoracic intensive care unit (CTICU). In addition, ROTEM® data, coagulation profile in CTICU, chest tube output at 6 and 24 hours, use of factors concentrate, and thromboembolic complications were recorded. RESULTS: The final cohort of patients included 28 patients in the control group and 40 patients in the ROTEM group. The cohort included neonates and infants undergoing the following procedures: arterial switch, aortic arch augmentation, Norwood procedure, and comprehensive stage II procedure. There were no differences in the demographics or procedure complexity between the two groups. Patients in the ROTEM® group received fewer platelets (36 ± 12 vs. 49 ± 27 mL/kg, p 0.028) and cryoprecipitate (8 ± 3 vs. 15 ± 10 mL/kg, p 0.001) intraoperatively when compared to the control group. CONCLUSION: The utilization of ROTEM® may have contributed to a significant reduction in some blood product administration during cardiac surgery for infants and neonates. ROTEM® data may play a role in reducing blood product administration in neonatal and infant cardiac surgery.
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Procedimentos Cirúrgicos Cardíacos , Recém-Nascido , Adulto , Humanos , Lactente , Procedimentos Cirúrgicos Cardíacos/métodos , Testes de Coagulação Sanguínea , Tromboelastografia/métodos , Estudos Retrospectivos , AlgoritmosRESUMO
Congenital aortic valve stenosis (AVS) is one of the most common valve anomalies and accounts for 3%-6% of cardiac malformations. As congenital AVS is often progressive, many patients, both children and adults, require transcatheter or surgical intervention throughout their lives. While the mechanisms of degenerative aortic valve disease in the adult population are partially described, the pathophysiology of adult AVS is different from congenital AVS in children as epigenetic and environmental risk factors play a significant role in manifestations of aortic valve disease in adults. Despite increased understanding of genetic basis of congenital aortic valve disease such as bicuspid aortic valve, the etiology and underlying mechanisms of congenital AVS in infants and children remain unknown. Herein, we review the pathophysiology of congenitally stenotic aortic valves and their natural history and disease course along with current management strategies. With the rapid expansion of knowledge of genetic origins of congenital heart defects, we also summarize the literature on the genetic contributors to congenital AVS. Further, this increased molecular understanding has led to the expansion of animal models with congenital aortic valve anomalies. Finally, we discuss the potential to develop novel therapeutics for congenital AVS that expand on integration of these molecular and genetic advances.
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After Fontan operation, decreased venous capacitance and venoconstriction are adaptive mechanisms to maintain venous return and cardiac output. The consequent higher venous pressure may adversely impact end-organ function, exercise capacity and result in worse clinical outcomes. This pilot study evaluated the safety and effect of isosorbide dinitrate (ISDN), a venodilator, on exercise capacity, peripheral venous pressure (PVP), and liver stiffness in patients with Fontan circulation. In this prospective single-arm trial, 15 individuals with Fontan circulation were evaluated at baseline and after 4 weeks of therapeutic treatment with ISDN. Primary aims were to assess the safety of ISDN and the effect on maximal exercise. We also aimed to evaluate the effect of ISDN on ultrasound-assessed liver stiffness, markers of submaximal exercise, and PVP at rest and peak exercise. Repeated measures t-tests were used to assess change in variables of interest in response to ISDN. Mean age was 23.5 ± 9.2 years (range 11.2-39.0 years), and 10/15 (67%) were male. There was no statistically significant change in peak VO2 (1401 ± 428 to 1428 ± 436 mL/min, p = 0.128), but VO2 at the anaerobic threshold increased (1087 ± 313 to 1115 ± 302 mL/min, p = 0.03). ISDN was also associated with a lower peak exercise PVP (22.5 ± 4.5 to 20.6 ± 3.0 mmHg, p = 0.015). Liver stiffness was lower with ISDN, though the difference was not statistically significant (2.3 ± 0.4 to 2.1 ± 0.5 m/s, p = 0.079). Of the patients completing the trial, mild headache was common (67%), but there were no major adverse events. Treatment with ISDN for 4 weeks is well-tolerated in patients with a Fontan circulation. ISDN is associated with an increase in VO2 at anaerobic threshold, lower peak PVP, and a trend toward lower liver stiffness. Larger, longer duration studies will be necessary to define the impact of ISDN on clinical outcomes in the Fontan circulation.Clinical Trial Registration: URL: https://clinicaltrials.gov . Unique identifier: NCT04297241.
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Impaired maternal glucose metabolism during pregnancy can have significant effects on the cardiovascular system of the developing fetus. Early in pregnancy the teratogenic effects may lead to structural heart defects, while later in gestation a form of hypertrophic cardiomyopathy can develop due to overgrowth driven by fetal hyperinsulinism. We describe an infant with the uncommon combination of both dextro-transposition of the great arteries and hypertrophic cardiomyopathy. We emphasize the importance of a longitudinal multi-disciplinary approach, from fetal diagnosis to post-operative management, that allowed for an excellent outcome in this rare combination of severe cardiac malformations.
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Cardiomiopatia Hipertrófica , Transposição dos Grandes Vasos , Artérias , Feminino , Humanos , Lactente , Recém-Nascido , Gravidez , Estudos Retrospectivos , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/cirurgia , Ultrassonografia Pré-NatalRESUMO
OBJECTIVES: To eliminate catheter-associated urinary tract infections in a pediatric cardiac ICU. DESIGN: Quality improvement methodology. SETTING: Twenty-five bed cardiac ICU in a quaternary freestanding children's hospital. PATIENTS: All patients with an indwelling urinary catheter admitted to the cardiac ICU. INTERVENTIONS: Catheter-associated urinary tract infection was defined according to National Healthcare Safety Network criteria. Failure modes and effects analysis and Pareto charts were used to determine etiology of process failures. We implemented a team-based multi-interventional approach in 2012 using the Model for Improvement, which included as follows: 1) establish indications for inserting and/or maintaining bladder catheterization, 2) standardization of maintenance care for the indwelling urinary catheters, 3) protocol for management of the leaking urinary catheters, 4) incorporation of urinary catheter days and prompts for removal in daily rounds, and 5) review of all cases of prolonged indwelling urinary catheter use (> 3 d). Process control charts were used to evaluate change. MEASUREMENTS AND MAIN RESULTS: From 2011 to 2018, we showed an early and sustained improvement in catheter-associated urinary tract infection prevention standards compliance from 44% to 96% (52% improvement). These interventions showed a reduction and then elimination of catheter-associated urinary tract infections from January 2012 to the present day, despite fluctuations in total indwelling urinary catheter days. CONCLUSIONS: Utilization of quality improvement methodology allowed us to identify components of care that contributed to catheter-associated urinary tract infections. After addressing these issues, we noted a substantial reduction and then elimination of catheter-associated urinary tract infections in our pediatric cardiac ICU. Widely disseminating these interventions across multiple pediatric hospitals to determine the ability to achieve similar results are important next steps.
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Infecções Relacionadas a Cateter , Infecção Hospitalar , Cateteres Urinários , Infecções Urinárias , Infecções Relacionadas a Cateter/etiologia , Infecções Relacionadas a Cateter/prevenção & controle , Cateteres de Demora/efeitos adversos , Criança , Humanos , Unidades de Terapia Intensiva Pediátrica , Melhoria de Qualidade , Cateterismo Urinário/efeitos adversos , Infecções Urinárias/etiologia , Infecções Urinárias/prevenção & controleRESUMO
BACKGROUND: Arginine vasopressin is a nonapeptide hormone with effects on intracellular water transport and arterial tone that is used in distributive shock and following cardiopulmonary bypass. We sought to evaluate the safety and efficacy of vasopressin infusion on hemodynamics and fluid balance in the early postoperative period after Fontan completion. METHODS: We conducted a randomized, double-blinded, placebo-controlled study of vasopressin infusion for 24 hours after cardiopulmonary bypass for Fontan completion. Patient characteristics, hospital outcomes, and measures of hemodynamic parameters, urine output, chest tube drainage, fluid balance, laboratory data, and plasma arginine vasopressin concentrations were collected at baseline and for 48 postoperative hours. Data were analyzed using mixed-effect regressions. RESULTS: Twenty patients were randomized, 10 to vasopressin and 10 to placebo. Transpulmonary gradient (6.4 ± 0.5 vs 8.3 ± 0.5 mm Hg, P = .011) and chest tube drainage (23 ± 20 vs 40 ± 20 mL/kg, P = .028) for 48 hours after surgery were significantly lower in the vasopressin arm compared to placebo. Arginine vasopressin concentrations were elevated above baseline after surgery until 4 hours post cardiac intensive care unit admission in both arms, and higher in the vasopressin arm during postoperative infusion. No differences in sodium concentration, liver function, or renal function were noted between groups. CONCLUSIONS: Vasopressin infusion after Fontan completion appears safe and was associated with reduced transpulmonary gradient and chest tube drainage in the early postoperative period. A larger multiinstitutional study may show further outcome benefit.
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Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/prevenção & controle , Vasopressinas/administração & dosagem , Antidiuréticos/administração & dosagem , Pré-Escolar , Relação Dose-Resposta a Droga , Método Duplo-Cego , Feminino , Seguimentos , Humanos , Incidência , Infusões Intravenosas , Masculino , Projetos Piloto , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Resultado do Tratamento , Estados Unidos/epidemiologia , Wisconsin/epidemiologiaRESUMO
OBJECTIVES: Frequency of venous thromboembolism in pediatric trauma patients admitted to PICUs is not insignificant, ranging up to 6%. Risk factors have been identified in this population. However, there is little consensus of actual venous thromboembolism prophylaxis practice. We examined factors associated with venous thromboembolism prophylaxis in PICUs. DESIGN: A retrospective study evaluating associations with mechanical venous thromboembolism prophylaxis, pharmacologic venous thromboembolism prophylaxis, or dual therapy (DUAL) prophylaxis compared with no venous thromboembolism prophylaxis. Multivariable logistic regression explored the relationship between prophylaxis type and selected covariates with stepwise selection method to identify the independent predictors of venous thromboembolism prophylaxis utilization. SETTING: Five level I/II pediatric trauma centers in the United States. PATIENTS: Children less than 18 years from January 1, 2013, to December 31, 2013, admitted to the PICU after a trauma, identified through combined trauma registry and Virtual Pediatric Systems database. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Six hundred ninety-two patients were included in the database, with 55 excluded for missing data. Of the remaining 637 patients, 538 (84.5%) had no venous thromboembolism prophylaxis by 48 hours, 77 (12.1%) had only mechanical venous thromboembolism prophylaxis, 11 (1.7%) had DUAL, and 11 (1.7%) had pharmacologic venous thromboembolism prophylaxis alone. Multivariable analysis showed increased age, and orthopedic procedure was associated with all forms of prophylaxis. Orthopedic procedures were associated with higher utilization of dual prophylaxis use (odds ratio, 5.2; 95% CI, 1.2-21.8), pharmacologic venous thromboembolism prophylaxis (odds ratio, 8.5; 95% CI, 2.3-31.7), and mechanical venous thromboembolism prophylaxis (odds ratio, 2.2; 95% CI, 1.1-4.2) alone. Brain/spinal cord procedures (odds ratio, 3.7; 95% CI, 1.9-7.3) and abdominal procedures (odds ratio, 6.6; 95% CI, 2.5-17.1) were associated with mechanical venous thromboembolism prophylaxis. Head injury was associated with a decreased use of any prophylaxis (odds ratio, 0.5; 95% CI, 0.3-0.9). Patient comorbidities were associated with decreased use of mechanical venous thromboembolism prophylaxis (odds ratio, 0.5; 95% CI, 0.3-1.0). CONCLUSIONS: Pharmacologic venous thromboembolism prophylaxis is not common in critically ill children after trauma. Patient age, orthopedic and vascular procedures, and higher injury severity are associated with pharmacologic venous thromboembolism prophylaxis.
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Anticoagulantes/uso terapêutico , Dispositivos de Compressão Pneumática Intermitente/estatística & dados numéricos , Tromboembolia Venosa/prevenção & controle , Ferimentos e Lesões/terapia , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Lactente , Escala de Gravidade do Ferimento , Unidades de Terapia Intensiva Pediátrica , Masculino , Avaliação de Resultados em Cuidados de Saúde , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Estados Unidos , Ferimentos e Lesões/complicaçõesRESUMO
In current practice, children with anatomically normal hearts routinely undergo fluoroscopy-free ablations. Infants and children with congenital heart disease (CHD) represent the most difficult population to perform catheter ablation without fluoroscopy. We report two neonatal patients with CHD in whom cardiac ablations were performed without fluoroscopy. The first infant had pulmonary atresia with intact ventricular septum with refractory supraventricular tachycardia, and the second infant presented with Ebstein's anomaly of the tricuspid valve along with persistent supraventricular tachycardia. Both patients underwent uncomplicated, successful ablation without recurrence of arrhythmias. These cases suggest that current approaches to minimising fluoroscopy may be useful even in challenging patients such as neonates with CHD.
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Procedimentos Cirúrgicos Cardíacos/métodos , Ablação por Cateter/métodos , Anomalia de Ebstein/cirurgia , Atresia Pulmonar/cirurgia , Feminino , Fluoroscopia , Cardiopatias Congênitas/cirurgia , Humanos , Recém-NascidoRESUMO
AIMS: Non-ï¬uoroscopic catheter ablation is becoming routine. In experienced centres, fluoroscopy is rarely required. The use of a traditional catheterization lab (cath lab) may no longer be necessary. We began performing catheter ablations at a paediatric centre outside the traditional cardiac cath lab in 2013. The purpose of this study was to compare procedural features of paediatric catheter ablation performed outside the cath lab to those performed within a cath lab. METHODS AND RESULTS: We prospectively looked at patients presenting to the paediatric centre with supraventricular tachycardia (SVT) undergoing catheter ablation outside the cath lab in a standard operating room (OR group). We compared retrospectively to a control group matched for age, type, and location of arrhythmia who had ablations in a traditional cath lab (CL group). Catheter visualization was exclusively by electro-anatomic mapping. Fifty-nine patients with SVT underwent catheter ablation in the OR from October 2013 to December 2015. Thirty-three patients had accessory pathways, 29 were manifest, and 13 of those were left sided. Twenty-six had atrioventricular nodal re-entrant tachycardia. Transseptal puncture with transoesophageal echocardiography guidance was used for 10 left-sided pathways, whereas the other 3 had patent foramen ovales. Procedure time did not differ significantly between groups (OR group mean 131 min, range 57-408; CL group mean 152 min, range 68-376; P = 0.12). Acute success was similar in both groups [OR group: 58/59 (98.3%) and CL group: 57/59 (96.6%)]. There were no major complications in either group. There was no fluoroscopy used in either group. CONCLUSION: Although performing paediatric catheter ablations outside the traditional cath lab is early in our experience, we produced similar outcomes and results without encountering procedural difficulties of performing ablations in a non-conventional setting. Larger multi-centred trials will be essential to determine the feasibility of this practice.
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Cateterismo Cardíaco/métodos , Ablação por Cateter/métodos , Salas Cirúrgicas , Radiografia Intervencionista/métodos , Taquicardia Supraventricular/cirurgia , Potenciais de Ação , Adolescente , Cateterismo Cardíaco/efeitos adversos , Ablação por Cateter/efeitos adversos , Criança , Pré-Escolar , Ecocardiografia Transesofagiana , Técnicas Eletrofisiológicas Cardíacas , Feminino , Fluoroscopia , Frequência Cardíaca , Humanos , Masculino , Ohio , Duração da Cirurgia , Valor Preditivo dos Testes , Estudos Prospectivos , Radiografia Intervencionista/efeitos adversos , Estudos Retrospectivos , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
Among infants, almost half of acute liver failure cases are classified as indeterminate, whereas only a small number of cases show a documented viral infection. We present the first reported case of isolated acute hepatic failure in an infant in the setting of a human parechovirus (HPeV) infection. HPeV also may have been contributory to the posttransplant complication of 2 intussusceptions. This is a 10-month-old girl who presented with only symptoms of fussiness and was noted to have progressive decline in synthetic liver function as well as worsening coagulopathy requiring a liver transplant. The acute liver failure was in the setting of a positive serum RNA HPeV, subtype 3 (HPeV-3), after extensive diagnostic testing with genetic, autoimmune, and infectious causes otherwise negative. After liver transplantation, the postoperative course was complicated by both an ileal-ileal intussusception as well as a jejunal intussusception. Viral testing in pediatric acute liver failure is often performed, but the workup is frequently incomplete. This case report would support more extensive viral testing in this population of patients. In the setting of HPeV, clinicians could be alerted to the possibility of delayed gastrointestinal pathology in the posttransplant phase. Wider use of routine HPeV testing may more clearly define the variable clinical presentations and outcomes.
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Falência Hepática Aguda/virologia , Infecções por Picornaviridae/complicações , Feminino , Humanos , Doenças do Íleo/etiologia , Doenças do Íleo/cirurgia , Lactente , Intussuscepção/etiologia , Intussuscepção/cirurgia , Doenças do Jejuno/etiologia , Doenças do Jejuno/cirurgia , Falência Hepática Aguda/cirurgia , Transplante de Fígado , Parechovirus/isolamento & purificação , Infecções por Picornaviridae/diagnóstico , Complicações Pós-Operatórias , RNA Viral/sangueAssuntos
Síndrome de Andersen , Desfibriladores Implantáveis , Imipramina/administração & dosagem , Canais de Potássio Corretores do Fluxo de Internalização/genética , Complexos Ventriculares Prematuros , Inibidores da Captação Adrenérgica/administração & dosagem , Adulto , Síndrome de Andersen/diagnóstico , Síndrome de Andersen/genética , Síndrome de Andersen/fisiopatologia , Síndrome de Andersen/terapia , Inibidores da Colinesterase/administração & dosagem , Gerenciamento Clínico , Resistência a Medicamentos , Eletrocardiografia , Feminino , Humanos , Mutação , Brometo de Piridostigmina/administração & dosagem , Resultado do Tratamento , Complexos Ventriculares Prematuros/diagnóstico , Complexos Ventriculares Prematuros/tratamento farmacológico , Complexos Ventriculares Prematuros/fisiopatologiaRESUMO
BACKGROUND: Although uncommon, supraventricular tachycardia is difficult to manage during pregnancy. Catheter ablation traditionally has been deferred owing to radiation exposure risks. Three-dimensional mapping is a new tool in cardiac electrophysiology, which is being utilized to eliminate fluoroscopy during catheter ablation. We report a case of ablation of supraventricular tachycardia during pregnancy without using fluoroscopy. CASE: A 27-year-old woman with a 22-week twin gestation was referred for incessant supraventricular tachycardia. Medical management with propranolol and flecainide was unsuccessful. An electrophysiology study was performed with catheter navigation guided by a three-dimensional mapping system instead of fluoroscopy. The patient underwent successful cryoablation. The procedure was performed without fluoroscopy or sedation. The patient delivered healthy twins at 35 weeks of gestation without complications. On follow-up at 26 months, she showed no evidence of recurrence. CONCLUSION: New tools in electrophysiology now make curative procedures more readily available to pregnant women and safer for the fetus.
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Ablação por Cateter , Complicações Cardiovasculares na Gravidez/cirurgia , Taquicardia Supraventricular/cirurgia , Adulto , Feminino , Fluoroscopia , Humanos , GravidezRESUMO
BACKGROUND: There are nearly 200,000 US infants/children transported annually for specialty care and there are no published best practices in transport intubation. OBJECTIVE: Respiratory interventions are a priority in pediatric and neonatal critical care transport (PNCCT). A recent Delphi study identified intubation performance as an important PNCCT quality metric, though data are insufficient. The objective of the study is to determine multi-center rates of first attempt intubation success in pediatric/neonatal transport and identify practice processes associated with higher performing centers. METHODS: Retrospective chart review where data was collected from the 9 participating centers over a 6-month period from January-June 2013. Data describing intubation training and practices were gathered using SurveyMonkey® (Palo Alto, CA). Data were tabulated in Microsoft Excel (Redmond, WA) and analyzed using descriptive statistics. Through the determination of 1(st) intubation success rate across multiple pediatric/neonatal critical care transport programs, we hypothesized that the features of higher and lower performing centers can be identified to inform practice. RESULTS: 9 of 14 invited institutions participated. The median (IQR) 6-month transport volume for neonates(neo) was 289(35-646) and pediatric (ped) 510(122-831). On average, 7%(+/-3.0) of neo and 1.6%(+/-0.7) of ped transport patients required intubation. Individual centers had their initial success rate calculated and a 95% confidence interval was determined for those centers satisfying the np > 5 and n(1-p) > 5 sample size requirement for normality assumption of proportions. Since the overall success rate was 64%, it was determined that n = 14 initial intubation attempts would be the minimum number needed per center in order to fulfill the sample size requirement for normality assumption. Centers whose 95% confidence interval did not contain the initial overall success rate were identified. CONCLUSION: This represents the first multi-center neo/ped intubation dataset in PNCCT. First attempt intubation success lags behind reported anesthesia intubation rates but parallels pediatric emergency department intubation success rates. Training and operational processes are variable in PNCCT, though top performing teams require live-patient intubation success to achieve initial intubation competency.
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Cuidados Críticos , Intubação Intratraqueal/normas , Transporte de Pacientes , Humanos , Recém-Nascido , Intubação Intratraqueal/estatística & dados numéricos , Auditoria Médica , Estudos Retrospectivos , Estados UnidosRESUMO
Background: Catheter ablation is the treatment of choice for most forms of SVT. Traditionally, fluoroscopy has been the primary tool for visualizing catheter position. However, newer, 3-dimensional mapping technologies offer multiple options for minimizing fluoroscopy use. We review our 8 year experience of a zero-fluoroscopy approach using the Ensite system, and discuss our current techniques. Methods: From January 2006 to October 2013, we performed 524 catheter ablation procedures with a zero-fluoroscopy approach. The Ensite system was used exclusively. Early in the study, NavX mode was employed. In the later time period, Velocity mode was used. The Ensite system allowed easy access to all right sided arrhythmias. For left sided arrhythmias, TEE was added to aid with transseptal puncture. Results: Reviewing 524 consecutive procedures, mean age was 14 years (range 7 weeks to 65 years). Mean weight was 60.7 kg (range 3 to 174 kg). Mean procedure time was 142 minutes (range 42 - 402 minutes). There were no complications. Twenty -five patients required the use of fluoroscopy, mostly as part of simultaneous diagnostic or interventional cath procedures. There was only one instance in which fluoroscopy was used when not anticipated at the start of the procedure. With this data available, and seeing that fluoroscopy is rarely needed unexpectedly, we hypothesized that catheter ablation no longer requires a traditional cath lab. We present our early approach to ablation outside the catheterization lab. Conclusions: Three dimensional mapping systems can eliminate fluoroscopy use in virtually all routine ablation procedures. As technology improves, ablation procedures will shift beyond the traditional cath lab.