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BackgroundExtracorporeal membrane oxygenation (ECMO) is used to support patients with severe respiratory and/or cardiac failure unresponsive to conventional treatments. Despite being one of the most complex supportive therapy used in intensive care unit, there is a still a lack of training programs dedicated to improve both clinical and nonclinical skills. The aim of the current study was to evaluate if the introduction of an ECMO high-fidelity simulation curriculum among personnel reduces the times to manage bedside emergencies and improves the behavioral skills.MethodsThis retrospective study was performed from 2011 to 2016 in a 6-beds general pediatric intensive care unit (PICU) of a tertiary children's hospital. The study population was the PICU personnel. From the beginning of 2011 to the end of 2013, ECMO education was provided without a simulation program. A high-fidelity simulation program instead, was provided from December 2013. Times to manage the most common ECMO emergencies (pump failure, oxygenator change and air embolism management) as well as the behavioral skills of the personnel were evaluated before and after the simulation intervention only in novice learners to reduce the bias related to the natural improvement associated with the bedside practice.ResultsThere were a total of 30 ECMO runs and 27 ECMO emergencies over the study period. Ten ECMO emergencies occurred during the pre-simulation period and 17 in the post-simulation period. The median time to change an oxygenator in case of failure was 5.3 (4.80-6.02) min during the pre-simulation period vs. 3.9 (3.50-4.15) min in the post-simulation period (P=0.02). The median time to manage an air embolism emergency was 22 (20.00-23.50) min during the pre-simulation period vs. 15 (13.75-16.50) min in the post-simulation period (P=0.048). Only one episode of pump failure occurred in either the pre-simulation and post-simulation periods. In the pre-simulation period the median cumulative behavioral score was 40 (35.00-44.75) whereas it was 48 (44.5-49.00) in the post-simulation period (P

Bladder cancer (BC) is the tenth most commonly diagnosed malignancy worldwide. In approximately 25% of cases, it presents as a muscle-invasive disease, requiring a radical treatment. Traditionally, the mainstay of treatment has been radical cystectomy (RC), but in the last decade, bladder-sparing treatments have been gaining growing interest. In particular, trimodal therapy (TMT) seems to yield survival results comparable to RC with less morbidity and better quality of life (QoL) outcomes. In this scenario, we aimed at shedding light on the role of the histological subtypes (HS) of BC and their prognostic significance in muscle-invasive BC (MIBC), treated either surgically or with TMT. We performed a narrative review to provide an overview of the current literature on this topic. When compared with patients diagnosed with conventional urothelial carcinoma (UC) of the same disease stage, survival did not appear to be significantly worse across the reports. But when sub-analyzed for separate subtype, some appeared to be independently associated with adverse survival outcomes such as the micropapillary, plasmacytoid, small-cell, and sarcomatoid subtypes, whereas others did not. Moreover, the optimal management remains to be defined, also depending on the therapeutic susceptibility of each histology. From this perspective, multi-disciplinary assessment alongside the routine inclusion of such entities in randomized clinical trials appears to be essential. [ABSTRACT FROM AUTHOR]

OBJECTIVES The use of ventricular assist devices (VADs) in children is increasing. However, absolute numbers in individual centres and countries remain small. Collaborative efforts such as the Paedi-European Registry for Patients with Mechanical Circulatory Support (EUROMACS) are therefore essential for combining international experience with paediatric VADs. Our goal was to present the results from the fourth Paedi-EUROMACS report. METHODS All paediatric (<19 years) patients from the EUROMACS database supported by a VAD were included. Patients were stratified into a congenital heart disease (CHD) group and a group with a non-congenital aetiology. End points included mortality, a transplant and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident and pump thrombosis. RESULTS A total of 590 primary implants were included. The congenital group was significantly younger (2.5 vs 8.0 years, respectively, P  < 0.001) and was more commonly supported by a pulsatile flow device (73.5% vs 59.9%, P  < 0.001). Mortality was significantly higher in the congenital group (30.8% vs 20.4%, P  = 0.009) than in the non-congenital group. However, in multivariable analyses, CHD was not significantly associated with mortality [hazard ratio (HR) 1.285; confidence interval (CI) 0.8111–2.036, P  = 0.740]. Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with body surface area (HR 0.524, CI 0.333–0.823, P  = 0.005), CHD (HR 1.641, CI 1.054–2.555, P  = 0.028) and pulsatile flow support (HR 2.345, CI 1.406–3.910, P  = 0.001) in multivariable analyses. CONCLUSIONS This fourth Paedi-EUROMACS report highlights the increasing use of paediatric VADs. The patient populations with congenital and non-congenital aetiologies exhibit distinct characteristics and clinical outcomes. [ABSTRACT FROM AUTHOR]

OBJECTIVES Restrictive cardiomyopathy is rare and is generally associated with worse clinical outcomes compared to other cardiomyopathies. Ventricular assist device (VAD) support for these children is seldom applied and often hampered by the surgical difficulties. METHODS All paediatric (<19 years) patients with a restricted cardiomyopathy supported by a VAD from the EUROMACS database were included and compared to patients with a dilated cardiomyopathy (retrospective database analyses). Participating centres were retrospectively contacted to provide additional detailed echo and Swan Ganz measurements to analyse the effect of VAD support on pulmonary artery pressure and right ventricular function. RESULTS Forty-four paediatric VAD-supported patients diagnosed with restricted cardiomyopathy were included, with a median age at implantation of 5.0 years. Twenty-six of the 44 patient with a restricted cardiomyopathy survived to transplantation (59.1%), 16 died (36.4%) and 2 are still on ongoing VAD support (4.5%) after a median duration of support of 95.5 days (interquartile range 33.3–217.8). Transplantation probability after 1 and 2 years of VAD support in patients with a restricted cardiomyopathy were comparable to patients with a dilated cardiomyopathy (52.3% vs 51.4% and 59.5% vs 60.1%, P  = 0.868). However, mortality probability was higher in the restricted cardiomyopathy cohort (35.8% vs 17.0% and 35.8% vs 19.0%, P  = 0.005). Adverse event rates were high (cerebrovascular accident in 31.8%, pump thrombosis in 29.5%, major bleeding 25.0%, eventual biventricular support in 59.1%). In the atrially cannulated group, cerebrovascular accident and pump thrombosis occurred in twice as much patients (21.1% vs 40.0%, P  = 0.595 and 15.8% vs 40.0%, P  = 0.464; probably non-significant due to the small numbers). Pulmonary arterial pressures improved after implantation of a VAD, and 6 patients who were initially labelled as ineligible due to pulmonary hypertension could eventually be transplanted. CONCLUSIONS VAD support in children with a restricted cardiomyopathy is rarely performed. Mortality and adverse event rates are high. On the other hand, survival to cardiac transplantation was 59.1% with all patients surviving the 1st 30 days after cardiac transplantation. Pulmonary arterial pressures improved while on support, potentially making cardiac transplantation a viable option for previously ineligible children. [ABSTRACT FROM AUTHOR]

The CardioMEMS HF System (Abbott, Abbott Park, IL) is the first FDA- and CE-Mark-approved device for monitoring patients with heart failure, significantly reducing hospitalizations and improving the quality of life for NYHA class III non-congenital adult patients. This device, implanted percutaneously, allows the direct monitoring of pulmonary arterial pressure with the wireless transfer of pressure data to the clinician, who can adjust the therapy remotely. Limited experience exists regarding its use in patients with congenital heart disease (CHD). CardioMEMS device implantation is feasible and safe in selected adults and children with CHD. The potential of the device to reduce heart failure hospitalizations in this population is enormous, but further multi-center studies are needed to demonstrate its efficacy. [ABSTRACT FROM AUTHOR]

Background: Programmed death-ligand 1 (PD-L1) expression has been recognized as a potential biomarker for various cancers, yet its diagnostic and prognostic significance in urothelial bladder cancer (BCa) requires further investigation. Methods: In this prospective single-center study, we aimed to assess the feasibility and diagnostic adequacy of PD-L1 expression analysis using cytoinclusion in BCa patients. We enrolled consecutive patients undergoing endoscopic transurethral resection of bladder tumor (TURBT), repeat TURBT, or robot-assisted radical cystectomy. Urinary and tissue specimens were collected from these patients for cytoinclusion and histopathological analysis to evaluate PD-L1 expression. Results: Out of 29 patients, PD-L1 expression was detected from cytoinclusion in 42.8% (3 out of 7), 10% (1 out of 10), and 66.8% (8 out of 12) of patients with negative/papilloma, low-grade, and high-grade tumors, respectively. Conversely, histopathological analysis identified PD-L1 expression in 57.2% (4 out of 7), 30% (3 out of 10), and 83.3% (10 out of 12) of patients with negative/papilloma, low-grade, and high-grade tumors, respectively. The diagnostic concordance between cytoinclusion and histopathology was 85.7%, 80%, and 83.3% in patients with negative/papilloma, low-grade, and high-grade tumors, respectively. Conclusions: Our study underscores the promise of cytoinclusion as a minimally invasive method for quantifying urinary PD-L1 percentages. This approach could serve as both a potential prognostic and diagnostic indicator, easily obtainable from urine samples. Standardizing this technique could facilitate its widespread use as a valuable tool. [ABSTRACT FROM AUTHOR]

Aims The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. Methods and results All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8–50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [ P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [ P   = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [ P = 0.06] with a higher incidence in Group 2 during the FU [ P = 0.005]. Conclusion Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU. [ABSTRACT FROM AUTHOR]

In current clinical practice, transplant clinicians create collaborative working relationships with histocompatibility laboratory scientists to identify the risk of long‐term graft failure, which may assist in establishing strategies for treatment and surveillance. Transplant immunology research also focuses on optimizing human leukocyte antibody tissue typing and defines the most effective test for detecting the presence of donor‐specific antibodies. Although several studies have been conducted, data on pediatric heart transplant recipients are limited. Epitope load information may be utilized to identify donors with permissible human leukocyte antibody mismatches to increase transplant success. Although current guidelines do not consider human leukocyte antibody epitope‐based matching tools, these guidelines could be useful for identifying recipients at a high risk of donor‐specific antibody production, which would be appropriate for routine donor‐specific antibody screening to initiate early interventions to prevent antibody‐mediated rejection. Human leukocyte antibody matching at the epitope level offers an effective approach for identifying acceptable mismatches in sensitized patients and provides information about epitope loads. In the future, eplet matching may be used to define the best immunosuppressive therapy protocol for cardiothoracic organ transplantation. This report provides an overview of the role of human leukocyte antibodies in heart and lung transplantation. [ABSTRACT FROM AUTHOR]