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Pulmonary hypertension has been reported as a crucial factor in the pathophysiology of severe bronchiolitis. The aim of this study was to evaluate pulmonary artery pressure (PAP) in patients with bronchiolitis and to analyze their correlation with clinical outcomes. This prospective cohort study examined children admitted for bronchiolitis. PAP was assessed by right ventricle (RV) acceleration/ejection time ratio (AT/ET), isovolumic relaxation time, eccentricity index, and the presence of a pulmonary systolic notch. Pulmonary hypertension (PH) was considered if at least two altered parameters were present. Severity of clinical course was established by higher N-terminal (NT)-prohormone BNP (NT-proBNP) values, the need for positive pressure respiratory support (PPRS), and the duration of hospital admission. One hundred sixty-nine children were included in analysis. Sixty-eight patients (40%) required PPRS, and those patients had increased NT-proBNP values and worse tricuspid annular systolic excursion (TAPSE) compared to mild cases (p 0.001and p 0.001, respectively). Twenty-two (13%) cases had at least two altered parameters of PAP and met criteria for presumed PH, with no differences in NT-proBNP values, TAPSE, need for PPRS or hospital length of stay compared to normal PAP group (p = 0.98, p = 0.07, p = 0.94 and p = 0.64, respectively). We found no correlation between altered RV AT/ET and worse cardiac function, NT-proBNP values or hospital length of stay. In our cohort, the presence of echocardiographic findings of PH were not associated with worse clinical outcomes. Patients with severe bronchiolitis had higher values ​​of NT-proBNP but, interestingly, no clear association with PH.

Mid-aortic syndrome (MAS) is an uncommon condition characterized by severe narrowing of the abdominal aorta, usually involving visceral and renal arteries. Most patients are asymptomatic and typically present with incidental hypertension which might evolve into end-organ damage if untreated. Our aim was to review 8 new pediatric MAS cases. A retrospective observational study of all pediatric patients with MAS diagnosis (April 1992-November 2021) was conducted. Patients underwent systematic evaluation (medical and family history; 12-lead electrocardiogram; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography). 8 pediatric patients with MAS were included. Median age at diagnosis was 2.6 [0.2-4.7] years; median follow-up time was 8.6 [6.6-10.0] years. 6/8 patients presented with incidental hypertension, 1/8 with heart murmur, and 1/8 with heart failure symptoms. All patients were on antihypertensive treatment. 1/8 patients underwent surgery and 7/8 an endovascular treatment. At the end of the study period, among the 6 patients that underwent a successful endovascular procedure, 2 achieved good blood pressure (BP) control, 2 acceptable BP control, 1 stage 1 hypertension and, another, stage 2 hypertension. There was 1 death during follow-up. BP monitoring in pediatric patients is crucial for early recognition of MAS. Treatment should be based on the individual clinical characteristics of patients with careful planning of surgical revascularisation, if possible, after adult growth is completed. Our study demonstrates that endovascular treatment might be a good alternative to surgery. Nevertheless, further trials with larger sample size and longer-term follow-up are required to determine the best treatment approach.

Extrapulmonary manifestations of bronchiolitis have been previously studied, with some identifying right ventricle (RV) diastolic/systolic dysfunction. We hypothesized that severe cases of bronchiolitis would have cardiac dysfunction resulting an increase in N-terminal pro-hormone B-type natriuretic peptide (NT-proBNP) values and worse outcomes. Therefore, the objective was to evaluate the existence of cardiac dysfunction and to determine its association with severe bronchiolitis.This prospective cohort study included children hospitalized for bronchiolitis under 1-year old between January 2019 and March 2020. At admission, an echocardiography was performed and plasma levels of NT-proBNP were measured. To analyze outcomes, the cohort was divided into two groups based on the need for positive pressure respiratory support (PPRS), and both were compared to healthy infants.bivariant analysis, significant differences p 0.05.One hundred eighty-one patients were included; median age was 2 months. Seventy-three patients required PPRS. Compared to controls, patients requiring PPRS showed worse RV systolic function, with lower tricuspid annular-plane systolic excursion (p = 0.002) and parameters of worse right and left diastolic function (trans-tricuspid E and A wave [p = 0.004 and p = 0.04, respectively] and tricuspid tissue doppler imaging [TDI] e' [p = 0.003], trans-mitral E and mitral TDI a' [p = 0.02 and p = 0.005, respectively]). An NT-ProBNP greater than 3582 pg/dl predicts the need for longer necessity of PPRS in patients younger than 2 months.In addition to the expected RV systolic dysfunction, patients with severe bronchiolitis have parameters of global diastolic worse function possibly secondary to intrinsic myocardial involvement. NT-ProBNP values at admission had strong discriminatory power to predict worse outcomes.

Valve dysfunction is not widely recognized as a feature in newborns born to mothers with positive anti-Ro/SSA antibodies, and only scarce reports have suggested an association between rupture of the atrioventricular valve tensor apparatus and these maternal antibodies. We report the follow-up from fetal life to the time of postnatal surgery of a patient with severe tricuspid regurgitation due to a flail of the anterior tricuspid valve leaflet who was born to an anti-Ro/SSA antibodies positive mother.

We report a case of massive ascending aortic dilatation in a patient with generalized arterial calcification of infancy (GACI). He was found to carry compound heterozygous mutations in ABCC6 gene, previously associated with pseudoxanthoma elasticum, although recently linked to GACI. Our case confirms previous reports of a genotypic overlap between both entities.

Presentamos dos casos clínicos de sendos niños con diagnóstico inicial de comunicación interventricular (CIV) con estenosis de tipo subpulmonar. La ecocardiografía inicial mostraba una posible CIV con algunas peculiaridades que precisaron más estudios de imagen. Estos estudios fueron la clave para el diagnóstico diferencial de una formación sacular en el septo basal por debajo de la válvula aórtica y que incluyó el divertículo o una cámara accesoria del ventrículo izquierdo. Ambos pacientes fueron intervenidos. Los hallazgos operatorios confirmaron el diagnóstico de divertículo de ventrículo izquierdo

Introduction: Congenital heart disease (CHD) patients requiring neonatal cardiopulmonary bypass (CPB) surgery often experience abnormal neurodevelopment. Open heart surgery and anesthesia induce changes in the developing brain concomitant with an increase in the level of neuromarkers. The aim of this study is to assess the relationship between neuromarkers, brain dysplasia and neurodevelopmental outcomes for CHD patients undergoing neonatal CPB. Hypothesis: Neuromarkers and neuroimaging brain dysplasia can predict the neurodevelopmental outcomes for CHD patients undergoing neonatal CPB. Methods: 42 neonates with CHD requiring CPB were included in this prospective, observational study. Using the Neurology 4-Plex A Assay, serum levels of glial fibrillary acidic protein (GFAP), ubiquitin carboxyl-terminal hydrolase L1 (UCH-L1), neurofilament light (NF-L), and Tau protein were measured pre-operatively and at 0 hours, 24 hours, and 48 hours post-operatively. Brain MRIs were obtained in 24 neonates. Neurodevelopment testing was completed at 6 months to 36 months of age using the Bayley-III Scales of infant. Findings of brain MRI were scored using an internally developed scoring system which evaluates the brains structures individually on degrees of dysplasia, hypoplasia, and abnormalities, as well as providing a comprehensive assessment of overall brain development combining the individual structural abnormalities into a composite total brain dysplasia score (TBDS). Results: Bayley-III scales were negatively correlated with NF-L and Tau. For UCH-L1, a positive correlation was seen at immediate postop. Hemorrhage is associated with low language score (P=0.009) and elevated UCH-L1 levels at 24 hours and 48 hours. Infraction (P=0.0375) and lower maturation (P=0.03) are associated with low fine motor scores. Lower maturation is related to more domains below average (P=0.01). Infarction is associated with high postoperative Tau level (P=0.04).TBDS is associated with high levels of GFAP, NFL-1, and decreased language development. Conclusion: A negative correlation of Tau and NF-L to Bayley-III scales was consistent with their known roles as markers of neural injury. High NF-L and GFAP are associated with brain dysplasia.

To improve the understanding of the pathophysiology of transposition of the great arteries with intact ventricular septum (TGA/IVS) and the cardiac remodeling occurring from fetal to neonatal life, we performed a morphometric and functional echocardiographic assessment in fetuses and newborns. This was a prospective case-control study performed in a tertiary referral center, which included fetuses and newborns with a diagnosis of TGA/IVS between 2011 and 2018. Morphometry and systolic and diastolic function parameters were compared with age and body surface-matched healthy controls. Twenty-one TGA/IVS patients were included during the study period and morphometric and functional echocardiographic data were recorded. TGA/IVS patients showed morphometric and functional changes of increased overall volume and output, predominantly in the aortic component from fetus to newborn, probably due to compensatory mechanisms secondary to brain hypoxia.

Introduction: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae. Patients and methods: We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes. Results: 51 patients were admitted. A total of 9 patients (17.6%) died during the followup. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of followup in the 42 survivors was 107.81 months (±53.56).The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae. Conclusions: Coronary artery to right ventricle fistulae may not be a contraindication for biventricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class. Resumen: Introducción: Los neonatos afectos de atresia pulmonar con tabique interventricular íntegro y estenosis pulmonar crítica representan un espectro amplio, incluyendo aquellos con hipoplasia significativa del ventrículo derecho. La presencia de fístulas arteriales coronarias a ventrículo derecho puede ser una contraindicación para la descompresión del ventrículo derecho. El principal objetivo del presente trabajo es analizar los resultados a corto y largo plazo durante 20 años de estos pacientes, e identificar los factores diferenciales entre ambos grupos incluyendo aquellos pacientes afectos por fístulas arteriales coronarias. Pacientes y métodos: Este un estudio retrospectivo donde se identificaron todos los pacientes diagnosticados de atresia pulmonar con septo interventricular íntegro y estenosis pulmonar crítica entre los meses de enero de 1996 y enero de 2018. Se recogieron y analizaron las características morfológicas del ventrículo derecho, el manejo quirúrgico, la intervención percutánea y la evolución a corto y a largo plazo. Resultados: Cincuenta y un pacientes fueron incluidos. Un total de 9 (17,6%) fallecieron durante el seguimiento. Ninguno de ellos presentaba fístulas arteriales coronarias a ventrículo derecho. La media de seguimiento de los restantes 42 supervivientes fue de 107,81 meses (±53,56).La clase funcional según la New York Heart Association en la revisión más reciente fue de 1,2. Los supervivientes del grupo de estenosis pulmonar crítica presentaban una clase funcional de 1,1 y los del grupo de atresia pulmonar con tabique interventricular íntegro de 1,6. No hubo diferencias entre los pacientes que presentaban fístulas arteriales coronarias a ventrículo derecho y los que no. Conclusiones: La presencia de fístulas arteriales coronarias a ventrículo derecho no es una contraindicación para la vía biventricular. Los pacientes con estenosis pulmonar crítica presentan una mejor evolución que los afectos de atresia pulmonar con tabique interventricular íntegro. La estrategia de apertura agresiva y precoz de la válvula pulmonar tiene una buena supervivencia global correlacionada con una buena clase funcional.

Resumen: Introducción: Los neonatos afectos de atresia pulmonar con tabique interventricular íntegro y estenosis pulmonar crítica representan un espectro amplio, incluyendo aquellos con hipoplasia significativa del ventrículo derecho. La presencia de fístulas arteriales coronarias a ventrículo derecho puede ser una contraindicación para la descompresión del ventrículo derecho. El principal objetivo del presente trabajo es analizar los resultados a corto y largo plazo durante 20 años de estos pacientes, e identificar los factores diferenciales entre ambos grupos incluyendo aquellos pacientes afectos por fístulas arteriales coronarias. Pacientes y métodos: Estudio retrospectivo donde se identificaron todos los pacientes diagnosticados de atresia pulmonar con septo interventricular íntegro y estenosis pulmonar crítica entre los meses de enero de 1996 y enero de 2018. Se recogieron y analizaron las características morfológicas del ventrículo derecho, el manejo quirúrgico, la intervención percutánea y la evolución a corto y a largo plazo. Resultados: Fueron incluidos cincuenta y un pacientes. Un total de 9 (17,6%) fallecieron durante el seguimiento. Ninguno de ellos presentaba fístulas arteriales coronarias a ventrículo derecho. La mediana de seguimiento de los restantes 42 supervivientes fue de 8,9 años (rango: 1-16).La clase funcional según la New York Heart Association en la revisión más reciente fue de 1,2. Los supervivientes del grupo de estenosis pulmonar crítica presentaban una clase funcional de 1,1 y los del grupo de atresia pulmonar con tabique interventricular íntegro de 1,6. No hubo diferencias entre los pacientes que presentaban fístulas arteriales coronarias a ventrículo derecho y los que no. Conclusiones: La presencia de fístulas arteriales coronarias a ventrículo derecho no es una contraindicación para la vía biventricular. Los pacientes con estenosis pulmonar crítica presentan una mejor evolución que los afectos de atresia pulmonar con tabique interventricular íntegro. La estrategia de apertura agresiva y precoz de la válvula pulmonar tiene una buena supervivencia global correlacionada con una buena clase funcional. Abstract: Introduction: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae. Patients and methods: We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes. Results: 51 patients were admitted. A total of 9 patients (17.6%) died during the followup. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of follow up in the 42 survivors was 8.9 years (1-16).The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae. Conclusions: Coronary artery to right ventricle fistulae may not be a contraindication for biventricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class.

Background There is an increasing number of young adults living with congenital heart disease (CHD). The goal of this study was to ascertain the frequency of acute kidney injury (AKI) as well as the risk factors and outcomes associated with AKI in young adults with CHD after a surgical procedure. Methods This was a single-center retrospective cohort study including all patients 18 to 40 years of age with a diagnosis of CHD admitted to a quaternary care children's hospital cardiac intensive care unit postoperatively from 2004 to 2015. We defined AKI using the Kidney Disease Improving Global Outcomes criteria for serum creatinine. We explored potential susceptibilities and exposures for AKI using multivariable logistic regression and determined the association of AKI with duration of mechanical ventilation and length of stay using Poisson regression. Results In 699 consecutively admitted patients AKI occurred in 13.2%. Suspected sepsis (odds ratio [OR], 2.87; 95% confidence interval [CI], 1.17 to 7.05), exposure to calcineurin inhibitors (OR, 5.80; 95% CI, 1.06 to 31.59), vancomycin (OR, 3.35; 95% CI, 1.11 to 10.14), and piperacillin-tazobactam (OR, 4.12; 95% CI, 1.23 to 13.78) increased the odds of AKI even after controlling for age, ejection fraction, recent cardiac catheterization, repeat cardiopulmonary bypass, bypass time, cross-clamp time, and other potential nephrotoxic medications. AKI was associated with a longer duration of mechanical ventilation (OR, 1.47; 95% CI, 1.15 to 1.89) and intensive care unit length of stay (OR, 1.50; 95% CI, 1.30 to 1.72). Conclusions AKI is common in young adults with CHD postoperatively and is associated with negative outcomes. The results highlight the importance future research and clinical efforts aimed at prevention and improved management of AKI in this patient group.

This study was conducted to determine the association between fluid balance metrics and mortality and other postoperative outcomes after neonatal cardiac operation in a contemporary multicenter cohort.This was an observational cohort study across 22 hospitals in neonates (≤30 days) undergoing cardiac operation. We explored overall percentage fluid overload, postoperative day 1 percentage fluid overload, peak percentage fluid overload, and time to first negative daily fluid balance. The primary outcome was in-hospital mortality. Secondary outcomes included postoperative duration of mechanical ventilation and intensive care unit (ICU) and hospital length of stay. Multivariable logistic or negative binomial regression was used to determine independent associations between fluid overload variables and each outcome.The cohort included 2223 patients. In-hospital mortality was 3.9% (n = 87). Overall median peak percentage fluid overload was 4.9% (interquartile range, 0.4%-10.5%). Peak percentage fluid overload and postoperative day 1 percentage fluid overload were not associated with primary or secondary outcomes. Hospital resource utilization increased on each successive day of not achieving a first negative daily fluid balance and was characterized by longer duration of mechanical ventilation (incidence rate ratio, 1.11; 95% CI, 1.08-1.14), ICU length of stay (incidence rate ratio, 1.08; 95% CI, 1.03-1.12), and hospital length of stay (incidence rate ratio, 1.09; 95% CI, 1.05-1.13).Time to first negative daily fluid balance, but not percentage fluid overload, is associated with improved postoperative outcomes in neonates after cardiac operation. Specific treatments to achieve an early negative fluid balance may decrease postoperative care durations.

Background: Infants undergoing congenital cardiac surgery with cardiopulmonary bypass are at high risk for respiratory complications. As impaired airway mucociliary clearance (MCC) can potentially contribute to pulmonary morbidity, our study objective was to measure airway clearance in infants undergoing congenital cardiac surgery and examine correlation with clinical covariables that may impair airway clearance function.Materials and Methods: Airway clearance in infants was measured over 30 min using inhaled nebulized Technetium 99m sulfur colloid administered either via a nasal cannula or the endotracheal tube in intubated infants. This was conducted bedside with a portable gamma camera. No difficulty was encountered in positioning the gamma camera over the patient, and neither the camera nor the MCC scan interfered with routine medical care or caused any adverse events. Patient and perioperative variables were examined relative to the MCC measurements.Results: We prospectively enrolled 57 infants undergoing congenital cardiac surgery and conducted a single MCC scan per patient. MCC data from 42 patients were analyzable, including five pre-operative, 15 (40.5%) in the immediate post-operative period (days 1–2), and 22 (59.5%) were later post-operative (≥3 days). Pre-operative MCC was inversely proportional to days requiring post-operative mechanical ventilation (p = 0.006) and non-invasive positive pressure ventilation (p = 0.017). MCC was higher at later post-operative days (p = 0.002) with immediate post-operative MCC being lower (3%; 0–13%) than either pre-operative (21%; 4–25%) (p = 0.091) or later post-operative MCC (18%; 0–29%) (p = 0.054). Among the infants with low post-operative MCC, significantly more were pre-mature [5/19 (26%) vs. 0/18 (0%); p = 0.046], were intubated [14/19 (75%) vs. only 7/18 (39%); p = 0.033] and were receiving higher FiO2 (40%, 27–47% vs. 26%, 21–37%; p = 0.015).Conclusions: This is the first study to show that infants undergoing congenital cardiac surgery have impaired MCC. MCC appeared lowest in the immediate post-operative period. Worse MCC was associated with pre-maturity, mechanical ventilation, or receiving higher FiO2. These findings suggest MCC scans should be further explored for informing clinical decision making to improve post-surgical respiratory outcomes. The possible therapeutic benefit of airway clearance maneuvers for infants with poor MCC function should also be investigated.

Multisystem inflammatory syndrome associated with the SARS-CoV-2 pandemic has recently been described in children (MIS-C), partially overlapping with Kawasaki disease (KD). We hypothesized that (a) MIS-C and prepandemic KD cytokine profiles may be unique and justify the clinical differences observed, and (b) SARS-CoV-2-specific immune complexes (ICs) may explain the immunopathology of MIS-C. Seventy-four children were included: 14 with MIS-C, 9 patients positive for SARS-CoV-2 by PCR without MIS-C (COVID), 14 with prepandemic KD, and 37 healthy controls (HCs). Thirty-four circulating cytokines were quantified in pretreatment serum or plasma samples and the presence of circulating SARS-CoV-2 ICs was evaluated in MIS-C patients. Compared with HCs, the MIS-C and KD groups showed most cytokines to be significantly elevated, with IFN-gamma-induced response markers (including IFN-gamma, IL-18, and IP-10) and inflammatory monocyte activation markers (including MCP-1, IL-1 alpha, and IL-1RA) being the main triggers of inflammation. In linear discriminant analysis, MIS-C and KD profiles overlapped; however, a subgroup of MIS-C patients (MIS-C-plus) differentiated from the remaining MIS-C patients in IFN-gamma, IL-18, GM-CSF, RANTES, IP-10, IL-1 alpha, and SDF-1 and incipient signs of macrophage activation syndrome. Circulating SARS-CoV-2 ICs were not detected in MIS-C patients. Our findings suggest a major role for IFN-gamma in the pathogenesis of MIS-C, which may be relevant for therapeutic management.

The purpose of this Neonatal and Pediatric Heart and Renal Outcomes Network study was to describe the epidemiology and outcomes of cardiac surgery-associated acute kidney injury (CS-AKI) after cardiac surgery without cardiopulmonary bypass (non-CPB).We performed a retrospective study of neonates (≤30 days) who underwent non-CPB cardiac surgery at 22 centers affiliated with the Pediatric Cardiac Critical Care Consortium. CS-AKI was defined using the modified Kidney Disease: Improving Global Outcomes serum creatinine and urine output criteria from postoperative days 0 to 6. CS-AKI defined by serum creatinine was further subclassified into transient (resolved by postoperative day 3) and persistent/late (≥3 days). Multivariable regression analyses were used to determine risk factors for CS-AKI and associations with outcomes of ventilation hours and cardiac intensive care unit length of stay.Five hundred eighty-two neonates (median age at surgery, 9 days [interquartile range, 5-15], 25% functional single ventricle] were included. CS-AKI occurred in 38.3%: Rate and severity varied across centers. Aggregate daily CS-AKI prevalence peaked on postoperative day 1 (17.1%). No stage of CS-AKI was associated with ventilation hours or length of stay. Persistent/late CS-AKI occurred in 48 patients (8%). Prostaglandin use and single-ventricle surgery were associated with persistent/late CS-AKI. Higher baseline serum creatinine but not persistent/late CS-AKI was associated with longer ventilation duration and intensive care unit length of stay after adjusting for confounders.Kidney Disease: Improving Global Outcomes-defined CS-AKI occurred commonly in neonates undergoing non-CPB cardiac surgery. However most CS-AKI was transient, and no CS-AKI classification was associated with worse outcomes. Further work is needed to determine the CS-AKI definition that best associates with outcomes in this cohort.

The newborn rabbit is a useful animal model for various pathologies and procedures. Airway management of the rabbit is complex due to its anatomical characteristics, which is further complicated in the case of the newborn. Of the different methods of advanced airway management, endotracheal intubation is less aggressive than tracheostomy, and is more feasible than supraglottic management given the lack of supraglottic devices of such a small size. As direct glottis visualization is very difficult in animals this size, this blind intubation model is presented as an effective alternative, especially for experiments requiring prolonged anesthesia. Using this method, we performed blind intubations with a 90% success rate.

OBJECTIVES: To describe presentation, hospital course, and predictors of bad outcome in multisystem inflammatory syndrome in children (MIS-C). METHODS: Retrospective data review of a case series of children meeting the published definition for MIS-C who were discharged or died between March 1, 2020, and June 15, 2020, from 33 participating European, Asian, and American hospitals. Data were collected through a Web-based survey and included clinical, laboratory, electrocardiographic, and echocardiographic findings and treatment management. RESULTS: We included 183 patients with MIS-C: male sex, 109 (59.6%); mean age 7.0 ± 4.7 years; Black race, 56 (30.6%); obesity, 48 (26.2%). Overall, 114 of 183 (62.3%) had evidence of severe acute respiratory syndrome coronavirus 2 infection. All presented with fever, 117 of 183 (63.9%) with gastrointestinal symptoms, and 79 of 183 (43.2%) with shock, which was associated with Black race, higher inflammation, and imaging abnormalities. Twenty-seven patients (14.7%) fulfilled criteria for Kawasaki disease. These patients were younger and had no shock and fewer gastrointestinal, cardiorespiratory, and neurologic symptoms. The remaining 77 patients (49.3%) had mainly fever and inflammation. Inotropic support, mechanical ventilation, and extracorporeal membrane oxygenation were indicated in 72 (39.3%), 43 (23.5%), and 4 (2.2%) patients, respectively. A shorter duration of symptoms before admission was found to be associated with poor patient outcome and for extracorporeal membrane oxygenation and/or death, with 72.3% (95% confidence interval: 0.56–0.90; P = .006) increased risk per day reduction and 63.3% (95% confidence interval: 0.47–0.82; P < .0001) increased risk per day reduction respectively. CONCLUSIONS: In this case series, children with MIS-C presented with a wide clinical spectrum, including Kawasaki disease–like, life-threatening shock and milder forms with mainly fever and inflammation. A shorter duration of symptoms before admission was associated with a worse outcome.

Introduction: Following the SARS-CoV-2 pandemic peak, children suffering from a multiorgan inflammatory disease that often leads to shock have been reported. This condition shares features with Kawasaki disease, but its etiopathogenesis is unknown. Hypothesis: We aimed to describe presentation and hospital course for this pediatric inflammatory multisystemic syndrome associated with COVID-19 (PIMS-TS). Methods: Data were collected from a retrospective review of children from 33 participating European, Asian and American sites. Results: We included 183 patients (109 males, 59·6%) with PIMS-TS, at a mean age of 7·0 (±4·7) years. Fifty-six (30·6%) had black ethnicity and obesity was present in 48 (26·2%) cases. Overall, 114/183 (62·3%) had biological evidence of current or recent SARS-CoV-2 infection. At admission, all presented with fever, 117/183 (63·9%) with gastrointestinal symptoms and 79/183 (43·2%) with shock, that was associated with more frequent black ethnicity, higher inflammatory markers and more cardiac involvement. Twenty-seven patients (14·7%) fulfilled criteria for Kawasaki disease. They were younger with no shock, fewer gastrointestinal, cardio-respiratory and neurological symptoms. Among the remaining PIMS-TS patients, 77 (49·3%) had mainly fever and inflammation with less cardiac involvement. For the entire cohort of 183 patients, the mean duration of admission was 8·6 (±5·6) days. Inotropic support, mechanical ventilation and ECMO were indicated in 72 (39·3%), 43 (23·5%) and 4 (2·2%) patients, respectively. Three patients (1·6%) died. A shorter duration of symptoms before admission was a risk factor for worse outcome and for ECMO/death, with 63% increased risk per day reduction (95%CI 0·39-0·93, p=0·03) and with 51·4% increased risk per day reduction (95%CI 0·36-0·9, p=0·03), respectively. Conclusions: We describe the first largest international series of children with PIMS-TS. Life-threatening shock is a common presentation. A shorter duration of symptoms prior to admission characterizes the fulminant form of the disease with potentially worse outcome.

Introduction Cardiac surgery (CS) in pediatric patients induces an overt oxidative stress (OS) response. Children are particularly vulnerable to OS related injury. The immaturity of their organs and antioxidant systems as well as the induction of OS in cardio-pulmonary bypass (CPB) surgery may have an important impact on outcomes. The purpose of this study was to describe the OS response, measured by urinary free 8-iso-PGF2α, in infants undergoing CS and to evaluate the relationship between OS response and post-operative clinical outcomes. Methods Infants with congenital heart disease undergoing CS with or without CPB were eligible for enrollment. Children were classified as neonates ( Results Sixty-two patients (60% neonates) were included. Urine 8-iso-PGF2α levels 24 hours after surgery (8.04 [6.4–10.3] ng/mg Cr) were higher than pre-operative levels (5.7 [4.65–7.58] ng/mg Cr) (p Conclusions Children undergoing CS, particularly neonatal patients, experience a significant post-operative OS response that might play an important role in postoperative morbidity. TGA patients undergoing arterial switch operations demonstrate the highest post-operative OS response. Rapid clearance of isoprostanes, which occurs more frequently in older patients with more mature antioxidant systems, might be associated with better clinical outcomes.

Right ventricular (RV) dilatation is the determining prognostic factor in the long-term follow up of patients with repaired Tetralogy of Fallot (TOF). The objective of this study is to analyze whether the results vary depending on the timing of the complete repair and on the surgical technique applied. This is a retrospective longitudinal study in which patients with standard TOF were divided into 3 groups depending on their age at surgical repair: group 1 = Early repair (n = 12,1-8 months), group 2 = Late repair (n = 26, > 8 months), and group 3 = Late repair with previous palliative surgery (n = 17, > 8 months). Clinical, echocardiographic and cardiac magnetic resonance (CMR) data from patients that had received complete reparative surgery in our institution from January 2000 to March 2014 were analyzed and compared. 55 patients with echocardiogram and CMR studies (13.39 ± 3.59 years) were reviewed. All patients had at least moderate pulmonary regurgitation (PR). We observed a positive correlation between PR and right ventricular end-diastolic volume (r2 = 0.418; p = 0,004). Group 3 had more severe right ventricular dilatation than patients in groups 1 and 2 (p = 0.001). No differences in right ventricular end-diastolic volume, PR, and pulmonary trunk dimensions were observed between groups 1 and 2. Patients in group 3 had a longer hospital stay. Although all patients from our cohort had significant PR, age at surgery was not related to RV or pulmonary trunk dilatation. Previous palliative surgery was associated with more severe right ventricular dilatation and longer hospital stays. No differences were observed between early and late repair groups. Our study suggests that postponing TOF repair to a late stage does not improve the degree of PR or long-term morbidity from RV dilatation. Palliative surgery should be avoided if possible.

Background During the severe acute respiratory syndrome Coronavirus 2 pandemic, there has been an increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi‐system inflammatory syndrome during the severe acute respiratory syndrome Coronavirus 2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain, and Pakistan with multi‐system inflammatory syndrome in children and had consultation with a pediatric cardiologist. Data collected included demographics, clinical presentation, laboratory values, electrocardiographic abnormalities, echocardiographic findings, and initial therapies. A total of 55 patients presented with multi‐system inflammatory syndrome in children. Thirty‐five patients (64%) had evidence of decreased left ventricular function, 17 (31%) had valvulitis, 12 (22%) with pericardial effusion, and 11 (20%) with coronary abnormalities. Twenty‐seven (49%) required intensive care unit admission and 24 (44%) had evidence of shock. Eleven patients (20%) fulfilled complete Kawasaki disease criteria and had lower NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide), D‐dimer, and ferritin levels compared with those who did not fulfill criteria. Electrophysiologic abnormalities occurred in 6 patients and included complete atrioventricular block, transient atrioventricular block, and ventricular tachycardia. Conclusions To our knowledge, we describe the first international cohort of pediatric patients with multi‐system inflammatory syndrome in children during the severe acute respiratory syndrome Coronavirus 2 pandemic with a range of cardiac manifestations. This article brings awareness and alertness to the global medical community to recognize these children during the pandemic and understand the need for early cardiology evaluation and follow‐up.

Coronavirus disease 2019 (COVID-19) has affected patients across all age groups, with a wide range of illness severity from asymptomatic carriers to severe multi-organ dysfunction and death. Although early reports have shown that younger age groups experience less severe disease than older adults, our understanding of this phenomenon is in continuous evolution. Recently, a severe multisystem inflammatory syndrome in children (MIS-C), with active or recent COVID-19 infection, has been increasingly reported. Children with MIS-C may demonstrate signs and symptoms of Kawasaki disease, but also have some distinct differences. These children have more frequent and severe gastrointestinal symptoms and are more likely to present with a shock-like presentation. Moreover, they often present with cardiovascular involvement including myocardial dysfunction, valvulitis, and coronary artery dilation or aneurysms. Here, we present a review of the literature and summary of our current understanding of cardiovascular involvement in children with COVID-19 or MIS-C and identifying the role of a pediatric cardiologist in caring for these patients.

Background During the SARS‐CoV2 pandemic, there has been increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi‐system inflammatory syndrome (MIS‐C) during the SARS‐CoV2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain and Pakistan with MIS‐C and had consultation with a pediatric cardiologist. Data collected included demographics, clinical presentation, laboratory values, electrocardiographic abnormalities, echocardiographic findings and initial therapies. A total of 55 patients presented with MIS‐C. Thirty‐five patients (64%) had evidence of decreased left ventricular function, 17 (31%) had valvulitis, 12 (22%) with pericardial effusion and 11 (20%) with coronary abnormalities. Twenty‐seven (49%) required ICU admission and 24 (44%) had evidence of shock. Eleven patients (20%) fulfilled complete Kawasaki disease criteria and had lower NT pro‐BNP, D‐dimer and ferritin levels compared with those who did not fulfill criteria. Electrophysiologic abnormalities occurred in 6 patients and included complete atrioventricular (AV) block, transient AV block and ventricular tachycardia. Conclusions We describe the first international cohort of pediatric patients with MIS‐C during the SARS‐CoV2 pandemic with a range of cardiac manifestations. This paper brings awareness and alertness to the global medical community to recognize these children during the pandemic and understand the need for early cardiology evaluation and follow‐up.

AIM: Cardiopulmonary bypass (CPB) generates a systemic capillary leak syndrome with pulmonary edema. Lung ultrasound (LUS) could be useful to monitor it. Primary objective was to compare sensitivity, specificity, positive and negative predictive values of chest X-ray and LUS to detect pulmonary edema using a new score (LUCAS). Secondary objectives were to evaluate correlation between LUCAS score and respiratory and inotropic support. METHODS: Prospective intervention study including patients

Background Point-of-care ultrasound (POCUS) is nowadays an essential tool in critical care. Its role seems more important in neonates and children where other monitoring techniques may be unavailable. POCUS Working Group of the European Society of Paediatric and Neonatal Intensive Care (ESPNIC) aimed to provide evidence-based clinical guidelines for the use of POCUS in critically ill neonates and children. Methods Creation of an international Euro-American panel of paediatric and neonatal intensivists expert in POCUS and systematic review of relevant literature. A literature search was performed, and the level of evidence was assessed according to a GRADE method. Recommendations were developed through discussions managed following a Quaker-based consensus technique and evaluating appropriateness using a modified blind RAND/UCLA voting method. AGREE statement was followed to prepare this document. Results Panellists agreed on 39 out of 41 recommendations for the use of cardiac, lung, vascular, cerebral and abdominal POCUS in critically ill neonates and children. Recommendations were mostly (28 out of 39) based on moderate quality of evidence (B and C). Conclusions Evidence-based guidelines for the use of POCUS in critically ill neonates and children are now available. They will be useful to optimise the use of POCUS, training programs and further research, which are urgently needed given the weak quality of evidence available.

Neonates with complex congenital heart disease (CHD) demonstrate microstructural brain dysmaturation, but the relationship with structural network topology is unknown. We performed diffusion tensor imaging (DTI) in term neonates with CHD pre-operatively (N = 61) and post-operatively (N = 50) compared to healthy term controls (N = 91). We used network topology (graph) analyses incorporating different weighted and unweighted approaches and subject-specific white matter segmentation to investigate structural topology differences, as well as a voxel-based analysis (VBA) to confirm the presence of microstructural dysmaturation. We demonstrate cost-dependent network inefficiencies in neonatal CHD in the pre-operative and post-operative period compared to controls, related to microstructural differences. Controlling for cost, we show the presence of increased small-worldness (hierarchical fiber organization) in CHD infants pre-operatively, that persists in the post-operative period compared to controls, suggesting the early presence of brain reorganization. Taken together, topological microstructural dysmaturation in CHD infants is accompanied by hierarchical fiber organization during a protracted critical period of early brain development. Our methodology also provides a pipeline for quantitation of network topology changes in neonates and infants with microstructural brain dysmaturation at risk for perinatal brain injury.

Gestational Diabetes Mellitus (GDM) is one of the main causes of perinatal mortality/morbidity. Today, a parameter offering useful information on fetal central nervous system (CNS) development/damage is eagerly awaited. We investigated the role of brain-protein S100B in the maternal blood of GDM pregnancies by means of a prospective case–control study in 646 pregnancies (GDM: n = 106; controls: n = 530). Maternal blood samples for S100B measurement were collected at four monitoring time-points from 24 weeks of gestation to term. Data was corrected for gender and delivery mode and correlated with gestational age and weight at birth. Results showed higher (p < 0.05) S100B from 24 to 32 weeks and at term in GDM fetuses than controls. Higher (p < 0.05) S100B was observed in GDM male new-borns than in females from 24 to 32 weeks and at term, in GDM cases delivering vaginally than by caesarean section. Finally, S100B positively correlated with gestational age and weight at birth (R = 0.27; R = 0.37, respectively; p < 0.01). The present findings show the usefulness of S100B in CNS to monitor high-risk pregnancies during perinatal standard-of-care procedures. The results suggest that further investigations into its potential role as an early marker of CNS growth/damage in GDM population are needed.