Myocardial function, mechanics and work by echocardiography in adolescents with severe obesity.

Introduction: Obesity and its metabolic complications can impact the heart's structure and function in childhood, although demonstrating this impact has been challenging. New echocardiographic parameters such as left atrial strain (LAε) and left ventricular strain (LVε), as well as myocardial work (MW), could reveal subclinical alterations in cardiac function. Objective: The aim is to evaluate the feasibility of these parameters in adolescents with severe obesity and explore their associations with body fat, metabolic comorbidities, and physical capacity. Methods: This is a cross-sectional study in adolescents with obesity who underwent echocardiography with analysis of LAε, LVε and MW using speckle tracking. Feasibility and association with anthropometry, body fat percentage, comorbidities and cardiopulmonary test were analyzed. Results: Twenty adolescents were recruited, 13 (65%) were males, median age 14.2 (interquartile range: 12.9-14.9) years old. The median Z-score for BMI (zBMI) was +3.03 (2.87-3.14), 14 (70%) had severe obesity (zBMI ≥+3), 12 (60%) body fat ≥95th percentile, 9 (45%) high blood pressure (HBP) and 8 (40%) metabolic syndrome. The analysis of the echocardiographic parameters was feasible in 95% (LAε) and 100% (LVε and MW). LVε was lower in adolescents with vs. without metabolic syndrome: 17.8% (17.5-19.3%) vs. 19.3% (18.3-20.3%), P = 0.046; and with vs. without HBP 17.8% (17.5-18.6%) vs. 19.7% (18.4-20.3%), P = 0.02. Those with body fat ≥95th percentile had lower LAε and MW parameters, without association with cardiopulmonary test. Conclusion: Echocardiographic evaluation of LAε, LVε and MW is feasible in adolescents with severe obesity. A higher proportion of body fat and the presence of comorbidities are associated with alterations in these new echocardiographic functional parameters suggesting myocardial impact of higher metabolic compromise.

Extubation failure after cardiac surgery in children with Down syndrome.

Extubation failure (EF) after cardiac surgery is associated with poorer outcomes. Approximately 50% of children with Down syndrome (DS) have congenital heart disease. Our primary aim was to describe the frequency of EF and identify risk factors for its occurrence in a population of patients with DS after cardiac surgery. Secondary aims were to describe complications, length of hospital stay, and mortality rates. This report was a retrospective case-control study and was carried out in a national reference congenital heart disease repair center of Chile. This study includes all infants 0-12 months old with DS who were admitted to pediatric intensive care unit after cardiac surgery between January 2010 and November 2020. Patients with EF (cases) were matched 1:1 with children who did not fail their extubation (controls) using the following criteria: age at surgery, sex, and type of congenital heart disease. Overall, 27/226 (11.3%) failed their first extubation. In the first analysis, before matching of cases and controls was made, we found association between EF and younger age (3.8 months vs 5 months; p = 0.003) and presence of coarctation of the aorta (p = 0.005). In the case-control univariate analysis, we found association between an increased cardiothoracic ratio (CTR) (p = 0.03; OR 5 (95% CI 1.6-16.7) for a CTR > 0.59) and marked hypotonia (27% vs 0%; p = 0.01) with the risk of EF. No differences were found in ventilatory management. CONCLUSIONS: In pediatric patients with DS, EF after cardiac surgery is associated with younger age, presence of aortic coarctation, higher CTR reflecting the degree of cardiomegaly and hypotonia. Recognition of these factors may be helpful when planning extubation for these patients. WHAT IS KNOWN: • Extubation failure after cardiac surgery is associated with higher morbidity and mortality rates. Some studies report higher rates of extubation failure in patients with Down syndrome. WHAT IS NEW: • In children with Down syndrome, extubation failure after cardiac surgery is associated with younger age, presence of aortic coarctation, higher CTR reflecting cardiomegaly and severe hypotonia.

COVID-19 Positive Versus Negative Complete Kawasaki Disease: A Study from the International Kawasaki Disease Registry.

To determine clinical differences for children with complete Kawasaki disease (KD) with and without evidence of preceding SARS-CoV-2 infection. From January 2020, contemporaneous patients with complete KD criteria were classified as either SARS-CoV-2 positive (KDCOVID+; confirmed household exposure, positive PCR and/or serology) or SARS-CoV-2 negative (KDCOVID-; negative testing and no exposure) and compared. Of 744 patients in the International Kawasaki Disease Registry, 52 were KDCOVID- and 61 were KDCOVID+. KDCOVID+ patients were older (median 5.5 vs. 3.7 years; p < 0.001), and all additionally met diagnostic criteria for multisystem inflammatory syndrome in children (MIS-C). They were more likely to have abdominal pain (60% vs. 35%; p = 0.008) and headache (38% vs. 10%; p < 0.001) and had significantly higher CRP, troponin, and BUN/creatinine, and lower hemoglobin, platelets, and lymphocytes. KDCOVID+ patients were more likely to have shock (41% vs. 6%; p < 0.001), ICU admission (62% vs. 10%; p < 0.001), lower left ventricular ejection fraction (mean lowest LVEF 53% vs. 60%; p < 0.001), and to have received inotropic support (60% vs. 10%; p < 0.001). Both groups received IVIG (2 doses in 22% vs. 18%; p = 0.63), but KDCOVID+ were more likely to have received steroids (85% vs. 35%; p < 0.001) and anakinra (60% vs. 10%; p = 0.002). KDCOVID- patients were more likely to have medium/large coronary artery aneurysms (CAA, 12% vs. 0%; p = 0.01). KDCOVID+ patients differ from KDCOVID-, have more severe disease, and greater evidence of myocardial involvement and cardiovascular dysfunction rather than CAA. These patients may be a distinct KD phenotype in the presence of a prevalent specific trigger.

Diagnóstico ecocardiográfico de hipertrofia ventricular izquierda en población pediátrica según método de normalización de masa ventricular e impacto del estado nutricional / Echocardiographic diagnosis of left ventricular hypertrophy in children according to the ventricular mass normalization method and impact of nutritional status

Antecedentes: la presencia de hipertrofia ventricular izquierda (HVI) es un marcador pronóstico y de severidad en condiciones de sobrecarga de presión. Se determina por masa ventricular (MV) aumentada en el ecocardiograma, debiéndose ajustar la MV por tamaño corporal en pediatría (normalización). Existen múltiples métodos de normalización, pero se desconoce si estos métodos son comparables. Objetivo: comparar distintos métodos de normalización de MV en sujetos con y sin sobrecarga de presión y evaluar el impacto del estado nutricional en el diagnóstico de HVI. Métodos: estudio de corte transversal en niños entre 5-18 años, divididos en 3 grupos: A) sin sobrecarga de presión (SSP), B) HTA (confirmado o sospecha), o C) Con cardiopatía obstructiva izquierda (CCOI, gradiente ≥25 mmHg). Se analizó antropometría, presión arterial y parámetros ecocardiográficos convencionales. Se determinó HVI por 4 mé-todos de normalización de MV: 1) Superficie corporal (SC) según sexo, 2) Talla2,7>51g/m2,7 3) Z-Score de Talla ≥ Z+2 y 4) Masa corporal magra ≥ Z+2. Se evaluó la concordancia de los métodos por grupo y según el diagnóstico nutricional. Resultados: se analizaron 1162 sujetos, 673(57,9%) hombres, edad 10,2 ± 3,2 años, 852(73,3%) SSP, 192(16,5%) con HTA y 118(10,2%) con CCOI. Un 38,6% presentaba malnutrición por exceso. Se observó diferencia entre los métodos para HVI en pacientes SSP y con CCOI(p=0,01), y en obesos con CCOI. La concordancia entre los métodos fue variable (rango de Kappa 0,38­0,71). Conclusiones: existe variabilidad y discrepancia entre los distintos métodos de normalización utilizados para definir HVI, influenciados por el estado nutricional.

Background: The presence of left ventricular hypertrophy (LVH) is both prognostic and severity marker in pressure overload conditions. It is determined by increased ventricular mass (MV) in the echocardiogram. MV must be adjusted for body size in pediatrics (normali-zation). There are multiple normalization methods, but it is unknown whether these methods are comparable. Objective: To compare different methods of MV normalization in subjects with and without pressure overload and to evaluate the impact of nutritional status on LVH diagnosis. Methods: Cross-sectional study in children aged 5-18 years, divided into 3 groups: A) without pressure overload (SSP), B) Systemic hypertension (confirmed or suspected), or C) Left obstructive heart disease (CCOI, gradient ≥ 25mmHg). Anthropometrics, blood pressure, and conventional echocardiographic parameters were analyzed. LVH was determined by 4 MV normalization methods: 1) Body surface area (BSA) adjusted by gender, 2) height2.7, 3) Z-Score height ≥ Z+2, and 4) lean body mass ≥ Z+2. The concordance of the methods was evaluated by group and according to the nutritional diagnosis. Results: 1162 subjects were analyzed, 673(57.9%) men, age 10.2 ± 3.2 years, 852(73.3%) SSP, 192(16.5%) with hypertension and 118(10.2%) with CCOI. 38.6% presented overnutrition. A difference was demonstrated between the methods for LVH diagnosis in patients with SSP and with CCOI (p=0.01), and in obese patients with CCOI. Agreement between methods was variable (Kappa range 0.38­0.71). Conclusions: There is variability and discrepancy between the different normalization methods used to define LVH, influenced by nutritional status.

Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve.

BACKGROUND: Bicuspid aortic valve (BAV) is the most common congenital heart disease, often associated with valve dysfunction, coarctation of the aorta, and ascending aorta dilatation. Aortic dilatation might result from abnormal regional hemodynamics or inherent vascular disease. Vascular function in pediatric BAV remains poorly characterized. METHODS: A cross-sectional study was performed to evaluate vascular function in 142 children with BAV aged 7-18 years compared with healthy control children. Echocardiography was performed to assess aortic dimensions, BAV function, and vascular function (aortic arch pulse wave velocity [PWV]), carotid intima media thickness, and aortic stiffness and distensibility). Carotid-femoral and carotid-radial PWV were assessed using tonometry. Vascular function was compared for 4 patient groups stratified according to aortic dilatation and a history of coarctation of the aorta. Multivariate regression analysis was performed to determine predictors of aortic dilatation. RESULTS: Children with BAV had stiffer and less distensible ascending aortas with higher aortic arch PWV compared with control children. Carotid-femoral and carotid-radial PWV were not increased in patients with BAV, and the vascular assessment of the abdominal aorta was unremarkable. Multivariate regression revealed that aortic arch PWV was the only vascular function parameter that was associated with aortic dilatation. CONCLUSIONS: Children with BAV have differences in vascular function that are confined to their proximal aorta, even in normal functioning BAV. The observed differences in vascular function are likely multifactorial, with contributions from abnormal regional flow and a potential localized primary aortopathy.

Preparación de los colegios y profesores de educación física en prevención de muerte súbita y soporte vital básico / Competence of schools and physical education teachers for sudden death prevention and basic life support

OBJETIVO: Conocer el nivel de preparación de colegios y profesores de educación física en prevención de muerte súbita (MS) y soporte vital básico, incluyendo disponibilidad y uso del desfibrilador externo automático (DEA). MÉTODOS: Estudio descriptivo de corte transversal, en profesores de educación física de establecimientos educacionales de distintos tipos de sostenedores (municipales, subvencionados y particulares) de Santiago, Chile, a través de un cuestionario online. Se analizaron datos demográficos del profesor, antecedentes de preparación y disposición de reanimación cardiopulmonar (RCP), presencia y uso de DEA y preparación del establecimiento ante un caso de MS. RESULTADOS: De 97 profesores encuestados, 71,1% no se siente capacitado para realizar RCP a pesar que un 70% del total ha realizado un curso. La disposición para realizar reanimación en aquellos que han realizado un curso, es 99%, comparado con 83% en quienes no lo han realizado (p=0,003). Existe una marcada diferencia en disponibilidad de DEA según tipo de sostenedor (52,4% en particulares, 29,6% en municipales y 15% en subvencionados, p=0,001), pero transversalmente no saben cómo utilizarlo (42,9 %, 40,7% y 25%, p=0,43). La mayoría de los colegios no cuenta con un plan de acción ante MS. CONCLUSIONES: Los colegios y profesores de educación física tienen una preparación insuficiente y desactualizada en prevención de muerte súbita y soporte vital básico, con una distribución heterogénea de DEA en establecimiento según tipo de sostenedor.

OBJECTIVE: To assess the competence of schools and physical education teachers in the prevention of sudden death (SD) and basic life support meassures, including availability and use of the automatic external defibrillator (AED). METHODS: A descriptive cross-sectional study included physical education teachers from different Schools (public, subsidized and private) in Santiago, Chile. An online questionnaire was applied including demographic data of the teacher, comentence and willingness to perform cardiopulmonary resuscitation (CPR), presence and use of AED and school preparation in case of a SD. RESULTS: From a total of 97 teachers surveyed do not feel capable of performing CPR, in spite of the fact that 70% of them completed a CPR course. Among those who took a course, their willingness to perform resuscitation was 99%, compared to 83% en those not having taken the course (p = 0.003). There was a marked difference in DEA availability according to class of school (private 52,4%, public 29,6 subsidized 15%, p=0,001), but the knowledge on how to use de DEA was uniformly insufficient (42,9 %, 40,7% and 25%, p=0,43) Most schools do not have a protocol to face SD. CONCLUSIONS: We observed that schools and physical education teachers have an insufficient and outdated preparation in sudden death prevention and basic life support. The availability of AED differed according to the class of establishment.

Management of Multisystem Inflammatory Syndrome in Children Associated With COVID-19: A Survey From the International Kawasaki Disease Registry.

BACKGROUND: Since April 2020, there have been numerous reports of children presenting with systemic inflammation, often in critical condition, and with evidence of recent infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This condition, since defined as the multisystem inflammatory syndrome in children (MIS-C), is assumed to be a delayed immune response to coronavirus disease 2019 (COVID-19), and there are frequently cardiac manifestations of ventricular dysfunction and/or coronary artery dilation. METHODS: We surveyed the inpatient MIS-C management approaches of the members of the International Kawasaki Disease Registry across 38 institutions and 11 countries. RESULTS: Among the respondents, 56% reported using immunomodulatory treatment for all MIS-C patients, regardless of presentation. Every respondent reported use of intravenous immunoglobulin (IVIG), including 53% administering IVIG in all patients. Steroids were most often used for patients with severe clinical presentation or lack of response to IVIG, and only a minority used steroids in all patients (14%). Acetylsalicylic acid was frequently used among respondents (91%), including anti-inflammatory and/or antiplatelet dosing. Respondents reported use of prophylactic anticoagulation, especially in patients at higher risk for venous thromboembolism, and therapeutic anticoagulation, particularly for patients with giant coronary artery aneurysms. CONCLUSIONS: There is variation in management of MIS-C patients, with suboptimal evidence to assess superiority of the various treatments; evidence-based gaps in knowledge should be addressed through worldwide collaboration to optimize treatment strategies.

CONTEXTE: Depuis avril 2020, de nombreux cas d'enfants présentant une inflammation généralisée, se trouvant souvent dans un état critique et montrant des signes d'une infection récente au coronavirus du syndrome respiratoire aigu sévère 2 (SRAS-CoV-2), ont été signalés. On pense que cet état, désigné depuis sous le nom de syndrome inflammatoire multisystémique de l'enfant (SIME), pourrait être une réponse immunitaire tardive au virus de la maladie à coronavirus 2019 (COVID-19); les patients présentent souvent des manifestations cardiaques associées à une dysfonction ventriculaire ou à une dilatation des artères coronaires. MÉTHODOLOGIE: Nous avons mené un sondage sur les stratégies de prise en charge du SIME en milieu hospitalier auprès des membres du registre international de la maladie de Kawasaki, qui sont rattachés à 38 établissements répartis dans 11 pays. RÉSULTATS: Au total, 56 % des répondants ont déclaré opter pour un traitement immunomodulateur pour tous les patients présentant un SIME, quelles qu'en soient les manifestations. Tous les répondants ont déclaré avoir recours à l'administration d'immunoglobulines par voie intraveineuse, 53 % d'entre eux utilisant ce traitement chez tous les patients. Les stéroïdes étaient plus souvent utilisés chez les patients présentant des symptômes cliniques graves ou ne répondant pas aux immunoglobulines administrées par voie intraveineuse; seule une minorité de répondants ont déclaré utiliser des stéroïdes chez tous les patients (14 %). Les répondants utilisaient aussi fréquemment l'acide acétylsalicylique (91 %), à des doses anti-inflammatoires ou antiplaquettaires. Ils ont en outre déclaré avoir recours à des anticoagulants en prophylaxie, en particulier chez les patients présentant un risque élevé de thromboembolie veineuse, et à une anticoagulothérapie chez les patients présentant des anévrismes coronaires géants. CONCLUSIONS: La prise en charge des patients présentant un SIME varie d'un médecin à l'autre, et les données permettant d'évaluer la supériorité des divers traitements employés sont insuffisantes; il conviendrait donc de mettre en place des initiatives de collaboration afin de combler les lacunes des connaissances et d'optimiser les stratégies thérapeutiques.

Talla baja en niños con defectos cardíacos congénitos sometidos a cirugía cardíaca / Short stature among children undergoing cardiac surgery for congenital heart defects

Introducción. Los niños con cardiopatías congénitas (CC) presentan malnutrición por déficit; una posible consecuencia a largo plazo es la talla baja. Objetivo. Describir la presencia de talla baja en niños con CC al momento de su cardiocirugía. Población y métodos. Estudio retrospectivo. Se incluyeron niños sometidos a cardiocirugía con circulación extracorpórea en 2009-2013. Se excluyeron prematuros, con síndromes genéticos u otra enfermedad con compromiso nutricional. Se estudiaron variables demográficas, diagnóstico cardiológico, cirugía de ingreso y evaluación antropométrica según estándares de la Organización Mundial de la Salud; se definió talla baja como ZT/E < -2 desvíos estándar, según sexo. Resultados. Se estudiaron 640niños; 361 varones (el 56,4 %); mediana de edad: 8 meses (RIC: 1,9; 34,6); 66 niños tuvieron > 1 cirugía; 27 de ellos (el 40,9 %), con hipoplasia del ventrículo izquierdo. Fueron CC cianóticas 358 (el 55,9 %), con fisiología univentricular 196 (el 30,6 %). La mediana de ZT/E fue -0,9 (RIC: -1,9; -0,1); presentaron talla baja 135 (el 21,1 %), el 11 % en neonatos y el 24,1 % en mayores de un mes. Se encontró mayor frecuencia de talla baja en reparación completa de canal atrioventricular en 4/6 niños, reparación de tetralogía de Fallot en 15/39, Glenn en hipoplasia del ventrículo izquierdo en 8/25, cierre de comunicación interventricular en 34/103. No se encontró asociación ni diferencia con significación estadística entre talla baja y cianosis ni según fisiología univentricular. Conclusiones. Existe una alta frecuencia de talla baja en niños con CC, con diferencias según el diagnóstico cardiológico y la cardiocirugía realizada.

Introduction. Children with congenital heart diseases (CHDs) suffer from malnutrition because of nutritional deficiencies, being short stature the possible long-term consequence. Objective. To describe the presence of short stature among children undergoing cardiac surgery for CHDs. Population and methods. Retrospective study. Children undergoing cardiac surgery with cardiopulmonary bypass pump between 2009 and 2013 were included. Preterm infants, carriers of genetic syndromes or other disease with nutritional compromise were excluded. Demographic data, type of CHD, admission surgery and anthropometric assessment using the WHO standards were studied. Short stature was defined as lenght/height for age Z score < -2 standard deviations, by sex. Results. A total of 640 children were studied; 361 (56.4 %) were boys; median age: 8 months (IQR: 1.9; 34.6); 66 children underwent > 1 surgery; 27 of them (40.9 %) had hypoplasia of the left ventricle. There were 358 (55.9 %) infants with cyanotic CHDs, 196 (30.6 %) with univentricular physiology. The median HAZ was -0.9 (IQR: -1.9; -0.1); 135 (21.1 %) had a short stature, 11 % of newborn infants and 24.1 % of older than one month old. A higher frequency of short stature was observed in 4 out of 6 children who underwent complete repair of the atrioventricular canal, in 15 out of 39 infants with repair of tetralogy of Fallot, in 8 out of 25 infants with hypoplasia of the left ventricle subjected to Glenn procedure, and in 34 out of 103 with closure of the ventricular septal defect. No association or statistically significant difference was found between short stature and cyanosis or univentricular physiology. Conclusions. There is a high frequency of short stature among children with CHDs, with differences according to the type of CHD and cardiac surgery performed.

Short stature among children undergoing cardiac surgery for congenital heart defects. / Talla baja en niños con defectos cardíacos congénitos sometidos a cirugía cardíaca.

Introduction: Children with congenital heart diseases (CHDs) suffer from malnutrition because of nutritional deficiencies, being short stature the possible long-term consequence. Objective: To describe the presence of short stature among children undergoing cardiac surgery for CHDs. Population and methods: Retrospective study. Children undergoing cardiac surgery with cardiopulmonary bypass pump between 2009 and 2013 were included. Preterm infants, carriers of genetic syndromes or other disease with nutritional compromise were excluded. Demographic data, type of CHD, admission surgery and anthropometric assessment using the WHO standards were studied. Short stature was defined as lenght/height for age Z score < -2 standard deviations, by sex. Results: A total of 640 children were studied; 361 (56.4 %) were boys; median age: 8 months (IQR: 1.9; 34.6); 66 children underwent > 1 surgery; 27 of them (40.9 %) had hypoplasia of the left ventricle. There were 358 (55.9 %) infants with cyanotic CHDs, 196 (30.6 %) with univentricular physiology. The median HAZ was -0.9 (IQR: -1.9; -0.1); 135 (21.1 %) had a short stature, 11 % of newborn infants and 24.1 % of older than one month old. A higher frequency of short stature was observed in 4 out of 6 children who underwent complete repair of the atrioventricular canal, in 15 out of 39 infants with repair of tetralogy of Fallot, in 8 out of 25 infants with hypoplasia of the left ventricle subjected to Glenn procedure, and in 34 out of 103 with closure of the ventricular septal defect. No association or statistically significant difference was found between short stature and cyanosis or univentricular physiology. Conclusions: There is a high frequency of short stature among children with CHDs, with differences according to the type of CHD and cardiac surgery performed.

Introducción. Los niños con cardiopatías congénitas (CC) presentan malnutrición por déficit; una posible consecuencia a largo plazo es la talla baja. Objetivo. Describir la presencia de talla baja en niños con CC al momento de su cardiocirugía. Población y métodos. Estudio retrospectivo. Se incluyeron niños sometidos a cardiocirugía con circulación extracorpórea en 2009-2013. Se excluyeron prematuros, con síndromes genéticos u otra enfermedad con compromiso nutricional. Se estudiaron variables demográficas, diagnóstico cardiológico, cirugía de ingreso y evaluación antropométrica según estándares de la Organización Mundial de la Salud; se definió talla baja como ZT/E < -2 desvíos estándar, según sexo. Resultados. Se estudiaron 640niños; 361 varones (el 56,4 %); mediana de edad: 8 meses (RIC: 1,9; 34,6); 66 niños tuvieron > 1 cirugía; 27 de ellos (el 40,9 %), con hipoplasia del ventrículo izquierdo. Fueron CC cianóticas 358 (el 55,9 %), con fisiología univentricular 196 (el 30,6 %). La mediana de ZT/E fue -0,9 (RIC: -1,9; -0,1); presentaron talla baja 135 (el 21,1 %), el 11 % en neonatos y el 24,1 % en mayores de un mes. Se encontró mayor frecuencia de talla baja en reparación completa de canal atrioventricular en 4/6 niños, reparación de tetralogía de Fallot en 15/39, Glenn en hipoplasia del ventrículo izquierdo en 8/25, cierre de comunicación interventricular en 34/103. No se encontró asociación ni diferencia con significación estadística entre talla baja y cianosis ni según fisiología univentricular. Conclusiones. Existe una alta frecuencia de talla baja en niños con CC, con diferencias según el diagnóstico cardiológico y la cardiocirugía realizada.

Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ±â€¯6.4% vs. 48.5 ±â€¯7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 ±â€¯17.7% vs. 75.6 ±â€¯20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ±â€¯3.9 vs.-25.7 ±â€¯4.4, P < .001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.

Quantification of Right Ventricular Electromechanical Dyssynchrony in Relation to Right Ventricular Function and Clinical Outcomes in Children with Repaired Tetralogy of Fallot.

BACKGROUND: Electromechanical dyssynchrony occurs ubiquitously following tetralogy of Fallot (TOF) repair, manifesting electrically as a wide QRS duration and mechanically as a right-sided septal/apical flash. Early septal activation and prestretch of the right ventricular (RV) basal lateral wall followed by its postsystolic shortening contributes to inefficient RV mechanics. However, a right-sided septal flash is a dichotomous finding, and the severity of RV dyssynchrony as a continuous spectrum in relationship to RV dysfunction and clinical outcomes in patients with repaired TOF has not been studied. The aim of this study was to quantify the severity of electromechanical dyssynchrony in relation to RV remodeling and clinical outcomes in a pediatric cohort following TOF repair. METHODS: A retrospective analysis was performed in 81 children with RV volume loading after TOF repair, aged 13.6 ± 2.9 years, and compared with 50 matched control subjects. RESULTS: Patients had higher RV basal-lateral prestretch and postsystolic strain amplitude and duration, RV mechanical dispersion, and basal lateral-septal wall delay compared with control subjects (P < .001 for all). All intra-RV dyssynchrony timing parameters were associated with reduced cardiac magnetic resonance-derived RV ejection fraction and/or echocardiography-derived RV longitudinal strain. Prestretch duration as a percentage of total shortening time and RV basal lateral-to-midseptal delay were independently associated with RV dysfunction. Postsystolic strain amplitude was higher in patients with ventricular arrhythmias compared with arrhythmia-free patients (7.8% [4.2%-13%] vs 2.0% [0%-12.5%], P = .03). CONCLUSION: RV prestretch duration, postsystolic strain, and RV lateral-septal delay quantify RV electromechanical dyssynchrony severity and reflect the underlying pathophysiology. The prestretch duration percentage and RV basal lateral-to-midseptal delay were independently associated with RV dysfunction, potentially providing a clinical tool to quantify RV electromechanical dyssynchrony.

Imaging in repaired tetralogy of Fallot with a focus on recent advances in echocardiography.

PURPOSE OF REVIEW: Imaging is essential for the management of adults with repaired tetralogy of Fallot (rToF). Echocardiography and cardiac magnetic resonance imaging are the central modalities to assess rToF. Here we review recent literature on imaging rToF, focusing on echocardiography and advances in assessment of cardiac mechanics. RECENT FINDINGS: Several two-dimensional, three-dimensional, and Doppler echo parameters have been proposed to assess pulmonary regurgitation, right ventricular volumes and ejection fraction, but most of them still have important limitations in their feasibility and reliability compared to cardiac magnetic resonance (CMR). Myocardial deformation imaging to study ventricular and atrial mechanics, regional function, ventricular-ventricular interactions, and electro-mechanical dyssynchrony has yielded insights into the pathophysiologic mechanisms of right ventricular and left ventricular dysfunction; thereby predicting clinical outcomes and exercise capacity, allowing among others, evaluation of the impact of pulmonary valve replacement (PVR). Emerging technologies are expected to further our understanding of the drivers of dysfunction and guide indications and timing of PVR. SUMMARY: Echocardiography and CMR have complementary and overlapping roles in rToF and contribute to our understanding of its pathophysiology and management.

Down's syndrome is a risk factor for severe lower respiratory tract infection due to respiratory syncytial virus.

AIM: Previous studies have suggested that Down's syndrome is an independent risk factor for severe respiratory infection due to respiratory syncytial virus (RSV). We compared the clinical characteristics of children with and without Down's syndrome hospitalised due to RSV. METHODS: This retrospective cohort study compared data from hospitalisations due to RSV lower respiratory tract infections (LRTI) in children under 14 years of age with (n = 58) and without (n = 58) Down's syndrome. RESULTS: The Down's group had longer hospital stays than the controls of six versus four days (p < 0.0001), even after adjusting for age, weeks of gestation at birth, presence of asthma, bronchopulmonary dysplasia, haemodynamically significant and nonsignificant congenital heart disease. This difference increased when only children under one year of age were analysed to 11 versus five days (p < 0.0001). Children with Down's syndrome were more likely to be admitted to intensive care unit (43.1% versus 22.4%, p = 0.017), need noninvasive mechanical ventilation (36.2% versus 13.7%, p = 0.005) and be prescribed antibiotics and steroids. CONCLUSION: Children with Down's syndrome hospitalised due to RSV LRTI had longer hospital stays and worse clinical courses than controls, highlighting the need for RSV prophylaxis for children with Down's syndrome, especially under one year of age.

Ambiente educacional y calidad de la docencia en la escuela de medicina / Eucational environment and quality of teaching in our school of medicine

El ambiente educacional es un concepto que cada vez cobra mayor relevancia en la educación médica por su impacto en el proceso de enseñanza, aprendizaje y posterior vida laboral. Existen numerosos instrumentos para evaluar el ambiente, según el ciclo de formación o el tipo de rotación. En la Escuela de Medicina de la Pontificia Universidad Católica de Chile se han utilizado las encuestas DREEM y PHEEM, que se complementan con otros instrumentos aplicados a los estudiantes, generados por el Centro de Educación Médica y la American Association of Medical Colleges (AAMC) en el contexto del proceso de la evaluación de la Escuela de Medicina. Todos estos confirman que el ambiente educacional en general es adecuado. Las fortalezas detectadas están relacionadas con el ambiente académico y la calidad de los profesores. Los aspectos deficitarios están relacionados con la retroalimentación, el proceso de evaluación y áreas de desarrollo social. La constante medición del ambiente educacional dentro de la Escuela de Medicina nos permitirá a futuro detectar las fortalezas, mejorar las áreas deficitarias y evaluar el impacto de las modificaciones curriculares.

The Educational Environment has increased its relevance in medical education due to the impact in the process of teaching and learning and later professional work. There are numerous instruments to evaluate the environment, according to the cycle of formation or the kind of rotation. In the Medical School of Pontificia Universidad Católica de Chile it has been used the DREEM and PHEEM questionnaires, which complement with other instruments applied to the students, developed by the Center of Medical Education and the American Association of Medical Colleges (AAMC) in the context of the School of Medicine evaluation. All these instruments confirm that the educational environment in general is good. The strengths are related to the academic atmosphere and the quality of teachers. The weaknesses are related to feedback, evaluation and areas related to social development. The measurement of the educational climate within the Medical School will allow in the future detecting the strengths, in order to improve the deficit areas and to evaluate the impact of curricular changes.