Estudio de la torsión de ventrículo izquierdo mediante "Speckle tracking" por ecocardiografía en niños

Comprender la función cardíaca desde la interpretación de técnicas de imagen como la ecocardiografía es fundamental en la práctica clínica diaria en el ámbito de la cardiología tanto en adultos como en pediatría. La aparición de nuevas técnicas, como la imagen Doppler tisular o el speckle tracking (su equivalente en español sería seguimiento de puntos), aportan herramientas que nos acercan a comprender mejor la mecánica cardíaca. La obtención de parámetros de motilidad o deformación de las paredes ventriculares pueden conectar la fisiología con la arquitectura tridimensional. Los mecanismos finales de la arquitectura miocárdica no se conocen claramente con una teoría en la que la de la presencia de capas de fibras helicoidales que formarían una banda única esta discutida por otras teorías de que el miocardio es una matriz tridimensional. La capacidad de estudiar y cuantificar el movimiento del ventrículo izquierdo en diferentes direcciones del espacio, comparar diferentes zonas desde el endocardio al epicardio y de la base al ápex y demostrar y cuantificar la rotación y la torsión nos permite acercarnos a esa estructura que formada por unidades como el miocito que cambiando un 15% de su longitud produce un 60% de fracción de eyección del ventrículo izquierdo. A su vez se conoce que el estudio de la mecánica cardiaca nos aporta un mejor entendimiento de la fisiopatología en condiciones preclínicas que actualmente no son detectadas por los parámetros clásicos de función. Desde que la investigación básica descubrió la tecnología speckle tracking a su desarrollo con aplicaciones clínicas en los últimos equipos ecocardiográficos no han pasado muchos años..

Safety and preliminary efficacy on cognitive performance and adaptive functionality of epigallocatechin gallate (EGCG) in children with Down syndrome. A randomized phase Ib clinical trial (PERSEUS study)

Purpose: Although some caregivers are using epigallocatechin gallate (EGCG) off label in hopes of improving cognition in young adults with Down syndrome (DS), nothing is known about its safety, tolerability, and efficacy in the DS pediatric population. We aimed to evaluate safety and tolerability of a dietary supplement containing EGCG and if EGCG improves cognitive and functional performance. Methods: A total of 73 children with DS (aged 6-12 years) were randomized. Participants received 0.5% EGCG (10 mg/kg daily dose) or placebo for 6 months with 3 months follow up after treatment discontinuation. Results: In total, 72 children were treated and 66 completed the study. A total of 38 participants were included in the EGCG group and 35 in the placebo group. Of 72 treated participants, 62 (86%) had 229 treatment-emergent adverse events (AEs). Of 37 participants in the EGCG group, 13 (35%) had 18 drug-related treatment-emergent AEs and 12 of 35 (34%) from the placebo group had 22 events. In the EGCG group, neither severe AEs nor increase in the incidence of AEs related to safety biomarkers were observed. Cognition and functionality were not improved compared with placebo. Secondary efficacy outcomes in girls point to a need for future work. Conclusion: The use of EGCG is safe and well-tolerated in children with DS, but efficacy results do not support its use in this population. (C) 2022 The Authors. Published by Elsevier Inc. on behalf of American College of Medical Genetics and Genomics

Palivizumab in the prevention of severe respiratory syncytial virus infection in children with congenital heart disease; a novel cost-utility modeling study reflecting evidence-based clinical pathways in Spain

Abstract Background Respiratory syncytial virus (RSV) infection remains one of the major reasons of re-hospitalization among children with congenital heart disease (CHD). This study estimated the cost-effectiveness of palivizumab prophylaxis versus placebo, in Spain, from the societal perspective, using a novel cost-effectiveness model reflecting evidence-based clinical pathways. Methods A decision-analytic model, combining a decision tree structure in the first year and a Markov structure in later years, was constructed to evaluate the benefits and costs associated with palivizumab versus no prophylaxis among children with CHD. In the first year of the model, children were at risk of mild (i.e. medically attended, MA-RSV) and severe (hospitalized, RSV-H) RSV infection. The impact of delayed corrective CHD surgery due to RSV infection and the consequence of performed surgery despite severe infection were considered. In later years, patients were at risk of developing asthma and allergic sensitization as sequelae of RSV infection. Input data for the model were derived from the pivotal clinical trial and systematic literature reviews. Indirect costs included parental absence from work and nosocomial infections. In agreement with Spanish guidelines, costs and effects were discounted at 3%. Results Over a lifetime horizon, palivizumab prophylaxis yielded 0.11 and 0.07 additional quality-adjusted life years (QALYs) and life years (LYs), respectively, at additional costs of € 1,693, resulting in an ICER of € 15,748 per QALY gained and € 24,936 per LY gained. Probabilistic sensitivity analyses demonstrated that the probability of palivizumab prophylaxis being cost-effective at a € 30,000 per QALY threshold was 92.7%. The ICER remained below this threshold for most extreme scenario analyses. Conclusions The model demonstrated that palivizumab prophylaxis results in more QALYs than no prophylaxis in children with CHD. Palivizumab prophylaxis was shown to be a cost-effective health care intervention according to the commonly accepted standards of cost-effectiveness in Spain (ICER below the threshold of € 30,000 per QALY)

Pediatric Catheter Ablation: Characteristics and Results of a Series in a Tertiary Referral Hospital

Introduction and objectives: Catheter ablation has become the treatment of choice in an increasing number of arrhythmias in children and adolescents. There is still limited evidence of its use at a national level in Spain. The aim was to describe the characteristics and results of a modern monocentric series form a referral tertiary care centre. Methods: Retrospective register of invasive procedures between 2004 and 2016 performed in patients under 17 years and recorded clinical characteristic, ablation methodology and acute and chronic results of the procedure. Results: A total of 291 procedures in 224 patients were included. Median age was 12.2 years, 60% male. Overall, 46% patients were referred from other autonomous communities. The most frequent substrates were accessory pathways (AP) (70.2%,>50% septal AP localization) and atrioventricular nodal reentrant tachycardia (AVNRT) (15.8%). Congenital and acquired heart disease was frequent (16.8%). Cryoablation was used in 35.5% of the cases. Overall acute success of the primary procedure was 93.5% (AP 93.8%; AVNRT 100%). Redo procedures after recurrence were performed in 18.9% of all substrates, with a long-term cumulative efficacy of 98.4% (AP 99.3%; AVNRT 100%). One (0.37%) serious complication occurred, a case of complete atrioventricular block. Conclusions: Our study replicated previous international reports of high success rates with scarce complications in a high complexity series, confirming the safety and efficacy of pediatric catheter ablation in our environment performed at highly experienced referral centers.Instituto de Investigación Carlos IIIFondo Europeo de Desarrollo RegionalDepto. de MedicinaFac. de MedicinaTRUEpu

Trasplante después del Fontan. Aspectos quirúrgicos

Introducción y objetivos: El trasplante cardiaco tras un Fontan supone un reto quirúrgico. Presentamos a 10 pacientes, destacando las variantes técnicas en las anastomosis previas a la implantación del injerto. Métodos: Recogemos 8 casos de Fontan y 2 «take-down» consecutivos durante 32 meses, con medianas de edad (9 años), peso (30 kg) e intervalo entre Fontan y trasplante de 3 años. Siete pacientes presentaban conducto extracardiaco (diámetro 18/20), 2 conexión aurículo-pulmonar y uno, túnel lateral. Tres niños desarrollaron enteropatía pierde-proteínas. Nueve «stents» fueron implantados previamente. Resultados: Hubo modificaciones en las 5 anastomosis. Una vez en la aurícula izquierda (sutura en ambas aurículas del receptor), 2 en la aorta (hemiarco), 2 en vena cava superior (plastia en vena innominada), 7 veces en la arteria pulmonar (parche de aorta/pericardio donante de hilio a hilio tras retirada de «stents»), vena cava inferior en 7 ocasiones (manguito de conducto). El seguimiento fue completo con una mediana de 25 meses (rango 3-34). Un paciente falleció. Dos precisaron extracorporeal membrane oxygenation por hipertensión pulmonar. Tres pacientes requirieron cierre de colaterales en hemodinámica, incluyendo colocación de «stent» en cava superior (1) y aorta (1). Se resolvió la enteropatía pierde-proteínas en 2 pacientes. Conclusiones: Trasplantar a pacientes con Fontan previo es un reto. Podemos anticipar variantes en cada una de las 5 anastomosis previstas. Es recomendable obtener tejido extra del donante (aorta y arco, vena cava superior e innominada, pericardio). Los resultados pueden superponerse a otras series de trasplante cardiaco