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Antecedentes: El síndrome de QT largo es una canalopatía que afecta a la repolarización ventricular y aumenta el riesgo de sufrir arritmias ventriculares graves. Puede ser congénito o adquirido, y es una causa conocida de muerte súbita. Caso clínico: Gestante primigesta, de 28 años, sin antecedentes de interés. En ecografías prenatales se objetivó en el feto bradicardia sinusal mantenida desde la semana 28, sin repercusión hemodinámica, que persistió hasta la finalización de la gestación (semana 37+3). Al nacimiento se realizaron electrocardiogramas seriados que mostraron alteraciones en la repolarización con alargamiento del intervalo QT corregido. Se realizó estudio genético que confirmó síndrome de QT largo tipo 1 y se inició tratamiento oral con beta-bloqueantes, con buena respuesta. Conclusiones: El síndrome de QT largo suele diagnosticarse posnatalmente. Es importante conocer sus características clínicas prenatales para poder establecer un diagnóstico precoz y minimizar así el riesgo de muerte súbita de estos pacientes.

Background: Long QT syndrome is a channelopathy that affects ventricular repolarization and increases the risk of severe ventricular arrhythmias. It can be congenital or acquired, and is a known cause of sudden cardiac death. Case report: A 28-year-old primigravida with no significant medical history. Prenatal ultrasounds revealed sustained fetal sinus bradycardia from week 28, without hemodynamic repercussion, which persisted until the end of gestation (at 37+3 weeks). Serial electrocardiograms were performed after birth, showing repolarization abnormalities with prolonged corrected QT interval. A genetic study confirmed long QT syndrome type 1, and oral treatment with beta-blockers was initiated, showing a positive response. Conclusions: Long QT syndrome is often diagnosed postnatally. It is important to be aware of his prenatal clinical features in order to establish an early diagnosis and minimize the risk of sudden death in these patients.

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Abstract Sudden cardiac death is a common occurrence. Out-of-hospital cardiac arrest is a global public health problem suffered by ≈3.8 million people annually. Progress has been made in the knowledge of this disease, its prevention, and treatment; however, most events occur in people without a previous diagnosis of heart disease. Due to its multifactorial and complex nature, it represents a challenge in public health, so it led us to work in a consensus to achieve the implementation of cardioprotected areas in Mexico as a priority mechanism to treat these events. Public access cardiopulmonary resuscitation (CPR) and early defibrillation require training of non-medical personnel, who are usually the first responders in the chain of survival. They should be able to establish a basic and efficient CPR and use of the automatic external defibrillator (AED) until the emergency services arrive at the scene of the incident. Some of the current problems in Mexico and alternative solutions for them are addressed in the present work.

Resumen La muerte súbita cardíaca (SCD) es un acontecimiento común. El paro cardiaco extrahospitalario (OHCA) es un problema de salud pública mundial que sufren ≈3.8 millones de personas al año. Se ha avanzado en el conocimiento de esta enfermedad, su prevención y tratamiento, sin embargo, la mayoría de los eventos se producen en personas sin diagnóstico previo de cardiopatía. Debido a su carácter multifactorial y complejo, representa un reto en salud pública, lo que obliga a trabajar en un consenso para lograr la implementación de "Espacios Cardio protegidos" en México, como mecanismo prioritario de atención a estos eventos. La reanimación cardiopulmonar básica (RCPB) y la desfibrilación temprana de acceso público requieren de entrenamiento al personal no médico, que suelen ser los primeros respondientes para iniciar la cadena de la supervivencia. Ellos deberían instaurar una RCPB eficiente y el uso del desfibrilador automático externo (AED) hasta que lleguen al lugar del incidente los servicios de emergencias. El presente trabajo menciona algunos de los problemas actuales en México y algunas opciones de solución para los mismos.

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Introducción: La hipoacusia súbita (HS) es poco frecuente y su etiopatogenia no está definida. La terapia con corticoides es de elección en base a recomendaciones de expertos por sus efectos teóricos y no en base a utilidad clínica demostrada. Objetivo: Evaluar si existe correlación entre el resultado auditivo final, de pacientes con HS tratados con corticoides, y la respuesta in vitro de sus leucocitos a corticoides, medida como diferencias en la expresión relativa de genes blanco del receptor de glucocorticoides. Material y Método: Estudio de casos (recuperación total) y controles (no recuperados) de pacientes con HS tratados con corticoides en el Hospital Clínico de la Universidad de Chile, durante 2017-2019. Se obtuvo DNA que fue almacenado en el Biobanco de Tejidos y Fluidos de la Universidad de Chile (BTUCH). Se purificaron y cultivaron leucocitos mononucleares de sangre periférica, expuestos in vitro a hidrocortisona. Se determinó la diferencia en la expresión relativa de genes blanco (IGFBP1, CAT, HSD17B12, APOA2), por Q-RTPCR, entre ambos grupos. Resultados: Se reclutaron 35 pacientes; se incluyeron para análisis 23: 11 casos y 12 controles, con edad promedio de 54,9 y 50,8 años respectivamente, distribución homogénea de sexo. No hubo diferencia estadísticamente significativa en la expresión relativa de los genes blanco, a la exposición in vitro a corticoides, entre ambos grupos. Conclusión: En nuestro estudio, modelo, y sistema de evaluación no se evidenciaron efectos de los corticoides. No podemos descartar que, con un número mayor de pacientes, otros genes blanco u otros protocolos de estudio podrían detectarse diferencias.

Introduction: Sudden hearing loss (SHL) is rare and its etiopathogenesis is still not clear. Corticosteroid therapy is of choice based on expert recommendations due to its theoretical effects and no based on proved clinical efficacy. Objectives: To assess whether there is a correlation between the final auditory outcome of patients with SHL treated with corticosteroids and the in vitro response of their leukocytes to corticosteroids, measured as differences in the relative expression of glucocorticoid receptor target genes. Material and Method: Case-control (total recovery and not recovered respectively) study of patients with SHL treated with corticosteroids at Clinical Hospital Universidad de Chile between 2017 and 2019. DNA was obtained and stored in the Biobanco de Tejidos y Fluidos de la Universidad de Chile (BTUCH). Peripheral blood mononuclear leukocytes were purified and cultured and then exposed to hydrocortisone. The difference in the relative expression of target genes (GFBP1, CAT, HSD17B12, APOA2), by Q-RTPCR was determined. Results: Thirty-five patients were recruited, 24 were included for the analysis: 11 cases and 12 controls, with and average age of 54.9 and 50.,8 years respectively, homogeneous sex distribution. There was no statistically significant difference in the relative expression of the target genes, upon in vitro exposure to corticosteroids, between both groups. Conclusion: In our study, model and evaluation system, no effects of corticosteroids were evidenced. With a larger number of patients, other target genes or other study protocols, we cannot rule out that differences could be detected.

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Resumen En Costa Rica, el Reglamento de la Autopsia Hospitalaria y Médico Legal establece que la autopsia médico legal es obligatoria para toda muerte súbita. La patología cardíaca es responsable aproximadamente del 80 % de las muertes súbitas que requieren una autopsia forense; el prolapso de la válvula mitral (degeneración mixomatosa de la válvula mitral) es una de las formas más comunes de valvulopatía cardíaca, es relativamente común (2%-3% de la población general), y a menudo se considera benigno, la tasa anual de muerte cardíaca súbita (MSC) en individuos con MVP (0,2%-0,4% /año) es aproximadamente el doble que el observado en la población general (0,1%-0,2% año).

Abstract In Costa Rica, the Hospital and Legal Autopsy Regulations establish that a legal medical autopsy is mandatory for all sudden deaths. Cardiac pathology is responsible for approximately 80% of sudden deaths requiring a forensic autopsy; Mitral valve prolapse (myxomatous mitral valve degeneration) is one of the most common forms of heart valve disease, is relatively common (2%-3% of the general population), and is often considered benign, the annual rate of Sudden cardiac death (SCD) in individuals with MVP (0.2%-0.4%/year) is approximately twice that observed in the general population (0.1%-0.2%/year).

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INTRODUCTION: Obstructive sleep apnea (OSA) is one of the main risk factors for cardiovascular diseases and is associated with both morbidity and mortality. OSA has also been linked to arrhythmias and sudden death. OBJECTIVE: To assess whether OSA increases the risk of sudden death in the non-cardiac population. METHODS: This is a systematic review of the literature. The descriptors "sudden death" and "sleep apnea" and "tachyarrhythmias" and "sleep apnea" were searched in the PubMed/Medline and SciELO databases. RESULTS: Thirteen articles that addressed the relationship between OSA and the development of tachyarrhythmias and/or sudden death with prevalence data, electrocardiographic findings, and a relationship with other comorbidities were selected. The airway obstruction observed in OSA triggers several systemic repercussions, e.g., changes in intrathoracic pressure, intermittent hypoxia, activation of the sympathetic nervous system and chemoreceptors, and release of catecholamines. These mechanisms would be implicated in the appearance of arrhythmogenic factors, which could result in sudden death. CONCLUSION: There was a cause-effect relationship between OSA and cardiac arrhythmias. In view of the pathophysiology of OSA and its arrhythmogenic role, studies have shown a higher risk of sudden death in individuals who previously had heart disease. On the other hand, there is little evidence about the occurrence of sudden death in individuals with OSA and no heart disease, and OSA is not a risk factor for sudden death in this population.

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ABSTRACT Objective To assess knowledge on sudden infant death syndrome (SIDS) prevention among postpartum women who received prenatal care in public and private services in Rio Grande, Rio Grande do Sul, Brazil, in 2019. Methods A cross-sectional study was conducted with postpartum women who gave birth in that municipality in 2019; the outcome was the indication of incorrect sleeping position (side/supine position) to prevent SIDS; the chi-square test was used to compare proportions between those who underwent prenatal care in public and private services. Results Among all 2,195 postpartum women, 67.7% (95%CI 65.7;69.6) were unaware of the position that prevents SIDS, 71.6% were public care service users; 77.8% of them feared choking/suffocation; 1.9% were informed about SIDS during prenatal care; doctors/nurses (70.5%) and grandmothers (65.1%) were influential regarding the baby's sleeping position. Conclusion Most postpartum women were unaware of the sleeping position that prevents SIDS, especially those receiving care in the public sector; in general, this subject is not discussed in prenatal care.

RESUMEN Objetivo Evaluar el conocimiento sobre la prevención del síndrome de muerte súbita del lactante (SMSL) entre puérperas que realizaron prenatal en servicios públicos y privados en Rio Grande, Rio Grande do Sul, Brasil, en 2019. Métodos Estudio transversal, con puérperas que dieron a luz en Rio Grande, en 2019; el resultado consistió en la indicación de posición incorrecta para dormir (lado/supino) para prevenir el SMSL; utilizando chi-cuadrado, se compararon las proporciones entre mujeres que recibieron atención prenatal en servicios públicos y privados. Resultados Entre las 2.195 puérperas, 67,7% (IC95% 65,7;69,6) desconocían como se previene el SMSL, estando el 71,6%, en la red pública; 77,8% temía asfixiarse/ahogarse; el 1,9% fue informado sobre el SMSL durante el prenatal; los médicos(as)/enfermeros(as) (70,5%) y los abuelos (65,1%) influyeron en la posición para dormir del bebé. Conclusión La mayoría de las puérperas desconocían la posición que previene el SMSL, especialmente en la red pública; en general, este tema no está cubierto en la atención prenatal.

RESUMO Objetivo Avaliar o conhecimento sobre prevenção da síndrome da morte súbita do lactente (SMSL) entre puérperas com pré-natal realizado nos serviços público e privado de Rio Grande, Rio Grande do Sul, Brasil, 2019. Métodos Estudo transversal, com puérperas do município; seu desfecho constituiuse da indicação de posição incorreta para dormir (decúbito lateral ou dorsal), visando prevenir a SMSL; utilizou-se o teste qui-quadrado para comparar proporções do desfecho e de exposição entre puérperas que realizaram pré-natal nos serviços público e privado. Resultados De 2.195 puérperas, 67,7% (IC95% 65,7;69,6), majoritariamente atendidas na rede pública (71,6%), desconheciam a posição preventiva da SMSL; 77,8% temiam engasgo/afogamento; 1,9% foram informadas sobre SMSL no pré-natal; médicos(as)/enfermeiros(as) (70,5%) e avós (65,1%) mostraram-se influentes na decisão sobre como posicionar o bebê adormecido. Conclusão A maioria das puérperas, especialmente as atendidas na rede pública, desconhecia a posição que previne SMSL; geralmente, o tema não é abordado no pré-natal.

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ABSTRACT Objective: To capacitate pregnant women to comply with measures designed to prevent sudden infant death syndrome. Methods: A quasi-experimental study was conducted before and after the intervention that included pregnant women attending the Course of Preparation for Childbirth and Parenthood of Health Centers Cluster. Six training sessions were given in the context of preventing this syndrome. Three questionnaires were applied, one to evaluate the knowledge of pregnant women before classes, other was submitted after the sessions, and another, one month after the birth of the babies, to identify what skills were acquired and which were practiced. Results: Among 77 studied pregnant women, 70 answered pre-session questionnaire and the proportion of correct answers varied from from 60.0% to 84.3%. After the intervention, 64 women answered the questionnaire and the proportion of correct answers varied between 79.7% and 100% . Prior to the intervention, the most wrong answers were related to the role of smoking as a risk factor for sudden infant death syndrome and to the use of pacifiers as a protective measure. After the sessions, all women answered correctly to the questions concerning where the baby should sleep and the safest way to lay the baby in the cradle. Conclusions: Health education with the aim of establishing measures may have a significant impact in terms of care delivery and mortality rate caused by sudden infant death syndrome.

RESUMO Objetivo: Capacitar as grávidas para o cumprimento de medidas de prevenção da síndrome de morte súbita do lactente. Métodos: Realizou-se um estudo quase-experimental pré- e pós-intervenção que integrou as grávidas que frequentavam o Curso de Preparação para o Parto e Parentalidade do Agrupamento de Centros de Saúde. Foram ministradas seis sessões formativas no âmbito da prevenção desta síndrome. Três questionários foram aplicados, um para avaliar os conhecimentos das gestantes antes das aulas, outro foi submetido após as sessões, e outro, um mês após o nascimento dos bebês para identificar que conhecimentos foram adquiridos e quais foram praticados. Resultados: Da amostra de 77 grávidas, relativamente ao questionário pré-sessão (n=70), obteve-se uma proporção de respostas corretas entre 60,0-84,3%. Posteriormente à intervenção (n=64), verificou-se um incremento dos conhecimentos com 79,7-100% de acertos. Previamente à intervenção, as respostas mais erradas às questões eram inerentes ao papel do tabagismo como fator de risco para síndrome de morte súbita do lactente e ao uso da chupeta como medida protetora. Após as sessões, todas as mulheres responderam corretamente às questões relativas ao local onde o bebê deve dormir e à forma mais segura de colocar o bebê no berço. Conclusões: Este projeto demonstrou que a educação para a saúde com o intuito de instituir medidas pode ter um impacto significativo em termos de prestação de cuidados e taxa de mortalidade por síndrome de morte súbita do lactente.

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Resumo Fundamento: Existem poucos estudos retrospectivos e prospectivos sobre cardiodesfibrilador implantável (CDI) na prevenção primária e secundária de morte súbita na cardiopatia chagásica crônica (CCC). Objetivos: Descrever a evolução a longo prazo dos portadores de CCC com CDI e identificar e analisar os preditores de mortalidade e de terapia apropriada do dispositivo nessa população. Métodos: Trata-se de um estudo prospectivo histórico com 117 pacientes portadores de CDI e CCC. Dispositivos foram implantados de janeiro de 2003 a dezembro de 2021. Fatores preditores de terapias apropriadas e mortalidade a longo prazo foram identificados e analisados. O nível de significância estatística é de p < 0,05. Resultados: Pacientes (n = 117) tiveram mediana de seguimento de 61 meses (25 a 121 meses), sendo o gênero masculino (74%) predominante e a mediana de idade de 55 anos (48 a 64 anos). Houve 43,6% de choques apropriados, 26,5% de estimulação cardíaca antitaquicardia (ATP) e 51% de terapias apropriadas. Durante o seguimento, 46 pacientes (39,7%) foram a óbito. A mortalidade foi de 6,2% pessoas-ano (intervalo de confiança [IC] 95%: 4,6 a 8,3), com 2 mortes súbitas durante o seguimento. A prevenção secundária (hazard ratio [HR] 2.1; IC 95%: 1,1 a 4,3; p = 0,029) e a fração de ejeção menor que 30% (HR 1.8; IC 95%: 1,1 a 3,1; p < 0,05) foram preditores de terapias apropriadas. Escore de Rassi intermediário apresentou uma forte associação com ocorrência de ATP isoladamente (p = 0,015). A classe funcional IV (p = 0,007), fração de ejeção do ventrículo esquerdo < 30 (p = 0,010) e a idade maior que 75 anos (p = 0,042) foram preditores de mortalidade total. Conclusão: Os desfibriladores na CCC apresentaram elevada incidência de acionamento apropriado especialmente naqueles pacientes de prevenção secundária, fração de ejeção do ventrículo esquerdo baixa e escore de Rassi intermediário. Os pacientes com insuficiência cardíaca congestiva, classe funcional avançada e idade maior que 75 anos apresentaram elevada mortalidade.

Abstract Background: There are few retrospective and prospective studies on implantable cardioverter-defibrillators (ICD) in primary and secondary prevention of sudden death in chronic Chagas heart disease (CCHD). Objectives: To describe the long-term evolution of patients with CCHD and ICD and to identify and analyze predictors of mortality and appropriate device therapy in this population. Methods: This was a historical prospective study with 117 patients with ICD and CCHD. Devices were implanted from January 2003 to December 2021. Predictors of appropriate therapies and long-term mortality were identified and analyzed. The level of statistical significance was p < 0.05. Results: Patients (n = 117) had a median follow-up of 61 months (25 to 121 months); they were predominantly male (74%), with a median age of 55 years (48 to 64 years). There were 43.6% appropriate shocks, 26.5% antitachycardia pacing (ATP), and 51% appropriate therapies. During follow-up, 46 patients (39.7%) died. Mortality was 6.2% person-years (95% confidence interval [CI]: 4.6 to 8.3), with 2 sudden deaths during follow-up. Secondary prevention (hazard ratio [HR] 2.1; 95% CI: 1.1 to 4.3; p = 0.029) and ejection fraction less than 30% (HR 1.8; 95% CI: 1.1 to 3.1; p < 0.05) were predictors of appropriate therapies. Intermediate Rassi score showed a strong association with the occurrence of ATP alone (p = 0.015). Functional class IV (p = 0.007), left ventricular ejection fraction < 30 (p = 0.010), and age above 75 years (p = 0.042) were predictors of total mortality. Conclusion: ICDs in CCHD showed a high incidence of appropriate activation, especially in patients with secondary prevention, low left ventricular ejection fraction, and intermediate Rassi score. Patients with congestive heart failure, elevated functional class, and age over 75 years showed elevated mortality.

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BACKGROUND: Sudden Sensorineural Hearing Loss was described by Mc. Cabe in 1979 and, since then, many authors have tried to define, explain and correctly treat this disease. The National Institute on Deafness and Other Communication Disorders defines it as sudden sensorineural hearing loss of at least 30 dB in three contiguous audiometric frequencies in a period of 72 hours. Among the therapeutic strategies, corticosteroids have been shown to have the greatest benefit due to their anti-inflammatory and anti-cellular stress effects. OBJECTIVE: To determine the hearing results with combined steroid therapy in patients with sudden sensorineural hearing loss (SSHL), according to the Siegel recovery criteria scale. METHOD: Study carried out in the otorhinolaryngology and head and neck surgery service of the Centro Médico Naval, Ciudad de México, where 150 patients diagnosed with SSHL and who received combined therapy with intratympanic dexamethasone and systemic prednisone were included. RESULTS: Therapeutic effectiveness was demonstrated by correlating therapeutic success in 82% of cases and therapeutic failure in 18% of cases, by correlating it with the Siegel recovery criteria scale. When evaluating the general average of the pure tone average levels at the beginning and 6 weeks after treatment, a statistically significant difference was obtained (p = 0.001). The average of the speech audiometry at the beginning and 6 weeks later had a statistically significant difference (p = 0.001). CONCLUSIONS: Initial combined steroid treatment for SSHL has been shown to have beneficial results according to Siegel recovery criteria scale.

ANTECENDENTES: La Hipoacusia Neurosensorial Súbita Idiopática fue descrita por Mc. Cabe en 1979 y, desde entonces, muchos autores han tratado de definir, explicar y tratar correctamente esta enfermedad. El Nacional Institute on Deafness and Other Communication Disorders la define como pérdida auditiva neurosensorial brusca de al menos 30 dB en tres frecuencias audiométricas contiguas en un periodo de 72 horas. Entre las estrategias terapéuticas, los corticosteroides han demostrado tener mayor beneficio por sus efectos antiinflamatorios y antiestrés celular. OBJETIVO: Determinar los resultados auditivos con la terapia de esteroides combinados en pacientes con hipoacusia neurosensorial súbita idiopática (HNSI), de acuerdo a la escala de criterios de recuperación de Siegel. MÉTODO: Estudio realizado en el servicio de otorrinolaringología y cirugía de cabeza y cuello del Centro Médico Naval, en Ciudad de México, en el que se incluyeron 150 pacientes con diagnóstico de HNSI y que recibieron terapia combinada con dexametasona intratimpánica y prednisona sistémica. RESULTADOS: Se demostró una efectividad terapéutica al correlacionar el éxito terapéutico en el 82% de los casos y un fracaso terapéutico en el 18% de los casos según la escala de criterios de recuperación de Siegel. Al evaluar el promedio general de los niveles de promedio de tonos puros al inicio y 6 semanas posterior al tratamiento se obtuvo una diferencia estadísticamente significativa (p = 0.001). El promedio de las logoaudiometrías al inicio y 6 semanas posterior al tratamiento tuvo una diferencia estadísticamente significativa (p = 0.001). CONCLUSIONES: El tratamiento combinado con esteroides de manera inicial para la HNSI ha demostrado tener resultados benéficos de acuerdo con la escala de criterios de recuperación de Siegel.

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RESUMEN La muerte súbita de un lactante puede ser de causa explicada, indeterminada -si no se investigó en forma suficiente- o inexplicada -cuando una investigación completa no permite determinar su causa-. La muerte súbita inexplicada, o síndrome de muerte súbita infantil, afecta en particular a las poblaciones más vulnerables. La muerte de estos niños que nacen con alteraciones del neurodesarrollo es la parte visible de una problemática que se origina en el embarazo. Disminuir la cantidad de niños vulnerables depende de políticas de salud y, sobre todo, de lograr mejorar las condiciones de vida de la población. Son acciones a largo plazo. Conocer a fondo los factores de riesgo que pueden desencadenar la muerte inesperada es lo que se puede hacer ya. La actualización de las recomendaciones sobre sueño seguro refleja nuevos conocimientos basados en la evidencia científica y un enfoque integral de los aspectos socioculturales relacionados con esta problemática.

ABSTRACT Sudden unexpected infant death may be explained, cause by an etiology, unexplained but insufficiently investigated, or unexplained when a full investigation fails to determine the cause. Unexplained sudden death in infancy or sudden infant death syndrome particularly affects the most vulnerable populations. The death of these children who are born with alterations in their neurodevelopment is the visible part of a problem that originates in pregnancy. Reducing the number of vulnerable children depends on health policies and, above all, on improving the living conditions of the population. These are long-term actions. Knowing in depth the risk factors that can trigger unexpected death is what can be done now. The update of the recommendations on safe sleep reflects new knowledge based on scientific evidence and a comprehensive approach to the sociocultural aspects related to this problem.

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La muerte súbita es aquella que ocurre dentro de las 24 horas posteriores al inicio de los síntomas y se caracteriza por ser clínicamente inexplicable, inesperada y repentina. Debido a la naturaleza de la muerte súbita, no es posible llegar a un diagnóstico preciso sin una autopsia. En esta comunicación breve, evaluaremos el caso de un empleado de crucero de 33 años, sin historial médico/farmacológico previo, el cual falleció súbitamente mientras reposaba en su camarote. Debido a las sospechas iniciales de una posible muerte causada por una sobredosis de cocaína, se le realizó un panel toxicológico abarcador el cual resultó negativo. Empero, una tomografía computarizada (TC) craneal sin contraste revirtió la hipótesis inicial y la autopsia neuropatológica -sorpresivamente- confirmó que la verdadera causa de muerte fue la ruptura de un aneurisma sacular desconocido en el polígono de Willis.

Sudden death occurs within 24 hours after the onset of symptoms and is characterized by being clinically inexplicable, sudden, and unexpected. Due to the nature of sudden death, it is not possible an accurate diagnosis without performing an autopsy. In this brief communication, we will evaluate the case of a 33-year-old cruise employee, with no prior medical/pharmacological history, who suddenly died while resting in his cabin. Due to initial suspicions of a possible cocaine overdose death, a comprehensive toxicology panel was performed, although yielding a negative result. A cranial computed tomography without contrast reversed the initial hypothesis and the neuropathological autopsy -surprisingly- confirmed that the true cause of death was the rupture of an unknown saccular aneurysm in the Circle of Willis.

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La muerte súbita de un lactante puede ser de causa explicada, indeterminada ­si no se investigó en forma suficiente­ o inexplicada ­cuando una investigación completa no permite determinar su causa­. La muerte súbita inexplicada, o síndrome de muerte súbita infantil, afecta en particular a las poblaciones más vulnerables. La muerte de estos niños que nacen con alteraciones del neurodesarrollo es la parte visible de una problemática que se origina en el embarazo. Disminuir la cantidad de niños vulnerables depende de políticas de salud y, sobre todo, de lograr mejorar las condiciones de vida de la población. Son acciones a largo plazo. Conocer a fondo los factores de riesgo que pueden desencadenar la muerte inesperada es lo que se puede hacer ya. La actualización de las recomendaciones sobre sueño seguro refleja nuevos conocimientos basados en la evidencia científica y un enfoque integral de los aspectos socioculturales relacionados con esta problemática.

Sudden unexpected infant death may be explained, cause by an etiology, unexplained but insufficiently investigated, or unexplained when a full investigation fails to determine the cause. Unexplained sudden death in infancy or sudden infant death syndrome particularly affects the most vulnerable populations. The death of these children who are born with alterations in their neurodevelopment is the visible part of a problem that originates in pregnancy. Reducing the number of vulnerable children depends on health policies and, above all, on improving the living conditions of the population. These are long-term actions. Knowing in depth the risk factors that can trigger unexpected death is what can be done now. The update of the recommendations on safe sleep reflects new knowledge based on scientific evidence and a comprehensive approach to the sociocultural aspects related to this problem.

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Introducción: Las evidencias científicas han demostrado que durante el período pandémico por la COVID-19 ha existido un incremento de la incidencia de muerte súbita cardiovascular, proporcional al incremento de los casos y a la letalidad por la enfermedad. Objetivos: Compilar información sobre los fármacos empleados en el tratamiento de la COVID-19 y sus posibles efectos en la prolongación del intervalo QT y la aparición de muerte súbita. Métodos: Se realizó una búsqueda de información a partir de las bases de datos PubMed, Medline y SciELO, en los idiomas español e inglés en el período de enero de 2020 a enero de 2023. Resultados: Los hallazgos más recientes sugieren que los factores relacionados con el tratamiento médico del paciente para sus enfermedades cardiovasculares previas, el empleo concomitante de drogas para otras comorbilidades, el ensayo de nuevas drogas que se investigan en la actualidad para el tratamiento de la enfermedad y el uso inadecuado de fármacos en complicaciones graves por la COVID-19, pueden ocasionar prolongación del intervalo QT y arritmias ventriculares tipo torsades de pointes, lo que puede conllevar a la aparición de muerte súbita. Conclusiones: Ha sido demostrado el efecto deletéreo de los fármacos en el tratamiento de la COVID-19 y sus posibles asociaciones a la terapéutica del paciente, en la prolongación del tiempo de repolarización ventricular cardíaca, cuya traducción eléctrica es un intervalo QT prolongado y su contribución a la génesis de arritmias malignas potencialmente fatales capaces de desencadenar un paro cardíaco y evolucionar a la muerte súbita(AU)

Scientific evidence has shown an increase in the incidence of sudden cardiovascular death during the COVID-19 pandemic period. This has been proportional to the increase in cases and mortality from the disease. Direct and indirect injury to the myocardium and vascular system allow to partially explain the statistics. Among the factors related to the medical treatment of the patient for previous cardiovascular diseases, it is the concomitant use of drugs for other comorbidities. The trial of new drugs for the treatment of this condition and the inappropriate use of drugs in serious complications from COVID-19 are currently being investigated. These can cause QT prolongation and torsades de pointes ventricular arrhythmias, which can lead to sudden death. Monitoring the QT interval is recommended, before and during treatment, in patients who come to the emergency room with a clinical condition suggestive of COVID-19. Additionally, modifiable factors favoring its prolongation should be evaluated. Decision-making in the application of therapeutic protocols in patients with COVID-19 with prolonged QTc at baseline, or with increased QTc after starting treatment, must go through the analysis of the risk/benefit ratio defined by a multi- and interdisciplinary team(AU)

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Resumen Introducción: El desfibrilador automático implantable (DAI) transvenoso es el tratamiento de elección para la prevención de muerte súbita (MS) cardiaca por arritmias ventriculares malignas. Su uso se puede ver limitado cuando existe infección del sistema de estimulación o en población pediátrica donde representa un reto por diversas razones, incluyendo: las mínimas dimensiones del sistema venoso de los niños, la longitud de los electrodos, el tamaño del generador, así como por la complejidad anatómica en casos de cardiopatía congénita asociada. Objetivo: El presente artículo tiene por objetivo presentar la primera serie de casos de pacientes mexicanos a los cuales se les implantó un DAI subcutáneo (DAI-SC) como terapia para la prevención de MS. Métodos: Se presentan los cuatro primeros casos que fueron implantados en el Instituto Nacional de Cardiología Ignacio Chávez con un DAI-SC (Emblem, Boston Scientific, EE.UU.), tres de ellos eran pacientes pediátricos, incluyendo el primer implante de este tipo de dispositivo en un paciente pediátrico en América Latina. Las técnicas de tres y dos incisiones fueron empleadas bajo anestesia general. Resultados: Se realizó el implante exitoso con técnica de tres incisiones en los dos primeros casos y con técnica de dos incisiones en los dos últimos casos. Se corroboró el adecuado funcionamiento del dispositivo en sala, con la verificación de terapia apropiada (65 J) de la fibrilación ventricular inducida mediante estimulación a 50 Hz. No hubo complicaciones inmediatas. Un paciente presentó descargas apropiadas a los dos meses del implante. En el seguimiento, un niño desarrolló erosión de la piel a nivel de la curva del electrodo en el esternón, sin datos de infección. En quirófano se resecó la piel dañada, se retiró el barril y la seda de fijación, se realizó lavado quirúrgico y se volvió a cerrar la piel, logrando así evitar el retiro del sistema. Conclusiones: El DAI-SC es una terapia alternativa al DAI endovenoso y puede ser considerado de primera elección en aquellos casos que no requieran de estimulación ventricular, incluyendo pacientes pediátricos. Pueden ocurrir complicaciones cutáneas, pero no representan una amenaza como las complicaciones venosas de los DAI convencionales.

Abstract Introduction: The transvenous implantable cardioverter defibrillator (ICD) is the treatment of choice for the prevention of sudden cardiac death (SCD). Its use could be restricted when device-related infections occurs or in the pediatric population. In the later, an ICD represents a challenge, due to the minimal dimensions of the venous system in children, the length of the electrodes, the size of the generator, as well as the anatomical complexity in cases with associated congenital heart disease. Objective: This article presents the first Mexican patients with a subcutaneous ICD (SC-ICD) implant as a therapy for the prevention of SCD. Methods: The first four cases were implanted at the Ignacio Chávez National Institute of Cardiology with a SC-ICD (Emblem, Boston Scientific, USA), three of them were pediatric patients, including the first implant of this type of device in a pediatric patient in Latin America. The 3-incision and 2-incision techniques were used under general anesthesia. Results: A successful implantation was obtained with the 3-incision technique in the first 2 cases and the last 2 with the 2-incision technique. Proper functioning of the device was corroborated in the operating room with proof of appropriate therapy (65 J) for ventricular fibrillation induced with 50 Hz stimulation. No immediate complications were observed. One patient had appropriate shocks two months after the implant. During follow-up, one child developed skin erosion at the level of the curve of the electrode on the sternum, with no signs of infection. In the operating room, the damaged skin was resected, the barrel and the fixation silk were removed, surgical lavage was performed, and the skin was closed again, thus avoiding removal of the system. Conclusions: The SC-ICD is an alternative therapy to the transvenous ICD. It can be considered first choice in subjects who do not require ventricular pacing, including pediatric patients. Skin complications can occur but do not pose a threat as venous complications of conventional ICDs.

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INTRODUCTION: The transvenous implantable cardioverter defibrillator (ICD) is the treatment of choice for the prevention of sudden cardiac death (SCD). Its use could be restricted when device-related infections occurs or in the pediatric population. In the later, an ICD represents a challenge, due to the minimal dimensions of the venous system in children, the length of the electrodes, the size of the generator, as well as the anatomical complexity in cases with associated congenital heart disease. OBJECTIVE: This article presents the first Mexican patients with a subcutaneous ICD (SC-ICD) implant as a therapy for the prevention of SCD. METHODS: The first four cases were implanted at the Ignacio Chávez National Institute of Cardiology with a SC-ICD (Emblem, Boston Scientific, USA), three of them were pediatric patients, including the first implant of this type of device in a pediatric patient in Latin America. The 3-incision and 2-incision techniques were used under general anesthesia. RESULTS: A successful implantation was obtained with the 3-incision technique in the first 2 cases and the last 2 with the 2-incision technique. Proper functioning of the device was corroborated in the operating room with proof of appropriate therapy (65 J) for ventricular fibrillation induced with 50 Hz stimulation. No immediate complications were observed. One patient had appropriate shocks two months after the implant. During follow-up, one child developed skin erosion at the level of the curve of the electrode on the sternum, with no signs of infection. In the operating room, the damaged skin was resected, the barrel and the fixation silk were removed, surgical lavage was performed, and the skin was closed again, thus avoiding removal of the system. CONCLUSIONS: The SC-ICD is an alternative therapy to the transvenous ICD. It can be considered first choice in subjects who do not require ventricular pacing, including pediatric patients. Skin complications can occur but do not pose a threat as venous complications of conventional ICDs.

INTRODUCCIÓN: El desfibrilador automático implantable (DAI) transvenoso es el tratamiento de elección para la prevención de muerte súbita (MS) cardiaca por arritmias ventriculares malignas. Su uso se puede ver limitado cuando existe infección del sistema de estimulación o en población pediátrica donde representa un reto por diversas razones, incluyendo: las mínimas dimensiones del sistema venoso de los niños, la longitud de los electrodos, el tamaño del generador, así como por la complejidad anatómica en casos de cardiopatía congénita asociada. OBJETIVO: El presente artículo tiene por objetivo presentar la primera serie de casos de pacientes mexicanos a los cuales se les implantó un DAI subcutáneo (DAI-SC) como terapia para la prevención de MS. MÉTODOS: Se presentan los cuatro primeros casos que fueron implantados en el Instituto Nacional de Cardiología Ignacio Chávez con un DAI-SC (Emblem, Boston Scientific, EE.UU.), tres de ellos eran pacientes pediátricos, incluyendo el primer implante de este tipo de dispositivo en un paciente pediátrico en América Latina. Las técnicas de tres y dos incisiones fueron empleadas bajo anestesia general. RESULTADOS: Se realizó el implante exitoso con técnica de tres incisiones en los dos primeros casos y con técnica de dos incisiones en los dos últimos casos. Se corroboró el adecuado funcionamiento del dispositivo en sala, con la verificación de terapia apropiada (65 J) de la fibrilación ventricular inducida mediante estimulación a 50 Hz. No hubo complicaciones inmediatas. Un paciente presentó descargas apropiadas a los dos meses del implante. En el seguimiento, un niño desarrolló erosión de la piel a nivel de la curva del electrodo en el esternón, sin datos de infección. En quirófano se resecó la piel dañada, se retiró el barril y la seda de fijación, se realizó lavado quirúrgico y se volvió a cerrar la piel, logrando así evitar el retiro del sistema. CONCLUSIONES: El DAI-SC es una terapia alternativa al DAI endovenoso y puede ser considerado de primera elección en aquellos casos que no requieran de estimulación ventricular, incluyendo pacientes pediátricos. Pueden ocurrir complicaciones cutáneas, pero no representan una amenaza como las complicaciones venosas de los DAI convencionales.

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Introducción: La miocardiopatía hipertrófica (MCH) constituye una enfermedad compleja y variable en cuanto a morfología, fisiopatología, pronóstico y sobrevida. Se caracteriza por una hipertrofia del ventrículo izquierdo, aunque en ocasiones puede ser biventricular o ventricular derecha aislada. En la edad pediátrica predominan las causas no sarcoméricas. La ecocardiografía es el método diagnóstico más utilizado para caracterizar la morfología y repercusión hemodinámica, sin embargo, la resonancia magnética cardíaca (RMC) es de elección por aportar mayores ventajas morfológicas y funcionales. Las pruebas genéticas son de gran importancia en la determinación del diagnóstico etiológico, manejo terapéutico y pronóstico. El tratamiento tiene como objetivo principal mejorar la capacidad funcional y aliviar los síntomas. Los betabloqueantes no vasodilatadores son considerados de primera líneay en algunos casos se puede recurrir a terapias avanzadas como disopiramida o terapia de reducción septal (TRS), uso del cardiodesfibrilador implantable (CDI), y en ocasiones se debe considerar trasplante cardiaco en pacientes con síntomas refractarios al tratamiento médico y en MCH del tipo obstructivo. Materiales y métodos: Estudio observacional, descriptivo, corte transversal. Resultados: Se estudiaron 12 pacientes, desde enero de 2020 a diciembre de 2022, con edades comprendidas desde 5 meses hasta 16 años, diagnosticados con MCH de presentación clínica variable y de distintas etiologías. Conclusión: La MCH de causas no sarcoméricas como los síndromes malformativos y errores innatos del metabolismo son las que tienen peor pronóstico y sobrevida. La finalidad de detectar precozmente la MCH es el inicio temprano de la terapia específica, para así retrasar el compromiso cardiovascular y mejorar la sobrevida.

Introduction: Hypertrophic cardiomyopathy (HCM) is a complex and variable disease in terms of morphology, pathophysiology, prognosis and survival. It is characterized by left ventricular hypertrophy, although it can sometimes be biventricular or isolated right ventricular. In the pediatric population, non-sarcomeric causes predominate. Echocardiography is the most widely used diagnostic method to characterize morphology and hemodynamic repercussions; however, cardiac magnetic resonance imaging (CMR) is preferred because it provides greater morphological and functional advantages. Genetic tests are of great importance in determining the etiological diagnosis, therapeutic management and prognosis. The main objective of the treatment is to improve functional capacity and alleviate symptoms. Non-vasodilator beta-blockers are considered first-line treatment and in some cases advanced therapies such as disopyramide or septal reduction therapy (SRT), use of implantable cardioverter-defibrillator (ICD) can be used; heart transplantation should be considered in patients with symptoms refractory to medical treatment and in HCM of the obstructive type. Materials and methods: This was an observational, descriptive, cross-sectional study. Results: 12 patients were studied, from January 2020 to December 2022, with ages ranging from 5 months to 16 years, diagnosed with HCM of variable clinical presentation and of different etiologies. Conclusion: HCM from non-sarcomeric causes such as malformation syndromes and inborn errors of metabolism have the worst prognosis and survival. The purpose of early detection of HCM is the early initiation of specific therapy, in order to delay cardiovascular compromise and improve survival.

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Resumen Se presenta el caso de una mujer de 60 años, con an tecedente de prolapso de la válvula mitral, que consultó por disnea y palpitaciones de 2 semanas de evolución hasta clase funcional IV. En el electrocardiograma de ingreso se evidenció ritmo de fibrilación auricular de moderada respuesta con extrasístoles ventriculares fre cuentes. Se realizó ecocardiograma transtorácico donde se observó prolapso de la válvula mitral con deterioro grave de la función ventricular. Se diagnosticó síndrome de Barlow. La paciente intercurrió durante la internación con tres episodios de paro cardiorrespiratorio que revir tieron con maniobras de reanimación cardiopulmonar avanzada. Durante la internación se realizó balance negativo, se revirtió a ritmo sinusal y se colocó cardio desfibrilador implantable en prevención secundaria. En el seguimiento persiste con deterioro grave de la función ventricular. Destacamos el síndrome de Barlow como una causa poco frecuente de muerte súbita y su asociación con miocardiopatía dilatada.

Abstract We present the case of a 60-year-old woman, with a history of mitral valve prolapse, who consulted for dyspnea and palpitations of 2 weeks of evolution up to functional class IV. The admission electrocardio gram showed a moderately responsive atrial fibrilla tion rhythm with frequent ventricular extra systoles. A transthoracic echocardiogram was performed which showed mitral valve prolapse with severe impairment of ventricular function. Barlow syndrome was diagnosed. During hospitalization, the patient presented three epi sodes of cardiorespiratory arrest that were reversed with advanced cardiopulmonary resuscitation maneuvers. During admission, a negative balance was performed, sinus rhythm was reverted and an implantable auto matic defibrillator was placed in secondary prevention. During follow-up, severe deterioration of ventricular function persisted. We highlight Barlow syndrome as a rare cause of sudden death and its association with dilated cardiomyopathy.

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Introdução: A síndrome da morte súbita do lactente (SMSL) pode ser definida como a morte inesperada de uma criança menor de um ano durante o sono, de causa inexplicada mesmo após necrópsia. É uma condição com fatores de risco conhecidos e, em sua maioria, modificáveis. Objetivo: Destacar as evidências científicas nas quais se baseiam as recomendações da Academia Americana de Pediatria e da Sociedade Brasileira de Pediatria para prevenção da SMSL. Métodos: Revisão da literatura a partir da revisão das recomendações das sociedades para prevenção de SMSL, em busca de evidências que corroborem cada recomendação. Resultados: As recomendações para prevenção da SMSL incluem dormir em posição supina; utilizar uma superfície firme, plana e não inclinada para dormir; aleitamento materno pelo maior tempo possível; dormir no quarto dos pais nos seis primeiros meses de vida; manter o berço livre de objetos macios; oferecer a chupeta durante o sono; evitar a exposição ao tabagismo; não utilizar monitores cardiorrespiratórios. Para todas essas recomendações, foram apresentadas evidências científicas provenientes, de forma majoritária, de estudos de caso-controle. Conclusão: As recomendações para prevenção da SMSL são baseadas em evidências científicas suficientes, e é preciso que os profissionais da saúde que atendem a crianças pequenas e suas famílias estejam sempre atualizados e atentos a elas, promovendo de forma ativa a segurança do sono.

Background: Sudden Infant Death Syndrome (SIDS), defined as the unexpected death of a child under one year of age during sleep, of unexplained cause even after autopsy, is a condition with known and mostly modifiable risk factors.Objective: To highlight the scientific evidence on which the recommendations of the American Academy of Pediatrics and the Brazilian Society of Pediatrics for SIDS prevention are based. Methods: Literature review based on the societies' recommendations for SIDS prevention, searching for evidence that supports each recommendation. Results:The recommendations for SIDS prevention include placing the infant in a supine position for sleep; using a firm, flat, and non-inclined sleep surface; breastfeeding for as long as possible; room-sharing with parents for the first six months of life; keeping the crib free from soft objects; offering a pacifier during sleep; avoiding exposure to smoking; not using cardiorespiratory monitors. For all these recommendations, scientific evidence was presented, predominantly from case-control studies. Conclusion: The recommendations for SIDS prevention are based on sufficient scientific evidence, and healthcare professionals who care for young children and their families need to stay updated and attentive to them, actively promoting sleep safety.

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