Access to emergency surgical treatment for congenital heart diseases in the state of São Paulo

Abstract Objective To analyze access to surgical care for congenital heart diseases in public specialized centers in the state of São Paulo before and during the COVID-19 pandemic and availability of surgical care in specialized hospitals, to identify bottlenecks in the care path for neonatal cardiac surgery. Methods This study included 1,437 children, under one year old, with congenital heart disease and formal referral to heart surgery between February 1, 2019, and February 28, 2021. Quantitative data analysis was performed using t-tests or the Mann-Whitney test. Results Approximately 30 % of children with urgent congenital heart disease could not access recommended care, mainly those needing complex surgeries (categories RACHS 4 to 6). The main diagnoses of neonates accessing care were patency of the ductus arteriosus (10.5 %) and coarctation of the aorta (10.1 %). Referral time for children in RACHS 1 to 3 was 4 days (median), while for those in categories 4 to 6, it was 7 days (p< 0.001). Longer referral time (20 days) was associated with court orders. During the pandemic, referral time decreased to 3 days, compared to 5 days (median) in the pre-pandemic period (p< 0.001). Conclusion The emergency surgical treatment supply for congenital heart diseases is insufficient compared to the current demand. Future research should evaluate if access to care in publicly funded hospitals could be improved by better access to prenatal care for pregnant women, increased diagnostic and therapeutic capacity in pediatric cardiology, and financial incentives for complex cardiac surgeries.

The care of the patients with hipoplastic left heart syndrome in places of social and economic vulneability. An ethical analysis / O cuidado de pacientes com síndrome do coração esquerdo hipoplásico em localidades de vulnerabilidade social e econômica. Uma análise bioética

ABSTRACT The birth of a child means hope and joy, particularly for the parents and the healthcare team. When this child is born with a severe malformation and a poor prognosis, as in the case of hypoplastic left heart syndrome, the scenario is one of great uncertainty and emotional suffering. The role of the health team becomes fundamental for the identification of conflicts of values and for the search for shared decisions that promote the best benefit to the child. When the diagnosis is made during fetal life, it is necessary to develop counseling strategies appropriate to the context of each family. In places with limited care resources, precarious prenatal care and short temporal conditions, the recommended counseling is compromised. Indication of treatment requires technical competence and a detailed analysis of ethical issues, and consultation with institutional clinical bioethics services or commissions is important. The article proposes to address the moral conflicts of two clinical cases and the respective bioethical analysis that involves principles and values in contexts of vulnerability and uncertainty, contrasting two situations where the indication of treatment was based on accessibility to treatment.

RESUMO O nascimento de uma criança significa esperança e alegria, particularmente para os pais e para a equipe de saúde. Quando essa criança nasce com uma malformação grave e de prognóstico reservado, como acontece na SHCE, o cenário é de grande incerteza e sofrimento emocional. O papel da equipe de saúde torna-se fundamental para a identificação de conflitos de valores e para a busca de decisões compartilhadas que promovam o melhor benefício à criança. Quando o diagnóstico é feito em vida fetal, é necessário a elaboração de estratégias de aconselhamento adequadas ao contexto de cada família. Em locais com recursos assistenciais limitados, acompanhamento prénatal precário e condição temporal curta, o aconselhamento preconizado fica comprometido. A indicação do tratamento requer competência técnica e uma análise pormenorizada de questões éticas, sendo importante a consultoria aos serviços ou comissões de bioética clínica institucional. O artigo propõe a abordar os conflitos morais de dois casos clínicos e a respectiva análise bioética que envolve princípios e valores em contextos de vulnerabilidade e incertezas, contrapondo duas situações onde a indicação do tratamento baseou-se na acessibilidade ao tratamento.

Fetal echocardiography audit in a tertiary hospital in Ibadan, Nigeria

Delayed detection of congenital heart diseases in low- and middle-income countries (LMICs) contributes to the poor outcome of infants with cardiac anomalies. Fetal echocardiography (FE) can detect heart defects in-utero as early as the 18th gestational week (GA), giving parents and medical professionals time to prepare for the baby's delivery and appropriate treatment. University College Hospital, Nigeria, is one of the few centres in Nigeria where FE is performed. Objectives: To examine the indications for referral for FE and the diagnoses made in our first four years of performing FE. Methods: FE was performed in the antenatal clinic of the University College Hospital, Ibadan, Nigeria, using the GE Voluson P6 machine with a 2-6-RS probe. Demographic information was obtained from the antenatal clinic records of the women who had FE using a semi-structured questionnaire. FE diagnoses were retrieved from the FE register. Results: A total of 129 women whose records were available were studied. The mean age of the women was 31.7±5.5 years. Forty-two women (32.6%) were referred because of echogenic foci, making this the most common indication for FE. Twenty-nine (22.5%) fetuses had structural heart defects, the most common being Ventricular Septal Defect. Conclusion: There is a need for Obstetricians and Paediatricians to be aware of the indications for fetal echocardiography and refer women appropriately

Surgical management of neonatal coarctation of the aorta with aortic arch hypoplasia: A retrospective study in a single center / 中国胸心血管外科临床杂志

@#Objective     To summarize the surgical treatment experience in neonates with coarctation of the aorta (CoA) and aortic arch hypoplasia (AAH). Methods     The neonates with CoA and AAH who underwent surgical treatment in the Department of Pediatric Cardiac Surgery of Guangdong Provincial People's Hospital from 2013 to 2020 were retrospectively enrolled. The postoperative complications, long-term survival rate, and freedom from aortic reobstruction were analyzed. Patients undergoing extended end-to-end anastomosis were allocated into an extended end-to-end group, those undergoing extended end-to-side anastomosis into an extended end-to-side group, and those undergoing pulmonary autograft patch aortoplasty into a patch aortoplasty group. Results     Finally 44 patients were  enrolled, including 37 males and 7 females, aged 5.00-30.00 (19.34±7.61) days and weighted 2.00-4.50 (3.30±0.60) kg. There were 19 patients of extended end-to-end anastomosis, 19 patients of extended end-to-side anastomosis, and 6 patients of pulmonary autograft patch aortoplasty. The mean values of the Z scores of the proximal, distal, and isthmus of the aortic arch were –2.91±1.52, –3.40±1.30, and –4.04±1.98, respectively. The mean follow-up time was 45.6±3.7 months. There were 2 early deaths and no late deaths. Aortic reobstruction occurred in 8 patients, and 3 patients underwent reoperation intervention. The 5-year rate of freedom from reobstruction was 78.8%. The Cox multivariable regression analysis showed that the related factors for postoperative reobstruction were the Z score of the preoperative proximal aortic arch (HR=0.152, 95%CI 0.038-0.601, P=0.007) and the postoperative left main bronchus compression (HR=15.261, 95%CI 1.104-210.978, P=0.042). Conclusion     Three surgical procedures for neonates with CoA and AAH are safe and effective, but the aortic reobstruction rate in long term is not low. The smaller Z score of the preoperative proximal aortic arch and the postoperative left main bronchus compression are risk factors for long-term aortic reobstruction.

Clinical Characteristics of Cardiac Arrhythmias in Pediatric Age Group

Background: The Symptomatic manifestation of pediatric arrhythmias varies according to the age. Patients with disturbances in cardiac rhythm have several complaints, but also could be totally asymptomatic. This study aimed to assess clinical characteristics of Tachy and Brady arrhythmia in neonates, infant, children and adolescents of various age groups. Methods: This cross-sectional study included 253 patients of pediatric age below 18 years old presented with different types of tachyarrhythmia or bradyarrhythmia. All patients were subjected to clinical general examination of patient, vital data collection, local cardiac examination, 12 leads electrocardiography, ambulatory ECG Holter monitoring, exercise ECG stress testing if indicated and transthoracic echocardiography. Results: There was a significant relation between age of presentation of the study patients and classification of arrhythmia (P < 0.001). Palpitations was the most common presentations of arrhythmia in our study patients (32%) followed by dyspnea in 17.8 % of the patients. 20.6 % of the study patients with arrhythmias were asymptomatic. Congenital heart anomalies were in 43 % of the patients. Conclusion: Pediatric arrhythmia can be totally asymptomatic and discovered accidentally. Congenital cardiac anomalies and cardiac arrhythmias are closely correlated. Holter monitoring and exercise ECG testing can help in diagnosing pediatric arrhythmia. Untreated or persistent arrhythmia causes LV myopathy and dilatation. Antiarrhythmic drugs can have their harmful effect on the growing child.

Programa educativo sobre sexualidad para adolescentes con cardiopatías congénitas en el Cardiocentro Pediátrico William Soler

Fundamento: las cardiopatías congénitas son malformaciones frecuentes y constituyen un problema de salud. Las personas con cardiopatías congénitas al llegar a la adolescencia muestran desconocimiento acerca de su sexualidad. El desconocimiento sobre estos temas promueve prácticas poco saludables y emociones negativas que afectan su bienestar. Objetivo: elaborar un programa educativo sobre sexualidad para adolescentes con cardiopatías congénitas que se atienden en el Cardiocentro Pediátrico William Soler de La Habana. Métodos: se realizó una investigación de tipo cualitativa que utiliza un paradigma dialéctico en el Cardiocentro Pediátrico William Soler durante el período del 2019 al 2021. Los instrumentos utilizados para la investigación fueron: observación, entrevista, grupo focal, análisis de documentos y registro de anécdotas. Se describió como categoría principal las necesidades educativas especiales sobre sexualidad, así como, aquellas dificultades en aspectos cognitivos, emocionales y psicosociales, que pueden tener las personas en su proceso de aprendizaje sobre la sexualidad con carácter temporal o duradero. Se utilizó la estadística descriptiva y el análisis de datos para profundizar en este fenómeno. Resultados: en los adolescentes estudiados se identificaron necesidades educativas especiales sobre sexualidad tales como: carencias de información, estereotipos y conocimientos limitados, dificultades para las relaciones personales y de pareja, afectaciones en los procesos autovalorativos, desconocimiento sobre género, salud sexual, cardiopatías congénitas, cambios en la adolescencia, anticoncepción y planificación familiar. Conclusiones: el programa propuesto constituye una herramienta para la modificación de conocimientos, actitudes y comportamientos hacia la sexualidad. Además, tiene la intención de contribuir al desarrollo de otras habilidades como la socialización, toma de decisiones y autonomía.

Background: congenital heart diseases are frequent malformations and constitute a health problem. People with congenital heart disease when they reach their adolescence show doubts about their sexuality. Ignorance about these issues promotes unhealthy practices and negative emotions that affect their well-being. Objective: to develop an educational program on sexuality for adolescents with congenital heart disease who are treated at the William Soler Pediatric Cardiocenter. Methods: a qualitative research was carried out using a dialectical paradigm at the William Soler Pediatric Cardiocenter during the period from 2019 to 2021. The used instruments in the research were: observation, interview, focus group, documents analysis and recording of anecdotes. Special educational needs on sexuality were described as the main category, as well as those difficulties in cognitive, emotional and psychosocial aspects that people may have in their learning process about sexuality on a temporary or lasting basis. Descriptive statistics and data analysis were used to delve into this phenomenon. Results: special educational needs on sexuality were identified in the adolescents studied, such as: lack of information, stereotypes and limited knowledge, difficulties in personal and couple relationships, affectations in self-assessment processes, lack of knowledge about gender, sexual health, congenital heart disease, changes in adolescence, contraception and family planning. Conclusions: the proposed program constitutes a tool for the knowledge modification, attitudes and behaviors towards sexuality. In addition, it is intended to contribute to the development of other skills such as socialization, decision-making and autonomy.

Critical Congenital Heart Disease Sadly Diagnosed at Autopsy in Calabar, South-South Nigeria

Congenital heart disease (CHD) is the most common type of birth defect causing more deaths in the first year of life than any other birth defect. Medical practice in a third-world country is faced with numerous challenges, created by poor health facilities and the unavailability of basic imaging studies at the community level where the majority of the poor resides. We present a case series of 4 neonates with a ratio of 1: 3 admitted into the neonatology unit of the University of Calabar Teaching Hospital, Calabar, Nigeria. Two of the patients were delivered at home by a traditional birth attendant via spontaneous vertex delivery and the rest two were booked cases in a private and obstetric unit of the University of Calabar Teaching Hospital. Their age ranged were 18 hours to 27 days of life and their common presentation were poor sucking, difficulty in breathing, central cyanosis and failure to thrive. A fetal echocardiogram was carried out for one of the cases and there was strong clinical suspicion of congenital heart disease but the precise type is unknown. The other three could not afford the necessary investigations required. They were all oxygen-dependent till death. Pathologic-anatomic findings showed a rare Critical congenital heart defect of the univentricular heart chamber of various types for the three cases and a case of TGA. The Immediate cause of death for all four cases was congestive cardiac failure.

Design of a mobile application for home monitoring of babies with complex congenital heart disease / Diseño de una aplicación móvil para la monitorización domiciliaria de bebés con cardiopatías congénitas complejas / Projeto de um aplicativo móvel para monitoramento domiciliar de bebês com cardiopatias congênitas complexas

Introduction: Congenital heart diseases are related to heart defects that develop during pregnancy and are present at birth. Children must regularly attend specialized clinical centers for treatment, which includes medical check-ups, hemodynamic procedures, and surgery. Nevertheless, the success of interventions largely depends on the education of parents and caregivers about the symptoms and warning signs that babies with complex congenital heart disease may present during the period between stages. Likewise, it is necessary for caregivers to have tools to face the barriers of the health system, such as, obstacles or delays in obtaining healthcare services. This facilitates timely health care for patients and avoids fatal outcomes or deterioration in quality of life. Materials and Methods: This document presents a system that provides support to patients with complex congenital heart disease in follow-up by a home monitoring program. The system consists of a mobile application that allows parents/caregivers to monitor physiological variables of the patients (weight, intake, and oximetry). The information registered in the application is sent for real-time evaluation by the medical team. Results:The application provides an effective means for sending data and communicating with the medical control center, in addition to offering educational and informative material. In this way, parents/caregivers get constant help and feedback about their baby's condition. Conclusions: The mobile application could help mitigate the costs of medical care and overcome the limitations of follow-up at home, giving doctors the possibility to see the patient's evolution and give timely recommendations.

Introducción: Las cardiopatías congénitas están relacionadas con defectos cardíacos que se desarrollan durante el embarazo y están presentes al nacer. El éxito de las intervenciones depende en gran medida de la educación de los padres/cuidadores sobre los signos y síntomas de alerta. Los cuidadores necesitan tener herramientas para hacer frente a las barreras del sistema de salud. Objetivo: crear un sistema como herramienta de seguimiento para los bebés con cardiopatías congénitas complejas que ayude a los padres/cuidadores y a los profesionales médicos a observar las variables fisiológicas del paciente y a brindar una retroalimentación oportuna y profesional a los padres/cuidadores desde la atención domiciliaria. Materiales y métodos:se presenta un sistema que proporciona apoyo a los pacientes que necesitan seguimiento de cardiopatías congénitas complejas mediante un programa de monitoreo en casa. El sistema consiste en una aplicación móvil que permite a los padres/cuidadores controlar las variables fisiológicas del paciente (peso, ingesta de alimento y oximetría). La información registrada en la aplicación se envía para ser evaluada en tiempo real por el equipo médico. Resultados: la aplicación proporciona un medio eficaz para enviar datos y comunicarse con el centro de control médico, además de ofrecer material educativo e informativo. De este modo, los padres/cuidadores reciben constantemente ayuda e información acerca del estado de su bebé. Conclusiones: la aplicación móvil podría ayudar a minimizar los costos de la atención médica y superar las limitaciones del seguimiento domiciliario, dando a los médicos la posibilidad de ver la evolución del paciente y dar recomendaciones oportunas.

Introdução: as doenças cardíacas congênitas estão relacionadas a defeitos cardíacos que se desenvolvem durante a gravidez e estão presentes no nascimento. O sucesso das intervenções depende em grande parte da educação dos pais/responsáveis sobre os sinais e sintomas de alerta. Os cuidadores precisam ter ferramentas para lidar com as barreiras do sistema de saúde. Objetivo: Criar uma ferramenta de sistema de monitoramento para bebês com doenças cardíacas congênitas complexas, que ajude pais/cuidadores e profissionais médicos a observar as variáveis fisiológicas do paciente e manter um feedback profissional e oportuno para os pais/cuidadores dos cuidados domiciliares. Materiais e Métodos: é apresentado um sistema que fornece suporte aos pacientes com cardiopatias congênitas complexas em acompanhamento através de um programa de monitoramento domiciliar. O sistema consiste em uma aplicação móvel que permite aos pais/responsáveis pelo acompanhamento das variáveis fisiológicas do paciente (peso, ingestão e oximetria). As informações registradas no aplicativo são enviadas para serem avaliadas em tempo real pela equipe médica. Resultados: a aplicação fornece um meio eficiente para enviar dados e se comunicar com o centro de controle médico, além de oferecer material educativo e informativo. Desta forma, os pais/responsáveis recebem ajuda constante e feedback sobre a condição de seu bebê. Conclusões: a aplicação móvel pode ajudar a minimizar os custos do atendimento médico e superar as limitações do acompanhamento domiciliar, dando aos médicos a possibilidade de ver a evolução do paciente e dar recomendações oportunas.

Sobrecarga dos cuidadores primários de crianças com cardiopatia congênita / Burden of primary caregivers of children with congenital heart disease

Objetivos: Identificar o nível de sobrecarga de cuidadores primários de crianças com cardiopatia congênita e analisar os fatores associados. Método: tratou-se de um estudo transversal descritivo com abordagem quantitativa. Participaram da pesquisa 100 cuidadores primários de crianças com cardiopatia congênita na faixa etária de 0 a 12 anos. Utilizou-se um questionário para caracterização da amostra (cuidadores e as crianças), que compôs as variáveis independentes do estudo e a versão brasileira da Escala de Sobrecarga do cuidador (Zarit Burden Interview) para avaliar o nível de sobrecarga, de onde foram extraídas as variáveis dependentes do estudo. A análise dos dados foi realizada por meio do programa SPSS 24 e aplicado o teste estatístico de regressão linear bivariada e multivariada, além da estatística descritiva e considerando p<0,05 para significância. Resultados: o escore médio da sobrecarga física, social e emocional dessas mães cuidadoras foi de 34,46 e o nível de sobrecarga que apresentaram foi sobrecarga moderada (56%). Houve correlação negativa entre a sobrecarga e a criança apresentar comorbidades, frequentar creche ou escola e a criança estar em acompanhamento especializado, e o cuidador que não vive com parceiro apresentou chance de maior nível de sobrecarga. Conclusões: a presença de cardiopatia congênita em crianças constitui causa de sobrecarga física, social, financeira e emocional em seus cuidadores primários. É inquestionável que haja vinculação da família nas ações de cuidados prestados em serviços de saúde, sobretudo em consultas ambulatoriais.

Objectives: To indentify the level of burden of primary caregivers of children with congenital heart disease and to analyze associated factors. Methods: This is an exploratory cross-sectional study with a quantitative approach. A total of 100 primary caregivers of children aged between 0 and 12 years old participated in the research. A questionnaire was used to characterize the sample (caregivers and children) and the Brazilian version of the Caregiver Burden Scale (Zarit Burden Interview) to characterize the sample (caregivers and children). assess the level of overload. Data analysis was performed using the SPSS 21 program and applied bivariate and multivariate linear regression statistical tests, in addition to descriptive statistics, considering p<0.05 for significance. Results: The average score of physical, social and emotional burden of these caregiver mothers was 34.46 and the level of burden they presented was moderate burden (56%). There was a negative correlation between burden and the child presenting comorbidities, attending daycare or school and the child being in specialized monitoring and the caregiver who does not live with a partner was likely to have a higher level of burden. Conclusions: The presence of congenital heart disease in children is a cause of great physical, social, financial and emotional strain on their primary caregivers. It is unquestionable that there is a link between the family in the care actions provided in health services, especially in outpatient consultations.

Objetivos: Identificar el nivel de sobrecarga de los cuidadores principales de niños con cardiopatías congénitas y analizar los factores asociados. Métodos: Se trata de un estudio transversal exploratorio con abordaje cuantitativo. Participaron de la investigación 100 cuidadores principales de niños con edades entre 0 y 12 años, se utilizó un cuestionario para caracterizar la muestra (cuidadores y niños) y la versión brasileña de la Caregiver Burden Scale (Zarit Burden Interview) para caracterizar la muestra. muestra (cuidadores y niños) evaluar el nivel de sobrecarga. El análisis de los datos se realizó con el programa SPSS 21 y se aplicaron pruebas estadísticas de regresión lineal bivariada y multivariada, además de estadística descriptiva, considerando p<0,05 para la significación. Resultados: El puntaje promedio de carga física, social y emocional de estas madres cuidadoras fue de 34,46 y el nivel de carga que presentaron fue carga moderada (56%). Hubo una correlación negativa entre la sobrecarga y el niño que presentaba comorbilidades, asistía a la guardería oa la escuela y el niño que estaba en seguimiento especializado y el cuidador que no vivía en pareja era probable que tuviera un mayor nivel de sobrecarga. Conclusiones:La presencia de cardiopatías congénitas en los niños es causa de gran desgaste físico, social, económico y emocional en sus cuidadores primarios. Es incuestionable que existe un vínculo entre la familia en las acciones de cuidado que se brindan en los servicios de salud, especialmente en las consultas externas.

Children with congenital heart disease in COVID-19 pandemic times: an integrative review / Niño con cardiopatía congénita en tiempos de la pandemia de COVID-19: revisión integradora / Criança com cardiopatia congênita em tempos de pandemia de COVID-19: revisão integrativa

ABSTRACT Objective: to identify the scientific production in health about children with congenital heart disease in COVID-19 pandemic times. Method: this is an integrative review, carried out in June 2020 in the information resources Latin American and Caribbean Literature in Health Sciences (LILACS), National Library of Medicine, National Institutes of Health (PubMed), Scientific Electronic Library Online Journal Portal (SciELO) and Brazilian Institute of Information Science and Technology (Oasis Ibict). Results: 14 studies composed this review. Most studies pointed to tests, interventional procedures and surgery for children with heart disease in pandemic times; others discussed possible complications of COVID-19 among children with congenital heart disease. Conclusion: the incipient production of studies and the weak level of evidence denote an important knowledge gap so far, highlighting the need for studies with strong scientific evidence for the formulation of care guidelines aimed at children with heart disease.

RESUMEN Objetivo: identificar la producción científica en el área de la salud sobre niños con cardiopatías congénitas en tiempos de la pandemia del COVID-19. Método: revisión integradora, realizada en junio de 2020 en los recursos de información Literatura Latinoamericana y del Caribe en Ciencias de la Salud (LILACS), National Library of Medicine National Institutes of Health (PubMed), Portal de Revistas Scientific Eletronic Library Online (SciELO) y Instituto de Ciencia y Tecnología de la Información (Oasis Ibict). Resultados: 14 estudios componen esta revisión. La mayoría de los estudios apuntaron a pruebas, procedimientos de intervención y cirugía para niños con enfermedades cardíacas en tiempos de pandemia; otros discutieron las posibles complicaciones del COVID-19 entre los niños con cardiopatías congénitas. Conclusión: la incipiente producción de estudios y el débil nivel de evidencia denotan un importante vacío de conocimiento hasta el momento, destacando la necesidad de estudios con fuerte evidencia científica para la formulación de guías de atención dirigidas a niños con cardiopatías.

RESUMO Objetivo: identificar a produção científica da área da saúde acerca da criança com cardiopatia congênita em tempos de pandemia de COVID-19. Método: revisão integrativa, realizada em junho de 2020 nos recursos informacionais Literatura Latino Americana e do Caribe em Ciências da Saúde (LILACS), National Library of Medicine National Institutes of Health (PubMed), Portal de Revistas Scientific Eletronic Library Online (SciELO) e Instituto Brasileiro de Informação Ciência e Tecnologia (Oasis Ibict). Resultados: 14 estudos compuseram esta revisão. A maioria dos estudos apontava para a realização de exames, procedimentos intervencionistas e cirurgia da criança cardiopata em tempos de pandemia; outros teceram considerações sobre possíveis complicações da COVID-19 entre crianças com cardiopatia congênita. Conclusão: a incipiente produção de estudos e o fraco nível de evidência denotam importante lacuna de conhecimento até o momento, ressaltando a necessidade de estudos com forte evidência científica para a formulação de diretrizes assistenciais voltadas à criança cardiopata.

Progress in the Role of Mechanical Stimulus in Cardiac Development / 中国医学科学院学报

Mechanical stimulus is critical to cardiovascular development during embryogenesis period.The mechanoreceptors of endocardial cells and cardiac myocytes may sense mechanical signals and initiate signal transduction that induce gene expression at a cellular level,and then translate molecular-level events into tissue-level deformations,thus guiding embryo development.This review summarizes the regulatory roles of mechanical signals in the early cardiac development including the formation of heart tube,looping,valve and septal morphogenesis,ventricular development and maturation.Further,we discuss the potential mechanical transduction mechanisms of platelet endothelial cell adhesion molecule 1-vascular endothelial-cadherin-vascular endothelial growth factor receptor 2 complex,primary cilia,ion channels,and other mechanical sensors that affect some cardiac malformations.

Experiencias materno-paternas en el afrontamiento a la cardiopatía congénita infantil / Maternal and paternal experiences confronting childhood congenital heart disease

RESUMEN Fundamento: Las cardiopatías congénitas son las más frecuentes de las enfermedades crónicas infantiles; inciden no solo en el bienestar psicológico del niño o niña sino también en el de toda su familia, a través de una compleja multitud de variables sintomáticas, terapéuticas, emocionales y sociales que impactan de forma importante los procesos de la dinámica y del funcionamiento de la vida familiar. Objetivo: Explorar las experiencias de madres y padres en el afrontamiento a la cardiopatía congénita infantil. Metodología: Se realizó un estudio de casos múltiples con un enfoque cualitativo dirigido a las madres y los padres de 3 niños que padecen cardiopatías congénitas. Se utilizaron la observación, entrevistas semiestructuradas, escala valorativa y triangulación de la información. Resultados: El diagnóstico, las intervenciones quirúrgicas y las hospitalizaciones frecuentes provocaron las reacciones más impactantes entre los padres, caracterizadas por miedo, impotencia, incertidumbre, angustia. Sucedieron cambios en las rutinas familiares. Los padres viven con temor a que su hijo se descompense, los niños reciben trato privilegiado, crianza permisiva y sobreprotectora y limitaciones en el desarrollo social. Conclusiones: Los padres acumulan experiencias traumáticas, desoladoras, impactantes, generadoras de ansiedad, miedos y preocupaciones por el pronóstico y por los riesgos que entraña para la vida de sus hijos, sin lograr una adaptación saludable a la situación generada por la enfermedad, cuyo afrontamiento no ha sido positivo.

ABSTRACT Background: Congenital heart diseases are the most common chronic childhood illnesses; they affect not only the psychological well-being of the child but also the whole family through complex lots of symptomatic, therapeutic, emotional and social variables that have an important impact on the dynamics and functioning of the family. Objective: To explore on the experiences of mothers and fathers facing childhood congenital heart diseases. Methodology: A multiple case study with a qualitative approach was conducted with mothers and fathers of 3 children suffering from congenital heart diseases. Observation, semi-structured interviews, a rating scale and information triangulation were used. Results: Diagnosis, surgical interventions and frequent hospitalizations triggered the most shocking reactions among parents, characterized by fear, helplessness, insecurity, distress. Changes in family routines occurred. Parents live in fear their children to be unbalanced, children receive privileged treatment, permissive and overprotective education so, limits for their social development. Conclusions: Parents accumulate traumatic, devastating, shocking experiences, generating anxiety, fears and worries about the prognosis and the risks involved to their children's live without a healthy adaptation to the situation generated by the disease, which confrontation has not been positive.

Ventana aortopulmonar. Reporte de caso familiar / Aortopulmonary window. Family case report

Resumen Antecedentes: La ventana aortopulmonar (VAP) es un defecto del septo aortopulmonar, una cardiopatía congénita rara. Es una comunicación entre la aorta ascendente y el tronco pulmonar y/o rama pulmonar derecha, en presencia de ambos planos valvulares sigmoideos separados, lo cual la diferencia del tronco arterioso. Se clasifica en cuatro tipos. Objetivo: Reportar un caso familiar de VAP. Paciente de sexo femenino de 1 mes de vida extrauterina que reingresa por insuficiencia cardiaca congestivo-venosa. Discusión: Se discute la alta letalidad y la recurrencia de esta rara malformación cardiaca. Conclusión: El asesoramiento genético es muy importante.

Abstract Background: The Aorto pulmonary window in one of the rarest congenital heart diseases. That results from a defect between the main pulmonary artery and the proximal aorta is named aortopulmonary window (APW) and differences between the Truncus arteriosus. Classification of the APW have been described 4 types. Objective: To report a Family case with APW. 1 month-old female who was refered to our center by congestive heart failure. Discussion: The high incidence of dead and the recurrence of this rarest congenital heart diseases. Conclusion: The Genetic assessment is very important in this Patology.

Fístula da Artéria Coronária: Associação entre Padrões de Trajetos, Características Clínicas e Cardiopatias Congênitas / Coronary Artery Fistula: Association between Pathway Patterns, Clinical Features and Congenital Heart Disease

Resumo Fundamento A fístula da artéria coronária (FAC) é uma conexão direta entre uma ou mais artérias coronárias e câmaras cardíacas ou um grande vaso; pode estar associada à cardiopatia congênita. Objetivo Estabelecer os padrões de trajetos de FAC a partir de dados ecocardiográficos e correlacioná-los com aspectos clínicos e cardiopatias congênitas. Métodos Um total de 7.183 prontuários médicos de crianças menores de 5 anos de idade com cardiopatia submetidas a ecodopplercardiograma colorido foram analisados utilizando o teste de correlação de Spearman para associar sinais, sintomas e cardiopatia à FAC, com nível de significância de 5%. Resultados Vinte e seis crianças (0,0036%) apresentaram FAC, nos seguintes trajetos: da artéria coronária direita para o ventrículo direito (26,92%), da artéria coronária esquerda para o ventrículo direito (23,08%), do ramo interventricular anterior para o ventrículo direito (23,08%), da artéria coronária direita para o átrio direito (11,54%), da artéria coronária esquerda para o tronco pulmonar (7,69%) e do ramo interventricular anterior para o tronco pulmonar (7,69%). Em 57,69% dos pacientes, houve uma correlação positiva entre sintomas e a FAC (p = 0,445), relacionada à dispneia ou cianose (53,84%). Em 96,15%, a cardiopatia congênita estava associada à FAC; principalmente, a comunicação interventricular e a comunicação interatrial, em 34,62% dos casos, correlacionaram-se positivamente com a FAC (p = 0,295). O trajeto da FAC foi representado em três dimensões pelo software de modelagem, texturização e animação Cinema 4D R19. Conclusão A FAC é uma entidade anatômica incomum que apresenta quadro clínico compatível com dispneia e cianose e está associada a cardiopatias congênitas, principalmente com a CIV ou a CIA. De acordo com as análises ecocardiográficas, as fístulas na ACD, na ACE ou no RIVA representam aproximadamente um terço dos pacientes, com trajeto prioritário para as câmaras cardíacas direitas.

Abstract Background Coronary artery fistula (CAF) is a direct connection of one or more coronary arteries to cardiac chambers or a large vessel; it may be associated with congenital heart disease. Objective To establish CAF pathway patterns from echocardiographic data and to correlate them with clinical aspects and congenital heart disease. Methods A total of 7.183 medical records of children under the age of five years with cardiac disease submitted to color Doppler echocardiography and Spearman's Correlation test were used to associate signs and symptoms and cardiopathy to CAF with a significance level of 5%. Results Twenty six children (0.0036%) presented CAF: from the right coronary artery (RCA) to the right ventricle (RV) 26.92%, from the left coronary artery (LCA) to the RV 23.08%, from the anterior interventricular branch (AIVB) to RV 23.08%, RCA to right atrium (RA) 11.54%, LCA for pulmonary trunk (PT) 7.69% or AIVB for PT 7.69%. In 57.69% of the patients, there was a positively correlated symptomatology to CAF with p=0.445 related to dyspnea or cyanosis (53.84%); in 96.15%, congenital heart disease associated with CAF, mainly interventricular communication (IVC) or interatrial communication (IAC) in 34.62% positively correlated to CAF with p=0.295. CAF pathway was represented in three dimensions by software modeling, texturing and animation Cinema 4D R19. Conclusion CAF is an uncommon anatomical entity that presents a clinical picture compatible with dyspnea and cyanosis, and this is associated with congenital heart disease, mainly with IVC or IAC. According to echocardiographic analyzes, fistulas in RCA, LCA, or AIVB represent about one-third of the patients, with a priority pathway for right heart chambers.

Primary peritoneal drainage in neonates with necrotizing enterocolitis associated with congenital heart disease: a single experience in a Brazilian tertiary center

Necrotizing enterocolitis (NEC) is a common condition in preterm infants. The risk factors that contribute to NEC include asphyxia, apnea, hypotension, sepsis, and congenital heart diseases (CHD). The objective of this study was to evaluate the association between the treatment (surgery or drainage) and unfavorable outcomes in neonates with NEC and congenital heart diseases (NEC+CHD). A 19-year retrospective cohort study was conducted (2000-2019). Inclusion criterion was NEC Bell II stage. Exclusion criteria were associated malformation or genetic syndrome and those who did not undergo echocardiography or had a Bell I diagnosis. We included 100 neonates: NEC (n=52) and NEC+CHD (n=48). The groups were subdivided into NEC patients undergoing surgery (NECS, n=31), NEC patients undergoing peritoneal drainage (NECD, n=19), NEC+CHD patients undergoing surgery (NECCAS, n=21), and NEC+CHD patients who were drained (NECCAD, n=29). Multivariate analysis was performed to estimate the relative risk of death and the length of stay. Covariates were birth weight and gestational age. The group characteristics were similar. The adjusted relative risk of death was higher in the drainage groups [NECD (Adj RR=2.70 (95%CI: 1.47; 4.97) and NECCAD (Adj RR=1.97 (95%CI: 1.08; 3.61)], and they had the shortest time to death: NECD=8.72 (95%CI: 3.10; 24.54) and NECCAD=5.32 (95%CI: 1.95; 14.44). We concluded that performing primary peritoneal drainage in neonates with or without CHD did not improve the number of days of life, did not decrease the risk of death, and was associated with a higher mortality in newborns with NEC and clinical instability.

Risk factors for deleyed recovery after surgical closure of congenital ventricular septal defect in infants with low weight / 中国胸心血管外科临床杂志

@#Objective    To reveal the risk factors for delayed recovery and complications in infants with weight≤ 5.0 kg after surgical ventricular septal defect (VSD) closure. Methods    We retrospectively reviewed a consecutive series of 86 patients with weight≤5.0 kg who were admitted to our institution for surgical VSD closure between January 2016 and July 2019, including 31 males and 55 females with an age of 17-266 (80.3±40.4) d and a weight of 2.5-5.0 (4.4±0.6) kg. The VSDs were divided into perimembranous (n=65, 75.6%), subaortic (n=17, 19.8%) and subaortic combined muscular types (n=4, 4.7%). Mechanical ventilation (MV) time≥24 h or ICU stay≥72 h were defined as delayed recovery. Death, sudden circulatory arrest, complete heart block requiring a permanent or temporary pacemaker implantation, neurological complications, reoperation (for residue shunt or valvular regurgitation), reintubation and diaphragmatic paralysis were considered as significant major adverse events. Results    There was no death, reoperation due to residual VSD or neurological complication. Totally 51 (59.3%) patients had MV timec≥24 h and 51 (59.3%) patients stayed in the ICU≥ 72 h. Two (2.3%) patients required temporary pacemaker and six (7.0%) patients required reintubation. During the follow-up of 3-36 (15.8±8.8) months, 1 patient died of pneumonia after discharge, 5 patients suffered mild tricuspid valve regurgitation and 1 patient suffered decreased left ventricular systolic function in the follow-up. No aortic valve injuries occurred. Conclusion    For patients whose weight≤5.0 kg, short-term results of surgical VSD closure are excellent. Low weight and age may prolong MV time; low birth weight and pulmonary hypertension may prolong ICU stay, but are not independent risk factors.

Tendência temporal de mortalidade infantil por Cardiopatias Congênitas no Sul do Brasil, 1996-2016 / Temporal trend of infant mortality for congenital heart disease in southern Brazil, 1996-2016

Introdução: A cardiopatia congênita é considerada a malformação mais comum e a principal causa de óbito entre os defeitos congênitos, apesar de serem subdiagnosticadas no Brasil. Objetivo: Analisar a tendência temporal de mortalidade infantil por cardiopatias congênitas no sul do Brasil entre 1996­2016. Método: Estudo ecológico, com dados sobre mortalidade por cardiopatia congênita do Sistema de Informação sobre Mortalidade. Foram analisadas as taxas de mortalidade, por cardiopatia congênita, por sexo, de 0 a 1 ano de idade, no Sul do Brasil, entre 1996 a 2016, com um total de 9559 óbitos. Para a análise das tendências temporais de mortalidade foram utilizados os coeficientes de mortalidade e o método de regressão linear simples. Resultados: No Brasil houve um acréscimo na tendência temporal da mortalidade por cardiopatia congênita, com queda na região Sul (p<0,05). Ao analisar os estados, houve decréscimo no Paraná (p<0,05) e estabilidade em Santa Catarina (p>0,05) e Rio Grande do Sul (p>0,05). Entre os sexos, houve estabilidade na tendência temporal no sexo feminino (p>0,05) e queda no sexo masculino (p<0,05). Conclusão: Há tendência de acréscimo na taxa de mortalidade infantil por cardiopatia congênita no Brasil, com queda na região Sul. Entre os estados, Paraná foi o que se verificou o maior decréscimo. O sexo masculino possui as maiores taxas de mortalidade infantil por malformação cardíaca, porém demonstra queda na tendência temporal no período estudado.

Introduction: Although underdiagnosed in Brazil, congenital heart disease is considered the most common malformation and the leading cause of death among congenital defects. Objective: To analyse the temporal trend of infant mortality due to congenital heart disease in southern Brazil between 1996­2016. Method: Ecological study with data on mortality due to congenital heart disease extracted from the Mortality Information System. The mortality rates from congenital heart disease by sex, from 0 to 1 year of age in southern Brazil, between 1996 and 2016, were analysed, whit a total of 9559 deaths. For the analysis of the temporal trends of mortality, the mortality coefficients and the simple linear regression method were used. Results: In Brazil there was an increase in the temporal trend of mortality due to congenital heart disease, with a decrease in the southern region (p<0.05). When analysing the states, there was a decrease in Paraná (p <0.05) and stability in Santa Catarina (p> 0.05) and Rio Grande do Sul (p> 0.05). Between genders, there was stability in temporal trend for females (p> 0.05) and a decrease for males (p <0.05). Conclusion: There is a tendency of increase in the infant mortality rate due to congenital heart disease in Brazil, with decrease in the South region. Among the states, Paraná was the one with the largest decrease. The male gender has the highest infant mortality rates due to cardiac malformation, but demonstrates a decrease in the temporal trend in the period studied.

Trasplante cardíaco en adultos con cardiopatías congénitas / Heart Transplantation in Adult Patients with Congenital Heart Disease

RESUMEN Introducción: En las últimas décadas los pacientes con cardiopatías congénitas (CC) han presentando nuevos desafíos en el manejo de las complicaciones, tanto de la patología originaria como de las cirugías correctoras que les han permitido llegar a la adultez. Objetivo: Comunicar los resultados y la evolución de los pacientes con CC que hayan sido evaluados para trasplante en un centro de alta complejidad. Se utilizó la base de datos institucional, y se analizaron los datos de 11 pacientes evaluados para trasplante con diversas patologías congénitas. Accedieron al trasplante 5 de ellos con una sobrevida a 1,6 años del 80%. Los pacientes que se hallaban en lista y no se trasplantaron tuvieron una mortalidad del 66%, y los descartados por comorbilidades presentaron una mortalidad del 35%. Conclusión: El trasplante cardíaco en adultos con CC tiene un riesgo periprocedimiento más elevado que para las cardiopatías adquiridas (CA). No obstante, los que sobreviven el primer año postrasplante tienen una excelente evolución a largo plazo.

ABSTRACT Background: Over recent decades, congenital heart disease (CHD) patients have posed new challenges in the management of complications, both of the original condition as of the corrective surgeries that have allowed them to reach adulthood. Objective: The aim of this study was to report the outcomes and evolution of CHD patients who had been evaluated for transplantation in a tertiary care center. Using the institutional database, data from 11 patients with different congenital diseases were evaluated for transplantation. A total of 5 patients underwent transplantation, with a 1.6-year survival rate of 80%. Mortality rate was 66% for patients who were on the waiting list but were not transplanted, and 35% for those who were ruled out due to comorbidities. Conclusion: Heart transplantation in CHD adult patients present a higher periprocedural risk than in patients with acquired heart diseases. However, those who survive the first post-transplant year have an excellent long-term outcome.

Primera experiencia en telemedicina dentro de un programa nacional / First experience in telemedicine within a national program

El Programa Nacional de Cardiopatías Congénitas (PNCC) inició el Proyecto de telemedicina en Cardiopatías Congénitas (CC) en el 2015. Los principales objetivos son promover el asesoramiento para el diagnóstico cardiológico en la patología compleja del niño o del feto, promover el conocimiento de patologías complejas de baja prevalencia para su pesquisa precoz, acompañar el proceso de mejora continua en el diagnóstico de CC en hospitales de referencia provinciales y promover la docencia y la capacitación entre los distintos actores de la red. Las videoconferencias fueron el principal método de contacto. Las provincias que más participaron fueron Buenos Aires, Neuquén y Misiones; el 55% de las consultas correspondieron a pacientes portadores de CC, un 20% a embarazadas con diagnóstico fetal de CC, 14% de arritmias y 11% otros motivos. Se mostraron imágenes de ecocardiografía en la mayoría de las conexiones.En un 35% se modificaron conductas, evitando el traslado de pacientes en un 23%. Conclusión: La telemedicina en CC representa una alternativa que llega de modo más efectivo al médico a través de la consulta y respuesta a distancia, optimizando los diagnósticos y las derivaciones oportunas. Además, difunde el conocimiento entre los centros de baja a los de alta complejidad, disminuye las asimetrías en el diagnóstico para los pacientes del interior del país, evita el traslado innecesario de pacientes y afianza el trabajo en red. Se presenta como un modelo innovador para la réplica hacia otras especialidades.(AU)

In 2015, the Project of Telemedicine for Congenital Heart Defects (CHD) was initiated for the National Program of Congenital Heart Disease (NPCHD). The main aims are to provide counseling for the cardiology diagnosis in complex conditions in the child or the fetus, promote knowledge on complex diseases of low prevalence for early screening, accompany the process of continuous improvement in the diagnosis of CHD at provincial reference hospitals, and encourage teaching and training of the different participants in the network. Videoconferences were the main method of interaction. The provinces that participated the most were Buenos Aires, Neuquen, and Misiones; 55% of the consultations concerned patients with CHD, 20% pregnant women with a prenatal diagnosis of CHD, 14% arrhythmias, and 11% other reasons. Echocardiography images were shown in the majority of the contacts. In 35% behaviors were modified, avoiding the transfer of patients in 23%. Conclusion: In CHD, telemedicine is an alternative that reaches physicians more effectively through outreach consultation and response, optimizing timely diagnosis and referrals. In addition, this modality disseminates knowledge among low-level and high-level centers, reduces asymmetries in diagnosis for patients from the provinces, avoids unnecessary transfer of patients, and strengthens networking. This innovative modality may serve as a model for other specialties. (AU)

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.