Paternal occupational exposures and infant congenital heart defects in the Japan Environment and Children's Study

BACKGROUND@#Few prospective studies have investigated the association between paternal occupational exposures and risk of infant congenital heart defects (CHDs). We investigated the associations between paternal occupational exposures, frequency of use, and concurrent or sequential exposure to a mixture of compounds and the risk of infant CHDs.@*METHODS@#Our study examined 28,866 participants in the Japan Environment and Children's Study. Logistic regression analysis was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs) associated with paternal occupational exposures during the 3 months until pregnancy was noticed after adjustment for potential confounding factors of the infant CHDs. CHD diagnosis was ascertained from medical record.@*RESULTS@#In total, 175 were diagnosed with infant CHDs. The number of fathers who were exposed to the following substances at least once a month were: 11,533 for photo copying machine/laser printer, 10,326 for permanent marker, 8,226 for soluble paint/inkjet printer, 6,188 for kerosene/petroleum/benzene/gasoline, 4,173 for organic solvents, 3,433 for chlorine bleach/germicide, 2,962 for engine oil, 2,931 for insecticide, 2,460 for medical sterilizing disinfectant, 1,786 for welding fumes, 1,614 for dyestuffs, 1,247 for any products containing lead-like solder, 986 for herbicide, 919 for radiation/radioactive substances/isotopes, 837 for lead-free solder, 341 for microbes, 319 for formalin/formaldehyde, 301 for agricultural chemical not listed above or unidentified, 196 for general anesthetic for surgery at hospital, 171 for anti-cancer drug, 147 for chromium/arsenic/cadmium, 88 for mercury and 833 for other chemical substances. Paternal occupational exposure regularly to photo copying machine or laser printer and soluble paint/inkjet printer were associated with higher risks of infant CHDs: the adjusted ORs (95%CIs) were 1.38 (1.00-1.91) and 1.60 (1.08-2.37), respectively. The higher risks were also observed for occasional exposure to engine oil, any products containing lead-like solder lead-free solder, and microbes; the adjusted ORs (95%CIs) were 1.68 (1.02-2.77), 2.03 (1.06-3.88), 3.45 (1.85-6.43), and 4.51, (1.63-12.49), respectively.@*CONCLUSIONS@#Periconceptional paternal occupational exposure was associated with a higher risk of infant CHDs. Further studies using biomarkers of the association between paternal occupational exposure and infant CHDs are warranted.

Anomalías congénitas frecuentes en Uruguay entre 2011 y 2014: importancia de un registro para la evaluación de las necesidades de salud / Frequent congenital anomalies in Uruguay in 2011-2014; importance of a medical record for health assessment / Anomalias congênitas frequentes no Uruguai entre 2011-2014, importância de um registro para avaliação das necessidades de saúde

Introducción: las anomalías congénitas (AC) son un problema de salud pública con impacto en la infancia, la mortalidad infantil (MI) y la discapacidad. En Uruguay, así como en otros países desarrollados, las AC y la prematuridad son las principales causas de MI. Objetivos: el objetivo de este trabajo fue analizar las anomalías más frecuentes en el país entre 2011 y 2014, y evaluar los factores de riesgo. Material y método: los datos se obtuvieron del Registro Nacional de Defectos Congénitos y Enfermedades Raras y Estadísticas Vitales del Ministerio de Salud Pública. Resultados: se determinó que prácticamente la mitad de los casos fueron: cardiopatías congénitas, síndrome de Down y defectos del tubo neural (anencefalia, encefalocele y mielomeningocele), representando 0,42% (812/191.820) y 2,85% (38/1334) de los nacidos vivos (NV) y de los óbitos fetales respectivamente. Las prevalencias por 10.000 NV y OF fueron: 38,52 y 149,93 para cardiopatías congénitas; 3,6 y 7,5 para síndrome de Down; 2,1 y 127,4 para defectos del tubo neural. La edad materna avanzada fue el principal factor de riesgo de síndrome de Down. Los factores de riesgo observados en las tres anomalías seleccionadas fueron: prematurez, depresión neonatal y bajo peso al nacer. Conclusiones: las anomalías congénitas en general y las seleccionadas en este trabajo en particular, constituyen una causa relevante de morbimortalidad en el período neonatal e infantil, plausibles de prevención. El diagnóstico temprano es fundamental para planificar servicios de salud especializados. Los resultados aquí presentados se pueden utilizar como línea de base para medir el impacto de las acciones de salud a nivel nacional.

Introduction: congenital anomalies (CA) are a public health problem with an impact on childhood, infant mortality (IM) and disability. In Uruguay, as well as in other developed countries, CA and prematurity are the main causes of IM. Objectives: analyze the most frequent anomalies in the country in 2011-2014 and evaluate risk factors. Material and methods: the data were obtained from the National Record of Congenital Defects and Rare Diseases and health statistics from the Ministry of Public Health. Results: it was determined that practically half of the cases were: congenital heart disease, Down syndrome and Neural Tube Defects (anencephaly, encephalocele and myelomeningocele), accounting for 0.42% (812/191,820) and 2.85% (38/1334) of live births and stillbirths respectively. The prevalence per 10,000 NV and stillbirths were: 38.52 and 149.93 for Congenital Heart Disease; 3.6 and 7.5 for Down Syndrome; 2.1 and 127.4 for neural tube defects. Advanced maternal age was the main risk factor for Down syndrome. The risk factors observed in the three selected anomalies were: prematurity, neonatal depression and low birth weight. Conclusions: congenital anomalies in general, and those selected in this study in particular, are a relevant cause of morbidity and mortality in newborns and infants, likely to be prevented. Early diagnosis is essential for planning specialized health services. The results presented in the present paper can be used as a baseline to measure the impact of health actions at national level.

Introdução: as anomalias congênitas (AC) são um problema de saúde pública com impacto na infância, na mortalidade infantil (MI) e na deficiência. No Uruguai, assim como em outros países desenvolvidos as AC e prematuridade são as principais causas de MI. Objetivos: analisar as anomalias mais frequentes no país, entre 2011-2014 e avaliar os fatores de risco. Material e métodos: os dados foram obtidos do Registro Nacional de Defeitos Congênitos e Doenças Raras e estatísticas vitais do Ministério da Saúde Pública. Resultados: determinou-se que praticamente a metade dos casos eram: cardiopatias congênitas, Síndrome de Down e Defeitos do tubo neural (anencefalia, encefalocele e mielomeningocele), representando 0,42% (812/191.820) e 2,85% (38/1334) de nascidos vivos-NV e natimortos, respectivamente. As prevalências por 10.000 NV e natimortos foram: 38,52 e 149,93 para Cardiopatia Congênita; 3,6 e 7,5 para Síndrome de Down; 2,1 e 127,4 para defeitos do tubo neural. A idade materna avançada foi o principal fator de risco para a síndrome de Down. Os fatores de risco observados nas três anomalias selecionadas foram: prematuridade, depressão neonatal e baixo peso ao nascer. Conclusões: as anomalias congênitas em geral e as selecionadas neste estudo em particular, constituem causa relevante de morbimortalidade no período neonatal e infantil, possível de ser prevenida. O diagnóstico precoce é fundamental para o planejamento de serviços de saúde especializados. Os resultados aqui apresentados podem ser usados como base para medir o impacto das ações de saúde realizadas a nível nacional.

Insuficiencia mitral congénita en el adulto: reporte de un caso y revisión de la literatura / Congenital mitral regurgitation in adults: a case report and literature review

Congenital mitral valve disease is a rare form of mitral regurgitation. The etiological diagnosis is often challenging. The transthoracic echocardiogram is presented as a good initial approach method. The case of a 29-year-old patient referred for the diagnosis of severe congenital mitral regurgitation in parachute is presented. This report aims to illustrate the clinical and echocardiographic presentation of congenital mitral regurgitation.

Incidencia de cardiopatías congénitas en pacientes ingresados en la Unidad de Neonatología del Hospital Infantil Robert Reid Cabral, 2016-2018 / Incidence of congenital heart diseases in patients admitted to the Neonatal Unit of the Robert Reid Cabral Children's Hospital, 2016-2018

Introducción: las cardiopatías congénitas son alteraciones estructurales del corazón y/o grandes vasos que se desarrollan durante la embriogénesis, existe un aumento aparente de la incidencia de las cardiopatías congénitas (CC) en las investigaciones recientes, especialmente de las cardiopatías acianógenas, como la comunicación interventricular (CIV) e interauricular (CIA), así como en menor grado otras cardiopatías, como transposición de grandes vasos (TGV) y el síndrome de corazón izquierdo hipoplásico (SCIH). Material y métodos: estudio observacional, descriptivo, transversal y retrospectivo sobre la incidencia de cardiopatías congénitas en pacientes ingresados en la Unidad de Neonatología del Hospital Infantil Robert Reid Cabral durante el período 2016-2018 (hasta la semana epidemiológica #38). La muestra fue de 76 pacientes (n=76). Resultados: la incidencia de cardiopatías congénitas fue 4.97 %. El 59.2 % eran masculino. Mas de 65 % de pacientes procedían de la región este del país. La disnea representó más de 70 % de los motivos de consulta, seguida de cianosis. 35.5 % de las cardiopatías correspondieron a CIV, que fue la patología cardíaca congénita más frecuentemente presentada, seguida por CIA. Conclusión: observamos una incidencia global de cardiopatías congénitas de 4.97 % con predominio en sexo masculino. Los pacientes procedían de la región este en más de la mitad de los casos, y presentaron con mayor frecuencia disnea como motivo de consulta. La incidencia más elevada de cardiopatía congénita fue la CIV

Introduction: Congenital heart diseases are structural alterations of the heart and/or great vessels that develop during embryogenesis. There is an apparent increase in the incidence of congenital heart disease (CHD) in recent research, especially acyanotic heart disease, such as communication interventricular (VSD) and interatrial (ASD), as well as to a lesser degree other heart diseases, such as transposition of the great vessels (TGV) and hypoplastic left heart syndrome (HLHS). Material and methods: Observational, descriptive, cross-sectional and retrospective study on the incidence of congenital heart disease in patients admitted to the Neonatology Unit of the Robert Reid Cabral Children's Hospital during the period 2016-2018 (until epidemiological week #38). The sample was 76 patients (n=76). Results: The incidence of congenital heart disease was 4.97%. 59.2% were male. More than 65% of patients came from the eastern region of the country. Dyspnea represented more than 70% of the reasons for consultation, followed by cyanosis. 35.5% of the heart diseases corresponded to VSD, which was the most frequently presented congenital heart disease, followed by ASD. Conclusion: We observed a global incidence of congenital heart disease of 4.97% with a predominance in males. The patients came from the eastern region in more than half of the cases, and more frequently presented dyspnea as a reason for consultation. The highest incidence of congenital heart disease was VSD

Detección de cardiopatías congénitas críticas en recién nacidos en Argentina a través del sistema nacional de vigilancia de anomalías congénitas (RENAC) / Detection of critical congenital heart disease among newborns in Argentina through the national surveillance system of congenital heart disease (RENAC)

Introducción. Las cardiopatías congénitas críticas (CCC) son las anomalías estructurales del corazón ductus-dependientes, que pueden llevar a la muerte o requieren tratamiento invasivo en el primer mes de vida. Objetivo. Conocer la prevalencia y distribución de CCC en recién nacidos de Argentina, en comparación con otros países, y la proporción de detección prenatal y de mortalidad perinatal. Material y métodos. Se utilizó material de la Red Nacional de Anomalías Congénitas de Argentina (RENAC) del período 2009-2018, y de otros sistemas de vigilancia de Estados Unidos (EE. UU.), Europa y Colombia. Para Argentina se analizó la proporción de detección prenatal, mortalidad perinatal y prevalencia de recién nacidos con CCC según jurisdicción y subsector de salud. Resultados. Prevalencia de CCC de 11,46 (IC95 %: 11,02-11,92) cada 10 000 nacimientos. El 43,93 % tuvo detección prenatal y la mortalidad perinatal fue del 25 %. La tetralogía de Fallot fue el defecto específico más frecuente. La prevalencia de CCC y el porcentaje de detección prenatal fue significativamente menor en el subsector público, mientras que la mortalidad perinatal fue mayor en este subsector. La prevalencia de CCC observada fue menor que en los registros de EE. UU. (NBDPN) y Europa (EUROCAT). El registro de Bogotá mostró prevalencias específicas diferentes. Conclusión. La prevalencia de CCC es más baja que la observada en otros países, y aún menor en el sector público de Argentina. Se enfatiza la necesidad de mejorar la detección prenatal e implementar la oximetría de pulso en recién nacidos como prueba de tamizaje obligatorio y universal.

Introduction. Critical congenital heart disease (CCHD) is a term that refers to ductus-dependent structural anomalies of the heart that may be fatal or require invasive management in the first month of life. Objective. To know the prevalence and distribution of CCHD among newborns in Argentina, compared to other countries, and the proportion of prenatal detection and perinatal mortality. Material and methods. Data provided by the National Network of Congenital Anomalies (Red Nacional de Anomalías Congénitas de Argentina, RENAC) for the 2009-2018 period and by other surveillance systems in the United States, Europe, and Colombia were used. For Argentina, the proportion of prenatal detection, perinatal mortality, and CCHD prevalence in newborns by jurisdiction and health system subsector were analyzed. Results. The prevalence of CCHD was 11.46 (95% confidence interval: 11.02-11.92) every 10 000 births. Prenatal detection was possible in 43.93% of cases, and perinatal mortality was 25%. Tetralogy of Fallot was the most frequent specific defect. The prevalence of CCHD and percentage of prenatal detection was significantly lower in the public subsector, whereas perinatal mortality was higher in this subsector. The prevalence of CCHD was lower than in the United States (NBDPN) and European (EUROCAT) registries. The Bogotá Registry showed different specific prevalence values. Conclusion. The prevalence of CCHD is lower than what has been observed in other countries, and even lower in the public sector of Argentina. The need to improve prenatal detection and implement pulse oximetry among newborns as a mandatory and universal screening is emphasized.

Hospitalizaciones por cardiopatías congénitas en la Unidad de Cardiología Pediátrica del Centro Hospitalario Pereira Rossell / Hospitalization due to congenital heart disease at the Pereira Rossell Pediatric Hospital's Cardiology Unit / Internações por cardiopatia cardíaca congênita na Unidade de Cardiologia Pediátrica do Centro Hospitalar Pereira Rossell

Introducción: las cardiopatías congénitas (CC) son patologías frecuentes en pediatría. Son causa de importante morbimortalidad en la infancia. Son pacientes complejos, que requieren abordaje integral, en equipo interdisciplinario. Objetivos: describir las características epidemiológicas, clínicas y evolutivas de niños portadores de CC asistidos en la Unidad de Cardiología del HP-CHPR durante el año 2015, así como su estado nutricional y los factores que pudieran influir en este. Material y método: estudio descriptivo, retrospectivo, a través de la revisión de historias clínicas de pacientes portadores de CC, hospitalizados en la Unidad de Cardiología del HP-CHPR en 2015. Se describieron características epidemiológicas y clínicas de los pacientes. Resultados: en el período evaluado egresaron 63 pacientes, que representaron una tasa de 6,58/1.000 egresos hospitalarios. Tenían un promedio de edad de 23,8 meses. Un total de 8 pacientes fueron diagnosticados en el período prenatal, 54 presentaban CC no cianóticas. Habían sido sometidos a tratamiento quirúrgico 24 niños, 13 con cirugía correctiva. Se diagnosticó desnutrición en 43%. Esta fue más prevalente en portadores de CC cianótica, de defectos complejos, y de cromosomopatías u otras malformaciones. Conclusión: los niños con CC representaron un pequeño porcentaje de quienes requirieron hospitalización en el HP-CHPR. El porcentaje de desnutrición fue elevado. Este estudio confirma la importante morbimortalidad de los niños pequeños portadores de CC. Es importante reconocer el mayor riesgo de estos pacientes y actuar en forma individualizada y oportuna.

Introduction: congenital heart disease (CHD) is a frequent pathology among children. It may cause significant morbidity and mortality during childhood. These are complex patients, who require a comprehensive approach and an interdisciplinary team. Objective: to describe the epidemiological, clinical and evolutionary characteristics of children with CHD assisted in the Cardiology Department of the Children Hospital-CHPR in 2015. Material and Methods: descriptive, retrospective study, review of clinical records of patients with CHD, hospitalized in the Cardiology Department of the Children's Hospital PRHC in 2015. Epidemiological and clinical characteristics of patients were described. Results: 63 patients were hospitalized during the evaluated period, a total rate of 6.58/1.000 discharges. They had an average age of 23.8 months. Eight patients were diagnosed in the prenatal period, 54 presented non-cyanotic CHD. Twenty-four children had undergone surgical treatment, 13 had had corrective surgery. Malnutrition was diagnosed in 43% and it was more prevalent in cyanotic CHD carriers, complex defects, and chromosomopathies or other malformations. Conclusion: CHD represented a small percentage of the children who required hospitalization at the PH-PRHC. The percentage of malnutrition was high. The study confirmed the significant morbidity and mortality of these patients. It is important to recognize the highest risk of these patients and act in a customized and timely fashion.

Introdução: as cardiopatias congênitas (CC) são patologias comuns em pediatria. Elas são uma causa significativa de morbidade e mortalidade na infância. Os pacientes são complexos e requerem uma abordagem integral e uma equipe interdisciplinar. Objetivos: descrever as características epidemiológicas, clínicas e evolutivas de crianças com CC atendidas na Unidade de Cardiologia do Hospital Pediátrico PHPR durante o ano de 2015, descrever seu estado nutricional e os fatores que poderiam influenciá-lo. Material e métodos: estudo descritivo, retrospectivo, feito por meio da revisão de prontuários de pacientes com CC, internados na Unidade de Cardiologia do HP-CHPR em 2015. Foram descritas as características epidemiológicas e clínicas dos pacientes. Resultados: no período avaliado, 63 pacientes receberam alta hospitalar, representando uma taxa de 6,5/1.000 altas hospitalares. Eles tinham uma idade média de 23,8 meses. Oito pacientes foram diagnosticados no período pré-natal, 54 apresentavam CC não cianótica. 24 crianças tinham sido submetidas a tratamento cirúrgico, 13 tinham recebido cirurgia corretiva. 43% foram diagnosticadas com desnutrição, a qual foi mais prevalente em portadores de CC cianótica, defeitos complexos e malformações cromossômicas ou outras. Conclusão: os CCs representaram um pequeno percentual de crianças que necessitaram de internação no HP-CHPR. O percentual de desnutrição foi alto. Este estudo confirma a morbidade e mortalidade significativas de crianças pequenas com CC. É importante reconhecer o risco aumentado desses pacientes e agir de forma individualizada e oportuna.

Congenital Heart Disesase: A Retrospective Analysis from a Tertiary Referral Centre in Portugal

Abstract Background: Congenital heart disease is the leading cause of mortality among all congenital malformations. Objectives: To evaluate the incidence of congenital heart diseases in a central maternity hospital in Portugal from January 2003 to December 2018 and to determine survival in the first year of life. Methods: Retrospective analysis of newborns diagnosed with congenital heart diseases within 72 hours after birth. Malformations were divided according to pathophysiology. Cumulative survival analysis was performed by the Kaplan-Meier test. Stastical significance was set at p <0.05. Results: A total of 297 newborns with cardiac malformation was recorded among 47,198 live births (incidence of 6:1000), 16% associated with extra-cardiac disease. The most frequent congenital heart diseases were left-to-right shunt lesions (n = 216), followed by cyanotic (n = 41), acyanotic obstructive (n = 31) and miscellaneous (n = 9). Seventy (24%) patients had prenatal diagnosis, 88% of them cyanotic defects, and a positive association was found between prenatal diagnosis and mortality (p <0.001). Coarctation of the aorta was associated with gestational diabetes (p = 0.014). Atrial septal defect was more common in females (p = 0.02). Mortality rate due to heart disease was 3.4%. Patients with cyanotic disease, 99%, 97%, 97%, respectively, for patients with left-to-right shunt lesions, and 97%, 97%,97% for those with obstructive lesion cases. Conclusion: The incidence of congenital heart disease was 6:1000, mostly left-to-right shunt lesions. Heart disease accounted for only half of deaths, and cyanotic diseases have a high nonspecific mortality rate.

Caracterização clínico-epidemiológica de crianças e adolescentes portadores de cardiopatia congênita / Clinical-epidemiological characterization of children and adolescents with congenital heart disease / Caracterización clínico-epidemiológica de niños y adolescentes con cardiopatía congénita

Objetivo: Analisar características clínico-epidemiológicas de crianças portadoras de cardiopatia congênita residentes do município de Rio das Ostras referenciadas para unidades especializadas no estado do Rio de Janeiro. Método: Pesquisa descritiva, transversal realizada em duas unidades hospitalares no município do Rio de Janeiro, através da técnica documental retrospectiva por meio de dados nos prontuários. A coleta ocorreu entre setembro de 2018 a fevereiro de 2019. Resultados: Foram analisados 48 prontuários, com maior proporção entre os escolares (33,3%); do sexo masculino (58,3%); Em relação as cardiopatias, verificou-se com maior proporção as acianóticas (62,5%); destacando a comunicação interatrial com (14,6%); seguida da comunicação interventricular (12,5%) e coarctação da aorta (12,5%). Conclusão: A identificação dessas crianças no município de Rio das Ostras e a distribuição destas pelo território nacional são informações imprescindíveis para o planejamento e implementação de programas e políticas públicas que atendam as reais demandas deste segmento populacional

Objetivo: Analizar las características clínicas y epidemiológicas de los niños con cardiopatía congénita que viven en la ciudad de Rio das Ostras remitidos a unidades especializadas en el estado de Río de Janeiro. Método: Investigación descriptiva, transversal realizada en dos hospitales de la ciudad de Río de Janeiro, a través de una técnica documental retrospectiva a través de datos en registros médicos. La recolección se realizó entre septiembre de 2018 y febrero de 2019. Resultados: Se analizaron 48 registros médicos, con una mayor proporción entre los estudiantes (33.3%); hombre (58,3%); Con respecto a la enfermedad cardíaca, hubo una mayor proporción de acianóticos (62.5%); destacando la comunicación interauricular con (14,6%); seguido de comunicación interventricular (12.5%) y coartación aórtica (12.5%). Conclusión: La identificación de estos niños en la ciudad de Rio das Ostras y su distribución en todo el territorio nacional son información esencial para la planificación e implementación de programas y políticas públicas que satisfagan las demandas reales de este segmento de la población

Objective: To analyze the clinical and epidemiological characteristics of children with congenital heart disease living in the city of Rio das Ostras referred to specialized units in the state of Rio de Janeiro. Method: Descriptive, cross-sectional research conducted in two hospitals in the city of Rio de Janeiro, through retrospective documentary technique through data in medical records. The collection took place between September 2018 and February 2019. Results: 48 medical records were analyzed, with a higher proportion among students (33.3%); male (58.3%); Regarding heart disease, there was a greater proportion of acyanotic (62.5%); highlighting interatrial communication with (14.6%); followed by interventricular communication (12.5%) and aortic coarctation (12.5%). Conclusion: The identification of these children in the city of Rio das Ostras and their distribution throughout the national territory are essential information for the planning and implementation of programs and public policies that meet the real demands of this population segment

Fetal echocardiography indications and lack of association between abnormal exams and advanced maternal age: A cross-sectional study - Fetal abnormal echocardiography / Indicações para realização de ecocardiografia fetal e ausência de associação entre exames alterados e idade materna avançada: Um estudo transversal - Ecocardiografia fetal anormal

Abstract Objective To analyze the most frequent referrals for fetal echocardiography, including advanced maternal age and its association with abnormal results. Methods We included all pregnant women referred to perform fetal echocardiography (gestational age 22-32 weeks) in 2 health centers in Rio de Janeiro, from June 2015 to June 2016. Advanced maternal age was considered when age was > 35 years at the time of delivery). Referral reasons and results were recorded, according to the Brazilian Fetal Cardiology Statement. Crude and adjusted prevalence ratios were calculated (Poisson regression). We considered p < 0.05 as significant. Results A total of 1,221 tests were analyzed. Abnormal fetal echocardiography was observed in 14.82% of the cases. The most frequent abnormalities were interventricular septal defect (6.39%), septal hypertrophy (3.35%) and atrioventricular septal defect (1.14%). Routine exams were performed in 559 women, 289 were referred for advanced maternal age and 373 were referred according to the Brazilian FetalCardiology Statement criteria. An obstetric ultrasound suggesting fetal cardiacabnormality, maternal diabetes, increased nuchal translucency, and obstetric ultrasound suggesting a noncardiac abnormality were strongly associated with an abnormal fetal echocardiography. Abnormal results were not more frequent in women with advanced maternal age when compared with the rest of the study group. Conclusions It was observed that routine exams and advancedmaternal age referrals were very frequent. Those exams were not associated to fetal echocardiography abnormalities. In this scenario, when the obstetric ultrasound suggests a fetal cardiac

Resumo Objetivo Analisar as indicações mais frequentes para realização de ecocardiografia fetal, incluindo idade materna avançada, e a associação destas com exames alterados. Métodos Foram incluídas todas as gestantes que realizaram ecocardiografia fetal na idade gestacional entre 22 e 32 semanas, em 2 centros de referência no Rio de Janeiro, no período de junho de 2015 a junho de 2016. Foi considerada idadematerna avançada se no momento do parto a idade materna fosse> 35 anos. As indicações e os resultados dos exames foram registrados, segundo a Diretriz Brasileira de Cardiologia Fetal. Foramcalculadas as razões de prevalência brutas e ajustadas através da regressão de Poisson, considerando-se p < 0,05. Resultados Um total de 1.221 exames foram analisados. A frequência de exame ecocardiográfico alterado foi 14,82%. As alterações mais frequentes foram defeito do septo interventricular (6,39%), hipertrofia septal (3,35%) e defeito do septo atrioventricular (1,14%). Quinhentos e cinquenta e nove exames foram realizados com indicação de rotina, 289 por idade materna avançada e 373 preenchiam critério de acordo com a Diretriz Brasileira de Cardiologia Fetal. O exame ecocardiográfico alterado foi associado ao ultrassom obstétrico sugerindo cardiopatia fetal, ao diabetes materno, à translucência nucal aumentada e ao ultrassom obstétrico sugerindo alteração extracardíaca. Não foi observada maior frequência de exame ecocardiográfico alterado nas gestantes com idade materna avançada, comparado ao restante da amostra. Conclusão Constatou-se elevada frequência de indicações de rotina, e por idade materna avançada isoladamente, que não foram associados a alterações da ecocardiografia fetal. Em nosso meio, quando o ultrassom obstétrico sugere cardiopatia fetal, é muito provável que a ecocardiografia fetal tambémseja anormal. Portanto, o ultrassom obstétrico é um bom método de rastreio pré-natal.

Clinical and Epidemiological Profiles of Patients Admitted to a Pediatric Cardiac Intensive Care Unit

Abstract Background: Congenital and acquired heart diseases are important causes of morbidity and mortality in children. In critical congenital heart defects, when treatment is not adequate, clinical manifestations may lead to death in the neonatal period. Objective: To establish the clinical and epidemiological profile of patients admitted to the pediatric cardiac intensive care unit (UTI) in a tertiary hospital. Methods: This was a cross-sectional study conducted from January 2013 to December 2014, based on analysis of patients' medical records. The study sample was composed of 307 children and adolescents with congenial and acquired heart diseases. The score Risk Adjustement for Congenital Heart Surgery 1 (RACHS-1) was used for categorization of the various surgical procedures. Descriptive statistics were calculated using the Satistical Package for Social Sciences (SPSS). Categorical variables were compared using the Pearson's chi-square test, considering a level of significance of 5%. Results: There was a predominance of patients aged between 28 days and one year (44%). Congenital heart diseases (91.9%) prevailed over acquired heart diseases (8.1%). Extracorporeal circulation was used in 138 patients who underwent surgical procedures, lasting from 12 to 261 minutes. Most patients (88.9%) were discharged from the ICU and 11.1% died. Using the score RACHS-1, corrective cardiac surgery was performed in 75.8% and paliative surgery in 24.2% of the patients. Conclusions: Patients aged between 28 days to one year, with cyanotic congenital heart disease, undergoing cardiac surgery with extracorporeal circulation duration longer than 120 minutes are at a higher risk of death.

Current situation of congenital heart diseases in two public hospitals in the state of Jalisco / Situación real de las cardiopatías congénitas en dos hospitales públicos del estado de Jalisco

Abstract Introduction: Congenital heart disease represents a public health issue worldwide. Objective: To know the number of patients with heart disease treated in two public hospitals of the State of Jalisco, as well as the mortality and resources available to participating hospitals for the care of these patients in the period from 2015 to 2018; the information was requested to the National Transparency Platform, and the database of pediatric cardiology services and pediatric cardiovascular surgery of the participating hospitals were also reviewed. Results: The second level hospital has human resources, but not the material to attend to these patients; so it is not possible to offer any type of palliative or corrective treatment. A total of 624 patients were evaluated, of which 92.2% corresponded to non-critical heart disease; overall mortality was 12% but in critical heart disease it was 79.5%. The third level hospital has human and material resources to care for these patients. During the study period, 289 operations were performed and the overall mortality was 20.4%. Conclusion: Congenital heart disease in the State of Jalisco is an important cause of mortality, with a high incidence and a very limited resolution capacity since the health services in the State of Jalisco for the care of these patients are insufficient and inadequate. It is essential to strengthen the health system for the care for these patients.

Resumen Introducción: Las cardiopatías congénitas representan un problema de salud pública a nivel mundial. Objetivo: Conocer la cantidad de pacientes cardiópatas atendidos en dos hospitales públicos del estado de Jalisco, así como la mortalidad y los recursos con que cuentan los hospitales participantes para la atención de estos pacientes en el período del 2015 al 2018. Se solicitó la información a la Plataforma Nacional de Transparencia y además se revisaron las bases de datos de los servicios de cardiología pediátrica y cirugía cardiovascular pediátrica de los hospitales participantes. Resultados: El hospital de segundo nivel cuenta con los recursos humanos, pero no con el material para atender a estos pacientes, por lo que no es posible ofrecer ningún tipo de tratamiento paliativo o correctivo (sólo se cierran algunos conductos arteriosos en la etapa neonatal). Se valoró a un total de 624 pacientes, de los cuales el 92.2% correspondió a cardiopatías no críticas; la mortalidad global fue del 12% pero en las cardiopatías críticas fue del 79.5%. El hospital de tercer nivel cuenta con recursos humanos y material para atender a estos pacientes; en el período de estudio se realizaron 289 operaciones y la mortalidad global fue del 20.4%. Conclusión: Las cardiopatías congénitas en el estado de Jalisco son una causa importante de mortalidad, con una incidencia elevada y una capacidad de resolución sumamente limitada, ya que los servicios de salud de Jalisco para la atención de estos pacientes son insuficientes e inadecuados. Es esencial fortalecer el sistema de salud para atender a estos pacientes.

Conhecimento sobre a Doença e a Prática de Atividade Física em Crianças e Adolescentes com Cardiopatia Congênita / Knowledge about the Disease and the Practice of Physical Activity in Children and Adolescents with Congenital Heart Disease

Resumo Fundamento O conhecimento sobre a própria doença pode ser importante para o autocuidado em pacientes com vários problemas e abrange a informação sobre o diagnóstico até as implicações clínicas mais importantes. Objetivo Identificar o nível de conhecimento de crianças e adolescentes com cardiopatia congênita (CC) sobre a sua doença, e analisar a relação entre o nível de conhecimento e a prática de atividade física. Métodos Estudo transversal com 335 pacientes com CC, de 8 a 13 anos, acompanhados em um serviço de cardiologia pediátrica de referência no Sul do Brasil. Os pacientes foram entrevistados em relação ao seu conhecimento sobre a CC e foi realizada revisão dos prontuários para obtenção de detalhes sobre a cardiopatia e os procedimentos. Foi utilizado o nível de significância p < 0,05. Resultados Mais de 50% das crianças e adolescentes não sabiam referir o nome de sua doença ou explicá-la. Após OR ajustado (ORaj), mostraram potencial para respostas incorretas ou não saber sua doença os pacientes cianóticos em relação aos acianóticos (ORaj: 2,29; IC95%: 1,76-6,71; p=0,019); crianças com menor nível de escolaridade (ORaj: 2,20; IC95%: 1,81-5,86; p=0,025); e não praticantes de atividade física (ORaj: 1,88; IC95%: 1,09-3,45; p=0,011). Conclusão As crianças e adolescentes cianóticos, com menor nível de escolaridade e que não praticavam de atividade física apresentaram pouco conhecimento sobre a sua doença. Há necessidade do desenvolvimento de estratégias de intervenções educativas para aumento do conhecimento e mudança comportamental na promoção da atividade física, de acordo com a complexidade da CC. (Arq Bras Cardiol. 2020; 114(5):786-792)

Abstract Background Knowledge about the disease itself can be important for self-care in patients with several problems and comprehends information about the diagnosis up to the most important clinical implications. Objective To identify the level of knowledge of children and adolescents with congenital heart disease (CHD) about their illness, and to analyze the association between the level of knowledge and the practice of physical activity. Methods Cross-sectional study with 335 patients with CHD, aged 8 to 13 years, followed at a referral pediatric cardiology service in Southern Brazil. Patients were interviewed regarding their knowledge about CHD and a review of medical records was performed to obtain details on heart disease and procedures. A significance level p<0.05 was used. Results More than 50% of the children and adolescents did not know how to say the name of their disease or explain it. After adjusted OR (AOR), cyanotic patients in comparison to acyanotic ones (AOR: 2.29; 95%CI: 1.76-6.71; p = 0.019); children with lower level of schooling (AOR: 2.20; 95%CI: 1.81-5.86; p = 0.025); and those who did not practice physical activity (AOR: 1.88; 95%CI: 1.09-3.45; p = 0.011) showed potential for incorrect answers or did not know their disease. Conclusion Cyanotic children and adolescents, with a lower level of schooling and who did not practice physical activity, had little knowledge about their disease. It is necessary to develop educational intervention strategies to increase knowledge and change behavior in physical activity promotion, according to the CHD complexity. (Arq Bras Cardiol. 2020; 114(5):786-792)

Síndrome del corazón izquierdo hipoplásico / Hypoplastic Left Heart Syndrome

Introducción: El síndrome del corazón izquierdo hipoplásico es una rara anormalidad que se presenta en el periodo prenatal. Sus manifestaciones clínicas se relacionan con la presencia de hipoxemia a nivel sistémico. Objetivo: Socializar las manifestaciones clínicas y elementos diagnósticos del síndrome del corazón izquierdo hipoplásico. Caso clínico: Recién nacido masculino, a término, que presenta manifestaciones clínicas y ecosonográficas que permiten llegar al diagnóstico de un síndrome del corazón izquierdo hipoplásico. Conclusiones: A pesar de los avances de la ciencia y la técnica, el síndrome del corazón izquierdo hipoplásico presenta una elevada mortalidad. Una adecuada planificación de la gestación, el seguimiento preciso del embarazo, un correcto asesoramiento genético y la utilización de técnicas quirúrgicas para corregir las deformidades resultantes parecen ser las herramientas más eficaces para enfrentar esta mortal afectación(AU)

Introduction: Hypoplastic left heart syndrome is a rare abnormality that occurs in the prenatal period. Its clinical manifestations are related to the presence of hypoxemia at the systemic level. Objective: To describe the clinical manifestations and diagnostic elements of hypoplastic left heart syndrome. Clinical case: A male newborn, at term, who presented clinical and echographic manifestations that permitted the diagnosis of hypoplastic left heart syndrome. Conclusions: Despite advances in science and technology, hypoplastic left heart syndrome presents a high mortality. Proper planning for pregnancy, accurate pregnancy monitoring, proper genetic counseling, and the use of surgical techniques to correct the resulting deformities seem to be the most effective tools to deal with this deadly condition(AU)

Cardiopatías congénitas y desarrollo del cerebro fetal artículo de revisión / Congenital heart disease and fetal brain development: review

La enfermedad cardíaca congénita se ha asociado con alteraciones del neurodesarrollo. Niños y adolescentes con cardiopatía congénita presentan déficits en distintos dominios neurológicos (motor, cognitivo, lenguaje, rendimiento escolar y habilidades sociales). Históricamente se atribuyeron a una potencial injuria cerebral durante la cirugía cardíaca. Sin embargo, evidencia actual demuestra que RN de término ya presentan anormalidades cerebrales y retraso de la maduración (dismaduración) con alta prevalencia 28% (IC 95%, 18-40%) Entre ellas se reporta ventriculomegalia, agenesia cuerpo calloso, hipoplasia de vermis, hemorragia intraventricular, aumento espacio subaracnoídeo, alteraciones de la sustancia blanca (Leucomalacia periventricular), desarrollo cortical alterado y quistes periventriculares. El mecanismo más estudiado es la interferencia de la cardiopatía con el shunt fisiológico de derecha a izquierda por el foramen ovale, que asegura la llegada de la sangre mejor oxigenada primeramente al cerebro. Se discute el beneficio de potenciales intervenciones prenatales neuroprotectoras y el diagnóstico temprano de cardiopatías congénitas.

Congenital heart disease has been associated with neurodevelopmental disorders. Children and adolescents with congenital heart disease present deficits in different neurological domains (motor, cognitive, language, school performance and social skills). Historically they were attributed to a potential brain injury during cardiac surgery. However, current evidence shows that term newborns already present brain abnormalities and delay of maturation (dysmaturation) with high prevalence 28% (95% CI, 18-40%) Among them is reported ventriculomegaly, Corpus Callosum agenesis, hypoplastic vermis, intraventricular hemorrhage, subarachnoid space enlargement, white matter alterations (periventricular leukomalacia), altered cortical development and periventricular cysts. The most studied mechanism is the interference of heart disease with the physiological shunt from right to left by the foramen ovale, which ensures the arrival of the best oxygenated blood first to the brain. The benefit of potential neuroprotective prenatal interventions and early diagnosis of congenital heart diseases is discussed.

Consultorio de condiciones crónicas complejas, seguimiento de cardiopatías congénitas críticas / Complex chronic conditions clinic, follow-up of critical congenital heart defects

Introducción: Las cardiopatías congénitas representan la segunda causa de mortalidad infantil en menores de un año. Objetivo: Describir la epidemiología, resultados y seguimiento de cardiopatías congénitas críticas (pacientes que requirieron intervención quirúrgica o cateterismo intervencionista antes del año de vida). Resultados: Se incluyeron 316 pacientes operados o intervenidos por cateterismo antes del año de vida con diagnóstico de cardiopatías congénitas, de los cuales el 30,7% eran sindromáticos y solo el 7,7% tuvieron diagnóstico prenatal. Se logró cirugía reparadora en un 86,7% de los casos siendo la CIV la cardiopatía congénita operada más frecuente en menores de un año. Los pacientes quedaron con defectos residuales significativos en un 23,73%, con una tasa de complicaciones de 39,64%. En el seguimiento post-operatorio un 19,62% requirieron reoperación, y un 5,06% cateterismo. Un 15,19% de los pacientes fallecieron. Conclusiones: El Hospital Juan P. Garrahan tiene una población numerosa y heterogénea de pacientes con cardiopatías críticas. Dado que solo un pequeño porcentaje de nuestros pacientes tuvieron diagnóstico prenatal, se debe mejo- rar en este aspecto, ya que el diagnóstico de cardiopatía en el período fetal mejora las posibilidades de sobrevida en el neona- to. Es importante el seguimiento multidisciplinario, coordinado y continuo de estos pacientes, dado que muchos niños operados tienen comorbilidades y lesiones residuales (AU)

Introduction: Congenital heart defects are the second leading cause of infant mortality in children under one year of age. Objective: To describe the epidemiology, results, and follow-up of patients with critical congenital heart defects (patients who required surgical intervention or interventional catheterization before the year of life). Results: 316 patients with congenital heart defects who underwent surgery or catheterization before the life year, of whom 30.7% were syndromic and only 7.7% were diagnosed prenatally. Repair surgery could be performed in 86.7% of cases with SVD being the most commonly operated congenital heart defect in children under one year of age. Of the patients, 23.73% had a significant residual defect with a complication rate of 39.64%. In the post-operative follow-up, 19.62% required reoperation and 5.06% catheterization. Overall, 15.19% of the patients died. Conclusions: The Juan P. Garrahan Hospital has a large and heterogeneous population of patients with critical heart disease. Since only a small percentage of our patients had a prenatal diagnosis, this aspect should be enhanced, as the diagnosis of heart disease in uterus improves the chances of survival of the neonate. Multidisciplinary, coordinated and continuous follow-up of these patients is important, as many children who undergo surgery have comorbidities and residual lesions (AU)

Consultorio de orientaciòn de cardilogía: epidemiología y perfil de la población / Cardiology guidance clinic: Epidemiology and profile of the population

Introducción: Las cardiopatías congénitas (CC) son las anomalías congénitas más frecuentes. Representan el 0,8-1,2% de todos los defectos del nacimiento y tienen una prevalencia de alrededor de 5,8 por cada 1000 personas. El Servicio de Cardiología del Hospital Garrahan es un centro de referencia nacional y de países limítrofes donde se realizan 18000 consultas anuales. Los pacientes que concurren por primera vez se atienden en el consultorio de orientación. Objetivo: Describir la epidemiologia y perfil de los pacientes que asisten diariamente al consultorio de orientación de cardiología infantil en un hospital pediátrico de tercer nivel de Buenos Aires. Métodos: Entre septiembre de 2017 y febrero de 2018 se recolectaron los datos de 1000 pacientes atendidos en forma consecutiva en el consultorio de orientación de cardiología. A la totalidad de los pacientes se les realizó anamnesis, examen físico cardiovascular, electrocardiograma, y en los casos en los que se consideró necesario, saturometría, radiografía de tórax y/o ecocardiograma. Las variables a considerar fueron edad, procedencia, presencia o ausencia de cardiopatías congénitas o adquiridas, soplo, cianosis, insuficiencia cardíaca, estado nutricional, síndromes genéticos asociados, métodos diagnósticos e indicaciones terapéuticas implementadas. Se subdividió la población en cinco grupos: Grupo A (pacientes con cardiopatía congénita), Grupo B (cardiopatías operadas), Grupo C (miocardiopatías), Grupo D (arritmias), Grupo E (corazón sano). Resultados: La edad mediana fue 4.86 años (0.03 a 18.9 años). El 64% de los pacientes procedían de la provincia de Buenos Aires. Los motivos de consulta fueron: interconsultas internas 29.5%, derivación por cardiopatía 27.2%, soplo 17.6%, síncope 7%, segunda opinión 5.1%, arritmias 4.8%, precordialgia 3.1%, palpitaciones 2.6%, episodio paroxístico 1.4%, cardiomegalia 0.7%, disnea 0.5%, mal progreso de peso 0.3%. El 10.6% tenían un síndrome genético. Grupo A: 252 pacientes con una edad mediana de 1.9 años. Las cardiopatías acianóticas con hiperflujo pulmonar fueron las más frecuentes (66.66%, 168/252). Grupo B: 51 pacientes, 23.52%(12/51) fueron Fallot reparados en otra institución. Grupo C: 22 pacientes, siendo la miocardiopatía hipertrófica la más frecuente. Grupo D: 47 pacientes, la preexcitación ventricular fue el hallazgo más frecuente (34,04%, 16/47). Grupo E: 628 pacientes, 45.70% (287/628) derivados por pediatras del área ambulatoria, principalmente para valoración de pacientes con enfermedades sistémicas o síndromes genéticos. Conclusión: Los motivos de derivación al consultorio de orientación de cardiología fueron muy diversos. La mayoría de los pacientes provenían de provincia de Buenos Aires. Solamente el 37.2% presentó algún problema cardiológico de base. El 91% de los pacientes que consultaron por soplo, no tuvieron cardiopatía. El grupo correspondiente a los pacientes con cardiopatías no operadas (grupo A) fue el de menor edad (mediana de 1.9 años) y las cardiopatías simples no cianóticas con hiperflujo pulmonar representaron el 66.66% de las cardiopatías. La implementación del ecocardiograma portátil en el consultorio de orientación permitió confirmar el diagnóstico y definir la conducta terapéutica en el 29.4% de los pacientes durante la primer consulta (AU)

Introduction: Congenital heart defects (CHD) are the most common congenital abnormalities. They account for 0.8-1.2% of all birth defects and have a prevalence of around 5.8 per 1000 people. The Department of Cardiology of Garrahan Hospital is a national and bordering-country reference center, receiving 18000 consultations annually. Patients seen for the first time are assessed at the cardiology guidance clinic. Objective: To describe the epidemiology and profile of patients who seen daily at the child cardiology guidance clinic of a third-level pediatric hospital in Buenos Aires. Methods: Between September 2017 and February 2018, data from 1000 patients consecutively seen at the cardiology guidance clinic were collected. All patients underwent anamnesis, cardiovascular physical examination, electrocardiogram and, if considered necessary, pulse oximetry, chest x-ray, and/or echocardiogram. The variables considered were age, place of origin, presence or absence of congenital or acquired heart disease, murmur, cyanosis, heart failure, nutritional status, associated genetic syndromes, diagnostic methods, and treatment. The population was divided into five groups: Group A (patients with congenital heart defects), Group B (operated cardiopathies), Group C (myocardiopathies), Group D (arrhythmias), Group E (healthy heart). Results: Median age was 4.86 years (0.03 to 18.9 years). Overall, 64% of patients came from the province of Buenos Aires. The reasons for consultation were: internal consultations 29.5%, cardiac shunt 27.2%, murmur 17.6%, syncope 7%, second opinion 5.1%, arrhythmias 4.8%, precordialgia 3.1%, palpitations 2.6%, paroxysmal episode 1.4%, cardiomegaly 0.7%, dyspnea 0.5%, 0.3% poor weight gain. A genetic syndrome was identified in 10.6%. Group A: 252 patients with a median age of 1.9 years. Acyanotic congenital heart defect with pulmonary hyperflow was the most common (66.66%, 168/252). Group B: 51 patients, 23.52% (12/51) had tetralogy of Fallot repaired at another institution. Group C: 22 patients, in whom hypertrophic cardiomyopathy was the most common. Group D: 47 patients, in whom ventricular preexcitation was the most common finding (34.04%, 16/47). Group E: 628 patients, 45.70% (287/628) referred by pediatricians from the outpatient clinics, mainly for the assessment of systemic diseases or genetic syndromes. Conclusion: The reasons for referral to the cardiology guidance clinic were varied. Most of the patients came from the province of Buenos Aires. Only 37.2% had an underlying heart disease. Of the patients who consulted because of a murmur, 91% did not suffer from heart disease. The group of patients with congenital heart disease who had not undergone surgery (group A) was the youngest (median 1.9 years) and simple non-cyanotic heart disease with pulmonary hyperflow accounted for 66.66% of heart diseases. The implementation of the portable echocardiography in the guidance clinic confirmed the diagnosis and defined the management in 29.4% of patients during the first consultation (AU)

Morbilidad y mortalidad neonatal por cardiopatías congénitas / Neonatal javascript:void(0)morbidity and mortality by congenital cardiopathies

RESUMEN Introducción: Las cardiopatías congénitas inciden en 8 x 1 000 recién nacidos vivos en el mundo y en gran medida determinan su mortalidad. Objetivo: Precisar la morbilidad y mortalidad neonatal por cardiopatías congénitas. Métodos: Estudio descriptivo, longitudinal y prospectivo realizado en 97 recién nacidos con estas cardiopatías, diagnósticados en el Servicio de Neonatología del Hospital Dr. Agostinho Neto de Guantánamo durante los años 2015- 2017. Se consideró el análisis segmentario, probable mecanismos que la determinan y tipo de cardiopatía. Resultados: Se identificaron cardiopatías congénitas en el 12,9 por ciento de los recién nacidos; la letalidad fue de 1,0 por ciento. Lo más común fue que se identificaron modos y tipos de conexión auriculoventricular y ventriculoarterial fisiológicos. Las anomalías en la muerte celular (47,3 por ciento) fue el mecanismo genético y molecular más observado. La cardiopatía más frecuente fue la comunicación interventricular (63,9 por ciento). La mayoría de las cardiopatías se clasificaron como: aisladas (95,9 por ciento), de gravedad moderada (92,8 por ciento), acianóticas (93,8 por ciento), no se asociaran a síndromes o enfermedades genéticas (94,9 por ciento) pero sí a malformaciones extracardiacas (94,9 por ciento). Se manifestaron sobre todo por soplo cardiaco (85,6 por ciento). El diagnóstico de 70,1 por ciento de las cardiopatías se realizó en etapa prenatal. Conclusiones: Estas afecciones no constituyen un problema de salud en el servicio de este hospital, pero a pesar de que la mayoría de las cardiopatías congénitas se diagnostican en etapa prenatal, se requiere continuar laborando en función de potenciar más su diagnóstico en esta etapa(AU)

ABSTRACT Introduction: Neonatal congenital cardiopathies have a global incidence of 8 x 1000 live births and it greatly determines their mortality. Objective: To specify the neonatal morbidity and mortality by congenital cardiopathies. Method: Descriptive, longitudinal and prospective study of 97 newborns with these cardiopathies diagnosed in the Neonatolgy Service in ´Dr. Agostinho Neto Hospital, Guantánamo province from 2015 to 2017. It was considered: the segmental analysis, probable mechanisms that determine it and cardiopathy type. Results: Congenital cardiopathies were identified in 12.9 percent of the newborns; the lethality was of 1.0 percent. The most common aspect was the identification of ways and types of aurículo-ventricular and physiologic ventrículo-arterial connection. Anomalies in the cellular death (47.3 percent) were the most frequent genetic and molecular mechanism. The most frequent congenital heart disease was the interventricular communication (63.9 percent). Most of the cardiopathies were classified as: isolated (95.9 percent); of moderate graveness (92.8 percent); acyanotic (93.8 percent); not associated to syndromes or genetic diseases (94.9 percent) but yes to extracardiac malformations (94.9 percent); and they manifested mainly by heart murmur (85.6 percent ). The diagnosis of 70.1 percent of the cardiopathies was carried out in prenatal stage. Conclusions: Congenital cardiopathies don't constitute a health problem in the Neonatology service of the Hospital; but instead that these were mainly diagnosed in the prenatal stage, it is required to continue working in function of boosting their prenatal diagnosis(AU)

Functional capacity in children and adolescents with congenital heart disease / Capacidade funcional em crianças e adolescentes com cardiopatia congênita

ABSTRACT Objective: To evaluate the physical activity level and functional capacity of children and adolescents with congenital heart disease and to describe correlations between functionality, surgical and echocardiographic findings, metabolic and inflammatory profile and differences between acyanotic and cyanotic heart defects. Methods: A cross-sectional study including children and adolescents with congenital heart disease between six and 18 years old that were evaluated with the 6-minute walk test (6MWT) to assess functional capacity. The short version form of the International Physical Activity Questionnaire (IPAQ) was performed to evaluate physical activity levels. Also, echocardiography and blood collection, to evaluate the metabolic (blood glucose, lipids, insulin) and inflammatory markers (C-reactive protein), were assessed. Results: Twenty-five individuals were evaluated. Of them, 14 had acyanotic heart defects and 11 cyanotic heart defects. Mean age was 12.0±3.7 years, and 20 (80%) were male. IPAQ showed that six (24%) individuals were very active, eight (32%) were active, nine (36%) had irregular physical activity, and two (8%) were sedentary. The mean distance walked in the 6MWT, considering all studied individuals, was 464.7±100.4 m, which was 181.4±42.0 m less than the predicted (p=0.005). There was a positive correlation between Z score 6MWT and the number of surgical procedures (r=-0.455; p=0.022). Conclusions: Children and adolescents with congenital heart disease have low functional capacity, but they are not completely sedentary.

RESUMO Objetivo: Avaliar o nível de atividade física e a capacidade funcional de crianças e adolescentes com cardiopatia congênita, além de descrever correlações entre funcionalidade, achados cirúrgicos e ecocardiográficos, perfil metabólico e inflamatório e diferenças entre cardiopatias congênitas acianótica e cianótica. Métodos: Estudo transversal com crianças e adolescentes com cardiopatia congênita entre seis e 18 anos de idade. Foi realizado o teste de caminhada de 6 minutos para avaliar a capacidade funcional, e aplicou-se a versão curta do Questionário Internacional de Atividade Física (IPAQ) para avaliar os níveis de atividade física. Foram feitos também: exame ecocardiográfico, coleta de sangue para avaliação de perfil metabólico e inflamatório (glicemia, triglicerídeos, colesterol total, lipoproteína de alta densidade - HDL-colesterol, lipoproteína de baixa densidade - LDL-colesterol, hemograma completo, proteína C reativa, insulina). Resultados: Foram avaliados 25 indivíduos, dos quais 14 tinham cardiopatia congênita acianótica e 11 cianótica. A média de idade foi de 12,0±3,7 anos, e 20 (80%) eram do sexo masculino. O IPAQ mostrou que seis (24%) indivíduos eram muito ativos, oito (32%) eram ativos, nove (36%) tinham atividade física irregular e dois (8%) eram sedentários. A média de distância percorrida no teste de caminhada dos 6 minutos, considerando todos os indivíduos estudados, foi de 464,7±100,4 m, sendo 181,4±42,0 m menor do que o previsto (p=0,005). Encontrou-se correlação entre o escore Z do teste de caminhada de 6 minutos e o número de procedimentos cirúrgicos realizados (r=-0,455; p=0,022). Conclusões: Crianças e adolescentes com cardiopatia congênita têm baixa capacidade funcional, mas não são completamente sedentários.

Ameaça à vida ao nascer: uma análise das causas de morte e estimativa de sobrevida de menores de cinco anos em coortes de nascidos vivos / Amenaza para la vida al nacer: un análisis de las causas de muerte y estimación de supervivencia de menores de cinco años en cohortes de nacidos vivos / Life-threatening conditions at birth: an analysis of causes of death and survival estimate for under-five children in live birth cohorts

Apesar da redução da mortalidade na infância, as causas ainda são majoritariamente evitáveis, e a sobrevida pode estar condicionada à situação de ameaça à vida ao nascer. Foram estimadas a carga de ameaça à vida ao nascer, de near miss neonatal, e a mortalidade, com ênfase na evitabilidade, e sobrevida na infância, em coortes de nascidos vivos. Estudo de coorte retrospectiva de nascidos vivos residentes no Município do Rio de Janeiro (2012-2016). Os bancos de dados dos Sistemas de Informações sobre Nascidos Vivos e sobre Mortalidade foram relacionados. Critérios pragmáticos foram utilizados para definir ameaça à vida e near miss. Óbitos foram classificados segundo a lista brasileira de causas de mortes evitáveis. Foram estimados indicadores de morbimortalidade e a sobrevida (Kaplan-Meier). Dos 425.505 nascidos vivos, 2,2% apresentaram ameaça à vida ao nascer. As taxas de mortalidade na infância, infantil e neonatal foram, respectivamente: 0,01; 0,06 e 14,97 por mil pessoas-dia. Causas evitáveis, não claramente evitáveis e mal definidas corresponderam, respectivamente, a 61%, 35% e 4% dos óbitos. O risco de morte por causas evitáveis atribuível ao nascimento com ameaça à vida foi de 97,6%. A sobrevida foi menor entre recém-nascidos com ameaça à vida, comparados àqueles sem ameaça à vida. Os critérios pragmáticos de ameaça à vida determinaram o perfil de mortalidade proporcional por causas de morte segundo os três grupos de causas da lista brasileira de causas de mortes evitáveis. Nascer com ameaça à vida define crianças com maior risco de morbimortalidade e põe, em pauta, a discussão sobre a vulnerabilidade e as necessidades de assistência às crianças e do apoio social às suas famílias.

A pesar de la reducción de la mortalidad en la infancia, las causas de fallecimiento todavía son mayoritariamente evitables y la supervivencia puede estar condicionada con la situación de amenaza para la vida al nacer. En este trabajo se estimaron la carga de amenaza para la vida al nacer, el near miss neonatal y la mortalidad, con énfasis en la evitabilidad y supervivencia en la infancia en cohortes de nacidos vivos. Es un estudio de cohorte retrospectivo de nacidos vivos, residentes en el municipio de Río de Janeiro, Brasil (2012-2016). Se relacionaron los bancos de datos de los Sistemas de Información sobre Nacidos Vivos y sobre Mortalidad. Se utilizaron criterios pragmáticos para la definición amenaza para la vida al nacer y near miss. Los óbitos se clasificaron según la lista brasileña de causas de muertes evitables. Se estimaron indicadores de morbimortalidad y supervivencia (Kaplan-Meier). De los 425.505 nacidos vivos, un 2,2% presentaron amenaza para la vida. Las tasas de mortalidad en la infancia, infantil y neonatal fueron, respectivamente: 0,01; 0,06 y 14,97 por 1.000 personas-día. Las causas evitables, no claramente evitables y mal definidas, correspondieron, respectivamente, a un 61%, 35% y 4% de los óbitos. El riesgo de muerte por causas evitables atribuible al nacimiento con amenaza para la vida fue de un 97,6%. La supervivencia fue menor entre recién nacidos con amenaza para la vida, comparados con aquellos sin amenaza para la vida. Los criterios pragmáticos de amenaza para la vida determinaron el perfil de mortalidad proporcional por causas de muerte, según los tres grupos de causas de la lista brasileña de causas de muertes evitables. Nacer con amenaza para la vida define a los niños con un mayor riesgo de morbimortalidad y pone en relevancia la discusión sobre vulnerabilidad y necesidades asistenciales para los niños, así como el apoyo social a sus familias.

Despite the reduction in under-five mortality, the causes are still mostly avoidable, and survival may be compromised by life-threatening conditions at birth. The study estimated the burden of life-threatening conditions at birth, neonatal near miss, and mortality, with an emphasis on avoidable causes, as well as under-five survival in live birth cohorts. This was a retrospective cohort study of live birth in the city of Rio de Janeiro, Brazil (2012-2016). The databases from the Brazilian Information System on Live Births and the Brazilian Mortality Information System were linked. Pragmatic criteria were used to define life-threatening conditions and near miss. Deaths were classified according to the Brazilian list of causes of avoidable deaths. Morbidity and mortality and survival indicators were estimated (Kaplan-Meier). Of the 425,505 live birth , 2.2% presented life-threatening conditions at birth. The under-five, infant and neonatal mortality rates were 0.01, 0.06, and 14.97 per 1,000 person-days, respectively. Avoidable, unclearly avoidable, and ill-defined causes accounted respectively for 61%, 35%, and 4% of the deaths. The risk of death from avoidable causes attributable to life-threatening conditions at birth was 97.6%. Survival was lower in newborns with life-threatening conditions compared to those without life-threatening conditions. The pragmatic criteria for life-threatening conditions determined the profile of proportional mortality by causes of death according to the three groups of causes in the Brazilian list of causes of avoidable deaths. Life-threatening conditions at birth increases the risk of morbidity and mortality in under-five children and raises the discussion on vulnerability and the need for care for these children and social support for their families.