Resumo Fudamento Em 2015, foram diagnosticados 2.368 portadores de malformação congênita (MC) por 100.000 nascidos vivos (NV) no mundo, uma taxa de 7,6%, dos quais 10,6% morreram no primeiro ano de vida, sendo 43% por malformações do aparelho circulatório (MAC), à semelhança do que ocorre no Brasil. Objetivo Verificar a associação de diagnóstico de MAC ao nascimento e morte por MAC no primeiro ano de vida com índice de desenvolvimento humano (IDH) e recursos tecnológicos e humanos para o diagnóstico e tratamento da MAC por macrorregião do Brasil. Métodos Estudo ecológico de dados disponíveis de 2000 a 2015. Informações sobre NV, óbitos e ecocardiógrafos foram obtidas do DATASUS, o IDH, do Atlas de Desenvolvimento Humano no Brasil, e as demais foram obtidas da demografia médica. Foram realizadas medidas de correlação entre as variáveis utilizando o índice de Kendall. Resultados A taxa de MC foi 660,8/100.000 NV, das quais, 18.444 por MAC (taxa de diagnóstico 38,55/100.000 NV). As regiões Sul e Sudeste, com maiores valores de IDH e recursos, apresentaram as maiores taxas de diagnóstico de MAC (56,94/100.000 e 62,83/100.000 NV, respectivamente). As regiões Norte e Nordeste, com os menores valores de IDH e recursos, apresentaram as menores taxas de diagnóstico de MAC (9,77/100.000 e 13,43/100.000 NV, respectivamente). Essa taxa de diagnóstico foi 6,4 vezes maior no Sudeste do que no Norte, mas as taxas de mortalidade foram similares. Conclusão Das MC, as MAC apresentaram a maior mortalidade nos menores de 1 ano no Brasil.

Abstract Background In 2015, the number of infants with congenital malformations (CMs) per 100 000 live births (LBs) was 2368 (7.6%) worldwide, of whom 10.6% died in the first year of life, 43% due to malformations of the circulatory system (MCSs), a scenario similar to what occurs in Brazil. Objective To assess, per Brazilian macroregion, whether diagnosis of MCS at birth and death due to MCS in the first year of life associate with human development index (HDI) and with technological and human resources. Methods Ecological study including data available in 2000–2015. Data of LBs, deaths and availability of echocardiography devices were obtained from the DATASUS website. The HDI was obtained from the Atlas of Human Development in Brazil, while other variables were obtained from medical demographic data. Correlation measures between the variables were performed using the Kendall index. Results The CM rate was 660.8/100 000 LBs, of which 18 444 were due to MCS (diagnosis rate, 38.55/100 000 LBs). Of all Brazilian macroregions, the Southern and Southeastern regions, with the highest HDI values and resources, had the highest MCS diagnosis rates (56.94/100 000 and 62.83/100 000 LBs, respectively). The Northern and Northeastern regions, with the lowest HDI values and resources, had the lowest MCS diagnosis rates (9.77/100 000 and 13.43/100 000 LBs, respectively). The MCS diagnosis rate was 6.4-fold higher in the Southeastern region as compared to the Northern region, but mortality rates were similar in both regions. Conclusion Of the CMs, the MCS accounted for the highest number of deaths in children under the age of 1 year in Brazil.

Resumo Fundamentos Os óbitos por malformações do aparelho circulatório (MAC) em 2015 corresponderam a 43% daqueles por malformações congênitas (MC) em menores de 20 anos de idade no mundo. Os óbitos por MAC apresentam maior impacto sobre a redução da mortalidade, pelo fato de serem evitáveis na maioria das vezes, com o correto diagnóstico e tratamento. Objetivo Conhecer a distribuição da mortalidade por MAC por sexo, grupos etários e macrorregiões do Brasil no período de 2000 a 2015, nos menores de 20 anos de idade. Métodos Estudo descritivo das taxas de mortalidade por 100 mil e sua mortalidade proporcional, por MAC, outras malformações congênitas (OutMC), doenças do aparelho circulatório (DAC), causas mal definidas (CMD) e causas externas (CE) no Brasil, no período de 2000 a 2015 nos menores de 20 anos. As populações foram obtidas no Instituto Brasileiro de Geografia e Estatística e os óbitos no Departamento de Informática do Sistema Único de Saúde/Ministério da Saúde. Resultados Ocorreram 1.367.355 óbitos por todas as causas nos menores de 20 anos de idade, sendo 61,7% do sexo masculino e 55,0% dos óbitos nos menores de 1 ano. Os óbitos por MC em quaisquer órgãos ou sistemas foram 144.057 e os por MAC corresponderam a 39% desses óbitos. Em ambos os sexos, a mortalidade anual por MAC foi de 5,3/100 mil habitantes e a mortalidade proporcional (MP) foi de 4,2%, por DAC 2,2%, por CMD 6,2% e por CE 24,9%. As MAC não especificadas apresentaram as maiores taxas de MP em todas as idades e sexos, notadamente nas regiões Norte e Nordeste (60%). Os óbitos por quaisquer MC ocorreram 5,7 vezes mais no primeiro ano de vida do que nas outras faixas etárias (MAC: 5,0; OutMC: 6,4). Conclusão No Brasil, de 2000 a 2015, nos menores de 20 anos de idade, a MAC foi a principal causa de óbito dentre todas as malformações, sendo duas vezes mais importante do que as DAC, principalmente nos menores de 1 ano de idade.A frequência de diagnósticos imprecisos de óbitos por MAC ainda é elevada em todas as idades, sexos, e principalmente nas regiões Norte e Nordeste, o que requer fortalecimento das estratégias de saúde pública e maior atenção ao recém-nascido com objetivo de diagnosticar e instituir tratamento precoce das cardiopatias congênitas com consequente redução na mortalidade. (Arq Bras Cardiol. 2020; 115(6):1164-1173)

Abstract Background Deaths from malformations of the circulatory system (MCS) have a major impact on mortality reduction. given that most cases are avoidable with correct diagnosis and treatment. Objectives To describe the distribution of mortality from MCS by sex. age. and macroregion in Brazil. in individuals under the age of 20. between 2000 and 2015. Methods A descriptive study of mortality rates and proportional mortality (PM) from MCS. other congenital malformations (OCM). circulatory system disease (CSD). ill-defined causes (IDC). and external causes (EC) in Brazil. Results There were 1.367.355 deaths from all causes in individuals younger than 20. 55.0% under 1 year of age. A total of 144.057 deaths were caused by congenital malformations. 39% of them by MCS. In both sexes. the annual mortality from MCS was 5.3/100.000. PM from MCS was 4.2%. CSD 2.2%. IDC 6.2% and EC 24.9%. Unspecified MCS showed the highest PM rates in both sexes and age groups. especially in the north and northeast regions (60%). Deaths from malformations occurred 5.7 times more frequently during the first year of life than in other ages (MCS: 5.0; OCM: 6.4). Conclusions MCS was the leading cause of death among all malformations. being twice as important as CSD. mainly under 1 year of age. The frequency of misdiagnosis of MCS as cause of death was high in all ages and both sexes. especially in the north and northeast regions. These findings highlight the need for the development of public health strategies focused on correct diagnosis and early treatment of congenital cardiopathies. leading to a reduction in mortality. (Arq Bras Cardiol. 2020; 115(6):1164-1173)

ABSTRACT OBJECTIVE To analyze the association of characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level, with death from diseases or malformations of the circulatory system in children under 18 years of age. METHODS The Brazilian Information System on Live Births and Information System on Mortality databases were linked and evaluated following a longitudinal cohort analysis strategy. The following independent variables were evaluated: characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level. Dependent variables were death from diseases or malformations of the circulatory system in children under 18 years of age. Crude relative risks were estimated and relative risks were adjusted for the variables. RESULTS 6,380 deaths were linked to 4,282,260 birth records, yielding 5,062 pairs considered as true. Low birth weight (RR = 2.26), asphyxia at 1 (RR = 1.72) and 5 minutes (RR = 1.51), prematurity (RR = 1.50), maternal age ≥ 40 years (RR = 2.06), and low maternal education level (RR = 1.45) increased the probability of death caused by circulatory system diseases. In the association with death by malformations of the circulatory system, the predictive variables showed the same association profile, but with greater intensity. CONCLUSIONS Fetal and maternal factors are associated with increased mortality due to diseases and malformations of the circulatory system. Measures to control these factors and improve access to their diagnosis and treatment would contribute to reducing the number of deaths caused by diseases and malformations of the circulatory system. However, the identification of environmental influences during gestation and birth on the risk of death should be carefully considered due to being influenced by genetic factors.

ABSTRACT OBJECTIVE To analyze the association of characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level, with death from diseases or malformations of the circulatory system in children under 18 years of age. METHODS The Brazilian Information System on Live Births and Information System on Mortality databases were linked and evaluated following a longitudinal cohort analysis strategy. The following independent variables were evaluated: characteristics recorded at the time of birth, including weight, occurrence of asphyxia, gestation duration, maternal age and education level. Dependent variables were death from diseases or malformations of the circulatory system in children under 18 years of age. Crude relative risks were estimated and relative risks were adjusted for the variables. RESULTS 6,380 deaths were linked to 4,282,260 birth records, yielding 5,062 pairs considered as true. Low birth weight (RR = 2.26), asphyxia at 1 (RR = 1.72) and 5 minutes (RR = 1.51), prematurity (RR = 1.50), maternal age ≥ 40 years (RR = 2.06), and low maternal education level (RR = 1.45) increased the probability of death caused by circulatory system diseases. In the association with death by malformations of the circulatory system, the predictive variables showed the same association profile, but with greater intensity. CONCLUSIONS Fetal and maternal factors are associated with increased mortality due to diseases and malformations of the circulatory system. Measures to control these factors and improve access to their diagnosis and treatment would contribute to reducing the number of deaths caused by diseases and malformations of the circulatory system. However, the identification of environmental influences during gestation and birth on the risk of death should be carefully considered due to being influenced by genetic factors.

Resumo Fundamento: Conhecer o perfil epidemiológico de mortalidade de uma população é importante para instituir medidas de melhoria da assistência em saúde e redução da mortalidade. Objetivo: Estimar as taxas de mortalidade e a mortalidade proporcional por doenças e malformações do aparelho circulatório em crianças e adolescentes. Métodos: Estudo descritivo das mortalidades por doenças e malformações do aparelho circulatório, todas as causas, causas mal definidas e causas externas em crianças e adolescentes no Estado do Rio de Janeiro, de 1996 a 2012. As populações foram obtidas no Instituto Brasileiro de Geografia e Estatística (IBGE), e os óbitos no Departamento de Informática do Sistema Único de Saúde (DATASUS)/Ministério da Saúde. Resultados: Ocorreram 115.728 óbitos por todas as causas, sendo 69.757 no sexo masculino. A mortalidade anual por doenças do aparelho circulatório foi 2,7/100mil no sexo masculino e 2,6/100 mil no feminino. A mortalidade anual por malformações do aparelho circulatório foi 7,5/100 mil no sexo masculino e 6,6/100 mil no feminino. Dentre as causas específicas de doenças do aparelho circulatório, as cardiomiopatias apresentaram as maiores taxas de mortalidade proporcional anual e, por malformações do aparelho circulatório, ocorreu por malformações não especificadas do aparelho circulatório, em todas as idades e sexos. Conclusão: A mortalidade por malformações do aparelho circulatório foi mais marcante nos primeiros anos de vida, enquanto as doenças do aparelho circulatório foram mais relevantes nos adolescentes. O baixo acesso ao diagnóstico pré-natal e/ou ao nascimento provavelmente impossibilitou o adequado tratamento das doenças e malformações do aparelho circulatório.

Abstract Background: The epidemiological profile of mortality in a population is important for the institution of measures to improve health care and reduce mortality Objective: To estimate mortality rates and the proportional mortality from cardiovascular diseases and malformations of the circulatory system in children and adolescents. Methods: This is a descriptive study of mortality from cardiovascular diseases, malformations of the circulatory system, from all causes, ill-defined causes and external causes in children and adolescents in the state of Rio de Janeiro from 1996 to 2012. Populations were obtained from the Brazilian Institute of Geography and Statistics (Instituto Brasileiro de Geografia e Estatística - IBGE) and deaths obtained from the Department of Informatics of the Unified Health System (DATASUS)/Ministry of Health. Results: There were 115,728 deaths from all causes, 69,757 in males. The annual mortality from cardiovascular diseases was 2.7/100,000 in men and 2.6/100,000 in women. The annual mortality from malformations of the circulatory system was 7.5/100,000 in men and 6.6/100,000 in women. Among the specific causes of circulatory diseases, cardiomyopathies had the highest rates of annual proportional mortality, and from malformations of the circulatory system, it occurred due to unspecified malformations of the circulatory system, at all ages and in both genders. Conclusion: Mortality from malformations of the circulatory system was most striking in the first years of life, while cardiovascular diseases were more relevant in adolescents. Low access to prenatal diagnosis or at birth probably prevented the proper treatment of malformations of the circulatory system.