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; [online].ahead print.

Background Congenital malformations (CMs) were the second cause of death in infants under the age of 1 year in North America and Europe in 2017, behind perinatal causes.Of the CMs, those of the circulatory system (MCSs) were the most lethal. Studies in Brazil, North America and England have reported higher mortality from MCS in the presence of prematurity, non-white skin color of the newborn, asphyxia, low birthweigth, advanced maternal age, and low maternal education level. The magnitudes of those associations vary. Those studies have investigated neither the risk of death from MCS, with control of the mediation provoked by those and other variables, nor the construction of a model to explain the relationship between variables and deaths. Objective To assess the associations of maternal geographic and socioeconomic factors, as well as gestational and birth factors, with deaths from MCSs in the first year of life in Brazil from 2006 to 2016, and to build an explanatory model of the relationship between variables and death. Methods Study of the Brazilian databases Information System on Live Births and Mortality Information System, which were linked by deterministic relationship and submitted to the cohort strategy. Crude and controlled relative risks were estimated. Acyclic graphs directed the creation of explanatory models of the relationship between maternal and fetal variables and the risks of death from MCSs and all causes. Results The deterministic linkage of 32,011,727 live birth certificates and 266,215 deaths yielded 248,027 linked pairs. Low birth weight (RR=14.38), asphyxia in the first (RR=5.95) and fifth minute (RR=11.86), maternal age ≥35 years (RR=1.66), male sex (RR=1.13), residing in the West-Central Brazil (RR=1.22) and small number of prenatal visits (RR=2.24) increased the risk of death from MCSs. Prematurity and multiple-gestation pregnancy had effects on death mediated by low birth weight and asphyxia regardless of the cause of death. Maternal education level and skin color did not increase the risk of death from MCS but influenced deaths from other causes. Conclusion The explanatory model of the relation of maternal geographic and socioeconomic, gestational and birth factors with deaths in the first year of life in Brazil from 2006 to 2016 showed that the small number of prenatal visits, male sex, low birthweigth, and presence of asphyxia in the first and fifth minutes are variables with a direct effect on the risk of death regardless of the cause of death. Deaths from MCSs in the West-Central regions and maternal age ≥35 years pose a higher risk of death, but this effect was not evidenced for other causes of death. Better gestational care with an adequate number of prenatal visits would enable the early diagnosis of MCSs with proper life support and treatment. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): Federal University of Rio de Janeiro

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.