Diagnóstico prenatal de enfermedad de canal atrio-ventricular, análisis epidemiológico y experiencia CERPO entre 2003 y 2021 / Prenatal diagnosis of atrio-ventricular canal disease, epidemiological analysis and CERPO experience between 2003 and 2021

Antecedentes: En Chile, la mitad de los casos de mortalidad perinatal son atribuibles a anomalías congénitas, y un tercio de estas corresponde a cardiopatías congénitas. Aproximadamente un 35% de estos últimos requerirán cirugía antes del año de vida, por lo que la pesquisa prenatal impacta profundamente en el pronóstico. Objetivo: Dar a conocer los resultados perinatales de pacientes con diagnóstico prenatal de canal atrio-ventricular controlados en el Centro de Referencia Perinatal Oriente (CERPO) entre los años 2003 y 2021, su asociación a otras anomalías, características demográficas y pronóstico a un año. Métodos: En este estudio se puede apreciar que tanto el pronóstico como el plan terapéutico posnatal dependerán de la presencia de otras alteraciones morfológicas y del estudio genético. De los factores estudiados, se puede concluir que tanto la presencia de anomalías cardiacas asociadas, como el grado de insuficiencia valvular y el tipo de canal no son predictores de la sobrevida perinatal. Conclusiones: Finalmente, en relación con la sobrevida posnatal, en este estudio, la sobrevida a un año de los recién nacidos vivos fue de un 52%, pero al desglosarlo en los niños con cariotipo euploide y trisomía 21, estos valores se tornan muy distintos, 44 y 81% respectivamente.

Background: In Chile, half of the perinatal mortality cases are attributable to congenital anomalies, and one third of these correspond to congenital heart disease. Approximately 35% of the later will require surgery before one year of life, so prenatal screening has a profound impact on the prognosis. Objective: To present the perinatal results of patients with a prenatal diagnosis of atrio-ventricular canal controlled at Centro de Referencia Perinatal Oriente CERPO) between 2003 and 2021, its association with other anomalies, demographic characteristics, and 1-year prognosis. Methods: In this study it can be seen that both the prognosis and the postnatal therapeutic plan will depend on the presence of other morphological alterations and the genetic study. From the factors studied, it can be concluded that the presence of associated cardiac anomalies, the degree of valvular insufficiency, and the type of canal are not predictors of perinatal survival. Conclusions: Finally, in relation to postnatal survival, in this study, the 1-year survival of live newborns was 52%, but when broken down into children with euploid karyotype and trisomy 21, these values become very different, 44 and 81% respectively.

Sobrevida de los recién nacidos con cardiopatías congénitas diagnosticadas prenatalmente / Survival of newborns with prenatally diagnosed congenital heart disease

Objetivo: Estimar la sobrevida al año de los recién nacidos con cardiopatías congénitas diagnosticadas prenatalmente y el perfil epidemiológico de sus madres. Método: Cohorte dinámica retrospectiva de 825 pacientes, ingresados entre el 1 de abril de 2003 y el 31 de marzo de 2019, con tiempo de seguimiento de 1 año, que se elaboró utilizando la base de datos del Centro de Referencia Perinatal Oriente (CERPO), Facultad de Medicina, Universidad de Chile. Resultados: Se estimó la función de supervivencia global de la muestra, obteniendo una supervivencia del 70% al año de seguimiento (error estándar (ES): 0,0164; intervalo de confianza del 95% [IC95%]: 0,66-0,73). Los recién nacidos con edad gestacional < 30 semanas tuvieron una menor sobrevida (hazard ratio [HR]: 4,17; IC95%: 1,52-11.44; p < 0,01). Los recién nacidos con un peso < 3000 g tuvieron una menor sobrevida (HR: 1,41; IC95%: 1,09-1,84; p < 0,01). La distribución de las cardiopatías congénitas según la gravedad en esta cohorte fue: riesgo vital 64%, clínicamente relevante 34% y clínicamente no relevante 2%. La menor sobrevida fue para la categoría riesgo vital (HR: 6,005; IC95%: 3,97-9,08; p < 0,01). Conclusiones: La prematuridad, el bajo peso al nacer y la gravedad de la cardiopatía se correlacionaron con una menor sobrevida.

Objective: To estimate the survival at one year of newborns with prenatally diagnosed congenital heart diseases and the epidemiological profile of their mothers. Method: Dynamic retrospective cohort of 825 patients, admitted between April 1, 2003 and March 31, 2019, with a follow-up time of 1 year, which was elaborated using the database of the Centro de Referencia Perinatal Oriente (CERPO), Faculty of Medicine, Universidad de Chile. Results: The overall survival function of the sample was estimated, resulting in a survival of 70% at one year follow-up (standard error (SE): 0.0164; 95% confidence interval [95% CI]: 0.66-0.73). Newborns with gestational age < 30 weeks had a lower survival (hazard ratio [HR]: 4.17; 95% CI: 1.52-11.44; p < 0.01). Newborns with a birth weight < 3000 g had a lower survival (HR: 1.41; 95% CI: 1.09-1.84; p < 0.01). The distribution of congenital heart disease according to severity in this cohort was: life-threatening 64%, clinically relevant 34% and clinically not relevant 2%. With a lower survival for the life-threatening category (HR: 6.005; 95% CI: 3.97-9.08; p < 0.01). Conclusions: Prematurity, low birth weight and severity of congenital heart correlated with a lower survival rate.

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.

Letalidad en lactantes con cardiopatías congénitas hospitalizados por virus respiratorio sincicial / Case fatality in infants with congenital heart disease hospitalized for respiratory syncytial virus

INTRODUCCIÓN. Las cardiopatías congénitas son un factor de riesgo para desarrollar enfermedad severa por virus respiratorio sincicial (VRS). En Chile no se conoce la hospitalización o letalidad por esta causa. El objetivo de este estudio fue determinar la letalidad de niños menores de 2 años con cardiopatías congénitas hospitalizados por infección por VRS en el hospital de niños Roberto del Río, Santiago, Chile. MÉTODO. Estudio descriptivo retrospectivo de revisión de fichas clínicas de niños menores de 24 meses con cardiopatía congénita, hospitalizados por infección respiratoria baja por VRS. Se registró edad, género, tipo de cardiopatía congénita, comorbilidades, días de hospitalización, ingreso a unidad de paciente crítico y letalidad en Hospital Roberto del Río durante los años 2014 a 2016. Se analizaron resultados mediante Stata 13. Aprobado por Comité de ética del Servicio de Salud Metropolitano Norte RESULTADOS: Se estudian 94 pacientes hospitalizados, mediana de edad de 7,3 meses, 45 (48%) hombres. Los casos de cardiopatía congénita cianótica fueron 7 (7,4%), obstructivas izquierdas 3 (3,2%) y no obstructivas 84 (89,3%). Las comorbilidades fueron: 17 (18,08%) trisomía 21, 10 (10,6%) patología neurológica, 6 (6,38%) reflujo gastroesofágico. Sin comorbilidad 48 pacientes (51,06%). La mediana de hospitalización es 8 días y la necesidad de ventilación mecánica de 1 día, siendo mayor en las cianóticas. Requieren ingreso a unidad de paciente crítico 49 (52,12%). La letalidad fue de un 8,5%. CONCLUSIÓN: La morbimortalidad de la infección por VRS observada en pacientes con cardiópatas congénitas es elevada, por lo que la prevención con anticuerpos monoclonales podría tener impacto. En el 2019 Chile implementa el uso de anticuerpos monoclonales anti VRS en niños con cardiopatías congénitas por lo que será interesante medir el impacto de esta medida.

INTRODUCTION. Congenital heart disease is a risk factor for developing severe disease due to respiratory syncytial virus (RSV). In Chile the rate of hospitalization or lethality due to this cause is not known. The objective of this study was to determine lethality of infants with congenital heart disease hospitalized for RSV infection. METHOD. Retrospective descriptive study, through review of clinical records of infants under 24 months with congenital heart disease, hospitalized due to low RSV respiratory infection. We recorded age, gender, type of congenital heart disease, comorbidities, days of hospitalization, admission to a critical patient unit and lethality in Roberto del Río Hospital between 2014 to 2016. The research had approved bay the ethical Comitee Servicio de Salud Metropolitano Norte. Results were analyzed through Stata 13. RESULTS. 94 hospitalized patients were studied, median age of 7.3 months, 45 (48%) men. The cases of congenital cyanotic heart disease were 7 (7.4%), left obstructive 3 (3.2%) and non-obstructive 84 (89.3%). The comorbidities were: 17 (18.08%) trisomy 21, 10 (10.6%) neurological pathology, 6 (6.38%) gastroesophageal reflux and 48 patients (51.06%) did not present. The hospitalization median is 8 days and the need for 1-day mechanical ventilation is greater in cyanotic patients. They require admission to the critical patient unit 49 (52.12%). The lethality is 8.5%. CONCLUSION: The lethality of RSV infection observed in patients with congenital heart disease is high. In 2019 Chile implemented the use of RSV monoclonal antibodies congenital heart disease. New studies are needed to measure the impact of this new policy. Keywords: congenital heart disease, respiratory syncytial virus

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.

Mortality due to congenital heart disease in Pernambuco from 1996 to 2016

SUMMARY BACKGROUND To determine the magnitude and temporal trends of deaths due to congenital heart disease (CHD) in Pernambuco between 1996 and 2016. METHODS This was an ecological, time-series study, involving all cases of deaths from congenital cardiovascular malformations in the state of Pernambuco, from 1996 to 2016, using data from DATASUS, SINASC and SIM. RESULTS There were 3,584 deaths from congenital cardiovascular malformations amongst individuals aged 0 to 14 years, of which 81.94% were concentrated in children aged under one year. The infant mortality rate (IMR) presented a linear growth trend of 0.4645 per year (p <0.01). The cause-of-death code Q24 (other congenital malformations of the heart) was present in 72.54% of the death records and 48.17% of the deaths occurred in infants aged between 28 and 364 days of life. The highest occurrence of deaths was identified in children with low birth weight (500 and 1,499g), male, premature, children of mothers without schooling, in deliveries at home (p <0.05). CONCLUSIONS Congenital heart disease still represents a public health problem as a cause of death, particularly in the first year of life, with IMR in a linear growth trend. Deaths from CHD were more prevalent in male children, born prematurely, with low birth weight, born to mothers with low schooling and deliveries without medical care.

RESUMO OBJETIVO Determinar a magnitude e tendência temporal dos óbitos por doenças cardiovasculares congênitas (DCC) em Pernambuco entre 1996 e 2016. MÉTODOS Trata-se de um estudo ecológico, de série temporal, com todos os casos de óbitos por doenças cardiovasculares congênitas no estado de Pernambuco, entre 1996 e 2016, por meio dos dados do Datasus, Sinasc e SIM. RESULTADOS Ocorreram 3.584 óbitos por DCC entre indivíduos de 0 a 14 anos de idade, dos quais 81,94% foram concentrados em crianças menores de 1 ano de idade. A taxa de mortalidade infantil apresentou tendência linear de crescimento de 0,4645 por ano (p<0,01). A causa Q24 (outras malformações congênitas do coração) esteve presente em 72,54% dos registros de óbitos e 48,17% dos óbitos ocorreram na faixa etária de 28 a 364 dias de vida. Maior ocorrência de óbitos foi identificada entre os nascidos com peso entre 500 e 1.499 g, do sexo masculino, pré-termos, filhos de mães sem escolaridade e em partos domiciliares (p<0,05). CONCLUSÕES As cardiopatias congênitas ainda representam um problema de saúde pública como causa de mortalidade, especialmente no primeiro ano de vida. A taxa de mortalidade por DCC apresentou tendência linear de crescimento. As características gerais demonstraram significância estatística para óbitos entre crianças prematuras, com baixo peso, do sexo masculino, nascidas de mães sem escolaridade e em partos domiciliares.

Associação do estado nutricional e os desfechos clínicos em cirurgia cardíaca pediátrica / Relación del estado nutricional y los resultados clínicos en cirugía cardíaca pediátrica / Association of nutritional status and clinical outcomes in pediatric cardiac surgery

Resumo Objetivo Verificar a associação do fator pessoal biológico estado nutricional, das crianças submetidas à cirurgia cardíaca, com os seguintes comportamentos: mortalidade/alta hospitalar, tempo de internação na Unidade de Terapia Intensiva (UTI) e tempo de ventilação mecânica (VM). Métodos Estudo transversal, retrospectivo realizado com 786 prontuários de crianças menores de cinco anos, submetidas à cirurgia cardíaca. O modelo de Nola J. Pender foi usado para analisar os dados. Aplicou-se o teste Qui-Quadrado de Pearson para verificar associação entre o fator pessoal biológico e o comportamento mortalidade/alta hospitalar. O teste Kruskal-Wallis foi utilizado para verificar a diferença entre medianas do fator pessoal biológico e os comportamentos tempo de VM e de UTI. Resultados A associação entre os fatores pessoais biológicos (pobre estado nutricional, desnutrição aguda e desnutrição crônica) com o comportamento mortalidade foi de OR 2,18 (1,42 - 3,34), p=0,003, OR 0,75 (0,46 - 1,2), p=0,24 e OR 2,7 (1,77 - 4,12), p<0,0001, respectivamente. A mediana de tempo em dias de uso de VM e tempo em dias de UTI foi, respectivamente de 3 (p<0,0001) e 8 (p<0,0001) para o pobre estado nutricional, 2 (p=0,041) e 6,5 (p=0,006) para a desnutrição aguda, 3 (p<0,0001) e 8 (p<0,0001) para a desnutrição crônica. Conclusão Os fatores pessoais biológicos que tiveram associação significativa com o comportamento mortalidade foram o pobre estado nutricional e desnutrição aguda. Foi verificado que as crianças com déficits nutricionais analisados tiveram uma superior mediana de tempo de VM e tempo de UTI quando comparadas com as crianças sem déficits nutricionais.

Resumen Objetivo Verificar la relación del factor personal biológico estado nutricional de niños sometidos a cirugía cardíaca, con los siguientes comportamientos: mortalidad/alta hospitalaria, tiempo de internación en Unidad de Cuidados Intensivos (UCI) y tiempo de ventilación mecánica (VM). Métodos Estudio transversal, retrospectivo realizado con 786 historias clínicas de niños menores de cinco años sometidos a cirugía cardíaca. Para analizar los datos se utilizó el modelo de Nola J. Pender. Se aplicó la prueba χ2 de Pearson para verificar la relación entre el factor personal biológico y el comportamiento mortalidad/alta hospitalaria. La prueba de Kruskal-Wallis fue utilizada para verificar la diferencia entre medianas del factor personal biológico y los comportamientos tiempo de VM y de UCI. Resultados La relación entre los factores personales biológicos (mal estado nutricional, desnutrición aguda y desnutrición crónica) y el comportamiento mortalidad fue de OR 2,18 (1,42 - 3,34), p=0,003, OR 0,75 (0,46 - 1,2), p=0,24 y OR 2,7 (1,77 - 4,12), p<0,0001, respectivamente. La mediana del tiempo en días de uso de VM y tiempo en días de UCI fue de 3 (p<0,0001) y 8 (p<0,0001) respectivamente para el mal estado nutricional, 2 (p=0,041) y 6,5 (p=0,006) para la desnutrición aguda, 3 (p<0,0001) y 8 (p<0,0001) para la desnutrición crónica. Conclusión Los factores personales biológicos que tuvieron relación significativa con el comportamiento mortalidad fueron el mal estado nutricional y la desnutrición aguda. Se verificó que los niños con deficiencias nutricionales estudiados tuvieron una mediana mayor de tiempo de VM y tiempo de UCI en comparación con niños sin deficiencias nutricionales.

Abstract Objective To find the association of the biological personal factor 'nutritional status' of children undergoing cardiac surgery with the following behaviors: mortality/hospital discharge, length of stay in the Intensive Care Unit (ICU) and time in mechanical ventilation (MV). Methods Cross-sectional, retrospective study of 786 medical records of children under five years of age who underwent cardiac surgery. Nola J. Pender's model was used for data analysis. The Pearson's Chi-Square test was applied to find the association between the biological personal factor and the mortality/hospital discharge behavior. The Kruskal-Wallis test was used to assess the difference between medians of the biological personal factor and the behaviors of time in MV and ICU length of stay. Results The association between personal biological factors (poor nutritional status, acute malnutrition and chronic malnutrition) with mortality behavior was OR 2.18 (1.42 - 3.34), p=0.003, OR 0.75 (0.46 - 1.2), p=0.24 and OR 2.7 (1.77 - 4.12), p<0.0001, respectively. The median time in days of MV use and ICU length of stay in days was, respectively, 3 (p<0.0001) and 8 (p<0.0001) for poor nutritional status, two (p=0.041) and 6.5 (p=0.006) for acute malnutrition, 3 (p<0.0001) and 8 (p<0.0001) for chronic malnutrition. Conclusion The personal biological factors with a significant association with mortality behavior were poor nutritional status and acute malnutrition. Children with analyzed nutritional deficits had a higher median time of MV and time of ICU compared with children without nutritional deficits.

Morbidity of congenital heart disease in children with anorectal malformations and related treatment / 浙江大学学报·医学版

OBJECTIVE@#To investigate the morbidity of congenital heart defects(CHDs) in children with anorectal malformation, and to summarize appropriate treatment.@*METHODS@#The clinical data and echocardiographic findings of 155 children with congenital anorectal malformations from the Third Affiliated Hospital of Zhengzhou University during January 2016 and October 2019 were reviewed. According to the surgical findings of anorectal malformations, the patients were categorized as the high/intermediate group and the low group; the CHDs were classified as minor CHDs and major CHDs. Multiple logistic regression was used to analyze the correlation of wingspread classification, and extracardiac malformations with the severity of CHDs.@*RESULTS@#Out of 155 children with anorectal malformations, 47 (30.3%) had different types of cardiac structural malformations, including 18 cases of minor CHDs (11.6%) and 29 cases of major CHDs (18.7%). Sixty children (38.7%) had extracardiac malformations, of which 38 cases (24.5%) had a single extracardiac malformation, 15 cases (9.7%) had multiple extracardiac malformations, 6 had trisomy 21 syndrome, and 1 had VATER syndrome. Multivariate logistic regression analysis showed that wingspread classification of anorectal malformation and extracardiac disorders were independent risk factors for major CHDs. The probability of major CHDs in children with high/intermediate anorectal malformation was 4.709 times higher than that with low anorectal malformation (@*CONCLUSIONS@#The morbidity of major CHDs is higher in severe cases with high/intermediate anorectal malformation and acute cases without fistula or with obstructed fistula and cases with multiple congenital disorders. Echocardiography can define the type and severity of CHDs, which are useful to develop the optimal diagnosis and treatment plan for children with anorectal malformation.

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge / Gravidez em Portadoras de Cardiopatias Congênitas Complexas: Um Constante Desafio

Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

Resumo Fundamento: A contínua habilidade na conduta das cardiopatias congênitas complexas (CCC) tem permitido o alcance da idade fértil. Contudo, a heterogeneidade das lesões cardíacas na idade adulta limita a estimativa do prognóstico da gravidez. Objetivo: Estudar a evolução materno-fetal das gestantes portadoras de CCC e analisar as variáveis presumíveis de prognóstico. Método: No período de 10 anos, 435 gestantes portadoras de cardiopatias congênitas foram consecutivamente incluídas no Registro do Instituto do Coração (Registro-InCor). Dentre elas, foram selecionadas 42 gestações em 40 mulheres com CCC (24,5 ± 3,4 anos) que haviam sido desaconselhadas a engravidar. As cardiopatias de base distribuíram-se em: transposição das grandes artérias, atresia pulmonar, atresia tricúspide, ventrículo único, dupla via de saída de ventrículo direito, dupla via de entrada de ventrículo esquerdo e outras lesões estruturais. As cirurgias realizadas foram Rastelli, Fontan, Jatene, Senning, Mustard e outros procedimentos combinados, como tunelização, Blalock Taussing e Glenn. Oito pacientes (20%) não haviam sido operadas, e 19 (47,5%) apresentavam hipoxemia. O protocolo de atendimento incluiu: registro da saturação de oxigênio, hemoglobina sérica, hematócrito, ajuste das medicações, anticoagulação individualizada e hospitalização a partir de 28 semanas de gestação, em face da gravidade do quadro clínico e obstétrico. Na análise estatística, o nível de significância adotado foi de 0,05. Resultado: Somente 17 gestações (40,5%) não tiveram complicações maternas nem fetais. Houve 13 problemas maternos (30,9%) e 2 mortes (4,7%) causadas por hemorragia pós-parto e pré-eclâmpsia grave, ambas em pacientes que apresentavam hipoxemia. Houve 7 perdas fetais (16,6%), 17 bebês prematuros (40,5%) e 2 recém-nascidos (4,7%) com cardiopatia congênita. As complicações materno-fetais foram significativamente maiores em pacientes que apresentavam hipoxemia (p < 0,05). Conclusão: O alcance da idade reprodutiva em pacientes com CCC é crescente; contudo, a má evolução materno-fetal desaconselha a gravidez, particularmente nas pacientes que apresentam hipoxemia.

Factores asociados a la supervivencia al año de vida en neonatos con cardiopatía congénita severa en un Hospital Nacional de Perú / Factors associated with survival at one year of life in neonates with severe congenital cardiopathy in A National Hospital in Peru

RESUMEN Objetivos. Determinar los factores asociados a la supervivencia en el primer año de vida en neonatos con cardiopatía congénita severa atendidos en un hospital nacional de Perú. Materiales y métodos. Se estudiaron 160 niños nacidos entre el 2012 y 2015 con diagnóstico de alguna cardiopatía congénita severa que ingresaron al Servicio de Neonatología del Hospital Nacional Edgardo Rebagliati Martins del Seguro Social del Perú. En el análisis de supervivencia se utilizó el método de Kaplan-Meier y la prueba Log-Rank. Se realizaron análisis crudos y ajustados mediante modelos de regresión de Cox. Resultados. El 52,5% de los pacientes fueron de sexo masculino y la cardiopatía congénita severa más frecuente fue la atresia pulmonar (26,3%). El 33,7% de los pacientes fallecieron, siendo la supervivencia al año del 66,3% (IC95% 58,4-73,0). El diagnóstico prenatal mejoró la supervivencia (HRa 0,54, IC95%: 0,30-0,98) mientras que las cardiopatías de tipo cianóticas (HRa 2,93, IC95%: 1,36-6,34) y la presencia de otra anomalía congénita (HRa 3,28, IC95%: 1,79-6,01) la disminuyeron, estos factores fueron también significativos en un segundo modelo estratificado por tratamiento quirúrgico con excepción del modelo estratificado por complicaciones donde un diagnóstico prenatal dejó de ser significativo. Conclusiones. El diagnóstico prenatal incrementa la supervivencia ante una cardiopatía congénita severa y permitiría un tratamiento quirúrgico oportuno; sin embargo, se debe considerar que las cardiopatías de tipo cianóticas y la presencia de otras anomalías congénitas extracardíacas disminuyen la supervivencia si se realiza una intervención quirúrgica o se presentan complicaciones.

ABSTRACT Objectives. To determine factors associated with survival in the first year of life in neonates with severe congenital heart disease treated in a national hospital in Peru. Materials and Methods. 160 children born between 2012 and 2015 with a diagnosis of severe congenital cardiopathy were studied and admitted to the Neonatology Service of the Edgardo Rebagliati Martins National Hospital of the Peruvian Social Security. The Kaplan-Meier method and the Log-Rank test were used in the survival analysis. Crude and adjusted analyses were performed using Cox regression models. Results. Fifty-two, point 5 percent (52.5%) of patients were male and the most frequent severe congenital cardiopathy was pulmonary atresia (26.3%). Thirty-three, point seven percent (33.7%) of patients died, with a 66.3% (IC95% 58.4-73.0) one-year survival. Prenatal diagnosis improved survival (HRa 0.54, 95% CI 0.30-0.98) while cyanotic cardiopathies (HRa 2.93, 95% CI 1.36-6.34) and the presence of another congenital anomaly (HRa 3.28, 95% CI 1.79-6.01) decreased it; these factors were also significant in a second model stratified by surgical treatment with the exception of the stratified model by complications where a prenatal diagnosis ceased to be significant. Conclusions. Prenatal diagnosis increases survival from severe congenital heart disease. However, cyanotic heart diseases and other congenital anomalies, which decrease this chance, should be considered, if surgery is performed or complications occur.

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)

Surgical treatment for congenital heart defects in down syndrome patients

Abstract Objective: To analyze data related to surgical treatment in patients with congenital heart defects (CHD) and Down syndrome (DS) based on information from International Quality Improvement Collaborative Database for Congenital Heart Disease (IQIC). Methods: Between July 1, 2010 and December 31, 2017, 139 patients with CHD and DS underwent surgery at Hospital de Base and Hospital da Criança e Maternidade de São José do Rio Preto (FUNFARME)/Faculdade de Medicina de São José do Rio Preto - SP (FAMERP). A quantitative, observational and cross-sectional study was performed in which the pre, intra and postoperative data were analyzed in an IQIC database. The data included gender, age, prematurity, weight, preoperative procedures, diagnosis, associated cardiac and non-cardiac anomalies, Risk Adjustment for Congenital Heart Surgery (RACHS-1), type of surgery, cardiopulmonary bypass (CPB), perfusion time, aortic clamping time and CPB temperature, bacterial sepsis, surgical site infection and other infections, length of stay in intensive care unit (ICU), length of hospital stay and in-hospital mortality. Results: The most prevalent procedures were complete atrioventricular septal defect repair (58 - 39.45%), followed by closure of ventricular septal defect (36 - 24.49%). The RACHS-1 categories 1, 2, 3 and 4 were distributed as 22 (15%); 49 (33.3%); 72 (49%) and 4 (2.7%), respectively. There were no procedures classified as categories 5 or 6. Bacterial sepsis occurred in 10.2% of cases, surgical site infection in 6.1%, other infections in 14.3%. The median length of ICU stay was 5 days and the median length of hospital stay was 11 days. In-hospital mortality was 6.8%. Conclusion: Surgical treatment in patients with CHD and DS usually does not require highly complex surgical procedures, but are affected by infectious complications, resulting in a longer ICU and hospital length of stay with considerable mortality.

Fetal and maternal factors are associated with mortality due to circulatory system disorders in children

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.

Supervivencia de los niños nacidos con cardiopatías congénitas en Costa Rica: estudio retrospectivo de la cohorte de nacimientos 2006-2007 / Survival of children born with congenital heart defects in Costa Rica: a retrospective study of the birth cohort 2006-2007

Resumen Justificación: alrededor del mundo las cardiopatías congénitas ocupan las primeras causas de mortalidad infantil. En Costa Rica son el grupo de defectos congénitos más frecuentes, con una prevalencia de 6 x 1000 nacimientos (IC95 % 5 -7 x 1000 nacimientos), que representan cerca del 13 % de la mortalidad infantil. El objetivo del estudio fue analizar la supervivencia a cinco años de edad de los niños nacidos con defectos cardiacos en Costa Rica. Métodos: se analizó una cohorte retrospectiva de 543 niños nacidos con cardiopatías congénitas entre enero de 2006 y junio de 2007 en Costa Rica; utilizando los registros médicos y la base nacional de defunciones se brindó un seguimiento mínimo de 5 años en todos los casos. Se obtuvo estimados de supervivencia de Kaplan-Meier al mes, año y cinco años de vida, y se evaluaron factores pronóstico empleando el modelo de riesgos proporcionales de Cox. Se estimaron riesgos relativos crudos y ajustados con su respectivo intervalo de confianza del 95 %. Resultados: la prevalencia de cardiopatías congénitas fue de 5,14 por 1000 nacimientos (IC95 % 4,73-5,60; n: 543) para el período de estudio. La mortalidad fue del 27,9 % (IC 95 % = 24,21- 31,73; n: 152). La supervivencia acumulada al año y cinco años fue del 76,1 % y el 72,4 %, respectivamente, frente al 99,1 % y 98,8 % sobrevivencia de la cohorte de nacimientos nacional (con y sin cardiopatía) de la misma edad. La edad temprana al diagnóstico, severidad, cardiopatías congénitas múltiples y la asociación de defectos congénitos mayores se asociaron significativamente (p≤0,05) a una menor probabilidad de supervivencia. Conclusiones: la cohorte de niños con cardiopatías congénitas estudiada presentó una alta mortalidad al año y cinco años. El peor pronóstico de supervivencia fue para aquellos que necesitaban una cirugía cardíaca a temprana edad.

Abstract Background: Around the world, congenital heart defects occupy the first causes of infant mortality. In Costa Rica heart malformations are the most frequent group of birth defects, with a prevalence of 6 x 1000 births (95% CI 5-7 x 1000 births). They represent about 13% of infant mortality and are. the leading cause of death due to birth defects. The objective of this study is to analyze the survival of children with CC in Costa Rica. Methods: A retrospective cohort of 543 children born with CC between January 2006 and June 2007 in Costa Rica was analyzed. Using medical records and the national database of deaths, a minimum follow-up of 5 years was given in all cases. Kaplan-Meier survival estimates were obtained at month, year and five years of life. Prognostic factors were assessed using the Cox proportional hazards model. Raw and adjusted relative risks were estimated with their respective 95% confidence interval. Results: The prevalence of CC was 5.14 per 1000 births (95% CI: 4.73-5.60; n: 543) for the study period. Mortality was 27.9% (95% CI: 24.21-31.73; n: 152). Cumulative survival at one year and five years was 76.1% and 72.4%, respectively, compared to 99.1% and 98.8% of survival in the same national birth cohort (with and without CC), at the same age. Early age at diagnosis, severity, multiple CC and the association of major BD were significantly associated (p≤0.05) with a lower probability of survival. Conclusions: The cohort of children with congenital heart disease studied had a high mortality rate at one year and five years. The worst prognosis of survival was for those who needed cardiac surgery at an early age.

Should sinus of valsalva be replaced in patients with dilated ascending aorta and aortic valve diseases?

Abstract Introduction: The aim of this study is to investigate the change in the dimension of sinus of Valsalva in patients who underwent supracoronary ascending aorta replacement with aortic valve replacement. Methods: A total of 81 patients who underwent supracoronary ascending aorta replacement with aortic valve replacement were included. Ten of 81 patients died during the follow-up. The patients were divided into three groups according to the aortic valve diseases. Group I (n=17) included patients with bicuspid valves, group II (n=30) included patients with stenotic degenerative valves, and patients with aortic regurgitation constituted group III (n=24). In preoperative and follow-up periods, the sinus of Valsalva diameter of the patients was evaluated by echocardiographic examination. The mean age was 54.1±15.1 years. Twenty-eight (34.6%) patients were female and 12 (14.8%) patients were in New York Heart Association functional class III. Results: There was no early mortality. Late mortality was developed in 10 (12.4%) patients, 8 (9.9%) due to non-cardiac reasons. Late follow-up was obtained in 71 patients with a mean of 60±30.1 months postoperatively. During follow-up, the increase in the diameter of the sinus of Valsalva was significant in Group I (P<0.01), while in Group II and III it was insignificant (P>0.05). Conclusion: To avoid the risks associated with sinus of Valsalva dilatation, it is reasonable to replace the sinus of Valsalva in the setting of aortic valve replacement and ascending aorta replacement for bicuspid aortic valve with a dilated ascending aorta and relatively normal sinuses of Valsalva in young patients.