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Resumo Objetivo: Sintetizar e analisar criticamente a literatura a respeito de potenciais biomarcadores associados à desfechos clínicos no pós-operatório de cirurgia cardíaca em lactentes e crianças em cuidados intensivos. Métodos: Revisão integrativa, cuja busca ocorreu nos meses de setembro e dezembro de 2019, nas bases de dados MEDLINE, ISI of Knowledge, CENTRAL Cochrane, EMBASE, CINAHL, Science Direct e LILACS para responder à questão norteadora: "Quais as evidências científicas acerca de potenciais biomarcadores relacionados à desfechos clínicos no pós-operatório de cirurgia cardíaca de lactentes e crianças em cuidado intensivo?" Foram incluídos artigos originais publicados entre 2000 e 2019, nos idiomas inglês, português ou espanhol. Excluiu-se toda a literatura cinzenta. Resultados: A amostra final foi constituída por oito artigos, sendo seis estudos observacionais prospectivos descritivos e dois coortes prospectivas. Na maioria dos estudos os pacientes pediátricos foram submetidos à técnica de Bypass Cardiopulmonar (BCP) intraoperatória durante cirurgia de cardiopatia congênita. Os potenciais biomarcadores analisados foram moléculas participantes de processos imune-inflamatórios, predominantemente citocinas pró-inflamatórias tais como IL-1β, IL-6, IL-8 e o fator de necrose tumoral-α (TNF-α) e seu receptor, ou citocinas anti-inflamatórias como a IL-10. Conclusão: As citocinas IL-6, IL-8 e IL-10, o cortisol e o lactato, apresentaram-se como moléculas promissoras para elucidação de mecanismos subjacentes a desfechos clínicos no pós-operatório de cirurgia cardíaca em lactentes e/ou crianças em cuidado intensivo. Tais moléculas podem assumir um caráter preventivo, podendo futuramente ser utilizadas como ferramentas diagnósticas e prognósticas alternativas para um regime que permita identificar pacientes sob alto risco de desenvolver complicações clínicas nos pós-operatórios.

Resumen Objetivo: Sintetizar y analizar críticamente la literatura sobre potenciales biomarcadores relacionados con resultados clínicos en el posoperatorio de cirugía cardíaca de lactantes y niños en cuidados intensivos. Métodos: Revisión integradora, cuya búsqueda ocurrió en los meses de septiembre y diciembre de 2019, en las bases de datos MEDLINE, ISI of Knowledge, CENTRAL Cochrane, EMBASE, CINAHL, Science Direct y LILACS para responder la pregunta orientadora: "¿Cuáles son las evidencias científicas sobre potenciales biomarcadores relacionados con resultados clínicos en el posoperatorio de cirugía cardíaca de lactantes y niños en cuidados intensivos?". Se incluyeron artículos originales publicados entre los años 2000 y 2019, en idioma inglés, portugués o español. Se excluyó toda la literatura gris. Resultados: La muestra final fue formada por ocho artículos, de los cuales seis eran estudios observacionales prospectivos y dos cohortes prospectivas. En la mayoría de los estudios, los pacientes pediátricos fueron sometidos a la técnica de bypass cardiopulmonar (BCP) intraoperatoria durante la cirugía de cardiopatía congénita. Los potenciales biomarcadores analizados fueron moléculas participantes de procesos inmunoinflamatorios, predominantemente citocinas proinflamatorias tales como IL-1β, IL-6, IL-8 y el factor de necrosis tumoral-α (TNF-α) y su receptor, o citocinas antinflamatorias como la IL-10. Conclusión: Las citocinas IL-6, IL-8 e IL-10, el cortisol y el lactato, se presentaron como moléculas promisorias para explicar mecanismos subyacentes de los resultados clínicos en el posoperatorio de cirugía cardíaca de lactantes o niños en cuidados intensivos. Estas moléculas pueden asumir un carácter preventivo y, en un futuro, pueden utilizarse como herramientas alternativas de diagnóstico y pronóstico para un régimen que permita identificar pacientes con alto riesgo de presentar complicaciones clínicas en el posoperatorio.

Abstract Objective: To summarize and critically analyze the literature on potential biomarkers associated with clinical outcomes in the postoperative cardiac surgery period in infants and children under intensive care. Methods: Integrative review, whose search was carried out in September and December 2019 in the databases MEDLINE, ISI Web of Knowledge, Cochrane Central Register of Controlled Trials, Embase, Cumulative Index to Nursing and Allied Health Literature, Science Direct, and Latin America and Caribbean Center on Health Sciences Information to answer the following guiding question: "What is the scientific evidence on potential biomarkers associated with clinical outcomes in the postoperative cardiac surgery period in infants and children under intensive care?". Original articles published between 2000 and 2019 in English, Spanish, or Portuguese were included. Gray literature was excluded. Results: Eight articles made up the final sample (six descriptive observational prospective studies and two prospective cohort studies). Most studies, pediatric patients were submitted to the intraoperative cardiopulmonary bypass technique during congenital heart disease surgeries. The potential biomarkers analyzed were molecules that participate in immune-inflammatory processes, mainly proinflammatory cytokines such as IL-1β, IL-6, IL-8, and tumor necrosis factor-alpha and its receptor, as well as anti-inflammatory cytokines such as IL-10. Conclusion: The IL-6, IL-8, and IL-10 cytokines, cortisol, and lactate showed as promising molecules for elucidating mechanisms underlying clinical outcomes in the postoperative cardiac surgery period in infants and/or children under intensive care. These molecules can take on a preventive role by being used as a diagnostic and prognostic tool in the future in a protocol that allows to identify patients with high risk to develop clinical complications during the postoperative period.

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OBJECTIVES: Prolonged critical illness after congenital heart surgery disproportionately harms patients and the healthcare system, yet much remains unknown. We aimed to define prolonged critical illness, delineate between nonmodifiable and potentially preventable predictors of prolonged critical illness and prolonged critical illness mortality, and understand the interhospital variation in prolonged critical illness. DESIGN: Observational analysis. SETTING: Pediatric Cardiac Critical Care Consortium clinical registry. PATIENTS: All patients, stratified into neonates (≤28 d) and nonneonates (29 d to 18 yr), admitted to the pediatric cardiac ICU after congenital heart surgery at Pediatric Cardiac Critical Care Consortium hospitals. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 2,419 neonates and 10,687 nonneonates from 22 hospitals. The prolonged critical illness cutoff (90th percentile length of stay) was greater than or equal to 35 and greater than or equal to 10 days for neonates and nonneonates, respectively. Cardiac ICU prolonged critical illness mortality was 24% in neonates and 8% in nonneonates (vs 5% and 0.4%, respectively, in nonprolonged critical illness patients). Multivariable logistic regression identified 10 neonatal and 19 nonneonatal prolonged critical illness predictors within strata and eight predictors of mortality. Only mechanical ventilation days and acute renal failure requiring renal replacement therapy predicted prolonged critical illness and prolonged critical illness mortality in both strata. Approximately 40% of the prolonged critical illness predictors were nonmodifiable (preoperative/patient and operative factors), whereas only one of eight prolonged critical illness mortality predictors was nonmodifiable. The remainders were potentially preventable (postoperative critical care delivery variables and complications). Case-mix-adjusted prolonged critical illness rates were compared across hospitals; six hospitals each had lower- and higher-than-expected prolonged critical illness frequency. CONCLUSIONS: Although many prolonged critical illness predictors are nonmodifiable, we identified several predictors to target for improvement. Furthermore, we observed that complications and prolonged critical care therapy drive prolonged critical illness mortality. Wide variation of prolonged critical illness frequency suggests that identifying practices at hospitals with lower-than-expected prolonged critical illness could lead to broader quality improvement initiatives.

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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.

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.

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Congenital heart diseases (CHDs) are considered the most frequent malformations. In Mexico, every year between 12 and 16 thousand children are born with some type of CHD and it is the second cause of mortality in children under 1 year of age and in children aged between 1 and 4 years. The problem of the care of CHDs is analyzed from a perspective of health policies in the country with an emphasis on regionalizing their care to rationalize the use of available resources and with the aim of serving the largest number of patients and achieve the best clinical results. It emphasizes the need to promote cooperation among the different civil society organizations and institutions to exchange ideas to create consensus and joint projects for a better use of available resources to achieve universal and quality care of children with CHD in all the country.

Las cardiopatías congénitas son consideradas las malformaciones más frecuentes. En México cada año entre 12 y 16 mil niños nacen con algún tipo de cardiopatía congénita, y es la segunda causa de mortalidad en menores de 1 año y en niños de entre 1 y 4 años. Se analiza la problemática de la atención de las cardiopatías congénitas desde una perspectiva de políticas de salud en el país, con énfasis en lograr regionalizar su atención con el fin de racionalizar la utilización de los recursos disponibles y con el objetivo de atender al mayor número de pacientes y lograr los mejores resultados clínicos. Se enfatiza la necesidad de promover la cooperación entre las diferentes organizaciones de la sociedad civil y las instituciones para intercambiar ideas que permitan crear consensos y proyectos conjuntos para una mejor utilización de los recursos disponibles para lograr la atención universal y de calidad de los niños con cardiopatía congénita en todo el país.

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Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease. Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery. Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Objetivo: Siguiendo el notable trabajo realizado por la Asociación Mexicana de Especialistas en Cardiopatías Congénitas (Asociación Mexicana de Especialistas en Cardiopatías Congénitas: AMECC) con el desarrollo de un registro nacional para la cirugía cardíaca congénita, la Sociedad Mundial de Pediatría y Cirugía Cardíaca Congénita ha implementado una plataforma internacional para recopilar datos y analizar los resultados de los niños con cardiopatía congénita. Metodología: Este manuscrito propone una posible colaboración entre la base nacional de datos cardiacos congénitos de México (RENACCAPE) y la Base de Datos Mundial para la Cirugía Cardíaca Pediátrica y Congénita (WDPCHS). Conclusión: Esta asociación promovería el deseo de los países de seguir desarrollando procesos de mejora de la calidad y mejorar el tratamiento general de los niños con cardiopatía congénita.

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Introduction and objectives: In some centers, the pulse oximetry is not performed with the justification of lack of the adequate oximeter. We compared the effectiveness of two brands of oximeters to perform it. Methods: In neonates, a term of the joint housing service of the Hospital General de Occidente in Zapopan, Jalisco, Mexico, from May to November 2018, an examination of the characteristics of the American Academy of Pediatrics with both oximeters (ChoiceMMed® and Masimo SET®) was carried out, comparing the detection of critical congenital heart disease, time of intake, and false positives. Results: In each group, 1022 patients were analyzed; with the Masimo SET® oximeter, 83 positive tests were obtained (8.12%), of which 22 cases had some heart disease (26.5%), which represents a sensitivity of 100%, specificity of 93.9%, positive predictive value of 26.5%, and negative predictive value of 100% (odds ratio [OR]: 0.73; 95% confidence interval [CI] 0.6-0.8). With the ChoiceMMed® oximeter, 168 positive tests were obtained (16.4%), of which 22 cases had some heart disease (13.09%), with a sensitivity of 100%, specificity of 85.4%, positive predictive value of 13.09%, and negative predictive value 100% (OR: 0.86; 95%CI: 0.8-0.92). Regarding the time to perform the cardiac sieve, the mean in minutes of the Masimo SET® oximeter was 5.38 and the ChoiceMMed® oximeter was 9.7 min. Conclusions: The ChoiceMMed® oximeter contains a large number of false positives and a greater number of echocardiograms and comparatively longer cardiac screen printing with Masimo SET®, however, both with a negative predictive value of 100% eliminating such excuses.

Introducción y objetivos: En algunos centros el tamiz cardíaco no se realiza con la justificación de no tener el oxímetro adecuado. Comparamos la efectividad de dos marcas de oxímetros para realizarlo. Métodos: En los neonatos a término del Servicio de alojamiento conjunto del Hospital General de Occidente en Zapopan Jalisco México de mayo a noviembre del 2018 se realizó tamiz cardíaco según las guías de la Academia Americana de Pediatría con dos oxímetros, el Masimo SET® (aprobado por la FDA) y el ChoiceMMed®, comparando su efectividad para la detección de cardiopatías congénitas críticas, el tiempo de toma y los falsos positivos. Resultados: En cada grupo se analizaron 1,022 pacientes; con el oxímetro Massimo SET® se obtuvieron 83 pruebas positivas (8.12%) de las cuales 22 casos presentaron alguna cardiopatía (26.5%), lo que representa una sensibilidad del 100%, una especificidad del 93.9%, un valor predictivo positivo del 26.5% y un valor predictivo negativo del 100% (Odd Ratio [OR]: 0.73; IC 95%: 0.6-0.8). Con el oxímetro ChoiceMMed® se obtuvieron 168 pruebas positivas (16.4%), de las cuales 22 casos presentaron alguna cardiopatía (13.09%), con una sensibilidad del 100%, una especificidad del 85.4%, un valor predictivo positivo del 13.09% y un valor predictivo negativo del 100% (OR: 0.86; IC 95%: 0.8-0.92). En cuanto al tiempo para realizar el tamiz cardíaco, la media en minutos del oxímetro Masimo SET® fue 5.38 y del oxímetro ChoiceMMed® fue 9.7 minutos. Conclusiones: El oxímetro ChoiceMMed® presentó mayor cantidad de falsos positivos y mayor tiempo de realización del tamiz cardíaco en comparación al Masimo SET®, sin embargo, ambos presentan un valor predictivo negativo del 100%, siendo igualmente útiles como método de detección de cardiopatías críticas.

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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.

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Abstract Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease. Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery. Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Resumen Objetivo: Siguiendo el notable trabajo realizado por la Asociación Mexicana de Especialistas en Cardiopatías Congénitas (Asociación Mexicana de Especialistas en Cardiopatías Congénitas: AMECC) con el desarrollo de un registro nacional para la cirugía cardíaca congénita, la Sociedad Mundial de Pediatría y Cirugía Cardíaca Congénita ha implementado una plataforma internacional para recopilar datos y analizar los resultados de los niños con cardiopatía congénita. Metodología: Este manuscrito propone una posible colaboración entre la base nacional de datos cardiacos congénitos de México (RENACCAPE) y la Base de Datos Mundial para la Cirugía Cardíaca Pediátrica y Congénita (WDPCHS). Conclusión: Esta asociación promovería el deseo de los países de seguir desarrollando procesos de mejora de la calidad y mejorar el tratamiento general de los niños con cardiopatía congénita.

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OBJECTIVES: Determine the risk factors for repeated episodes of acute kidney injury in children who undergo multiple cardiac surgical procedures. DESIGN: Single-center retrospective chart review. SETTING: Cardiac ICU at a quaternary pediatric care center. PATIENTS: Birth to 18 years who underwent at least two cardiac surgical procedures with cardiopulmonary bypass. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One-hundred eighty patients underwent two cardiac surgical procedures and 89 underwent three. Acute kidney injury was defined by the Kidney Disease: Improving Global Outcomes serum creatinine criteria. Acute kidney injury frequency was 26% (n = 46) after surgery 1, 20% (n = 36) after surgery 2, and 24% (n = 21) after surgery 3, with most acute kidney injury occurring on postoperative days 1 and 2. The proportion of patients with severe acute kidney injury increased from surgery 1 to surgery 3. Patients with acute kidney injury had a significantly longer duration of ventilation and length of stay after each surgery. The odds of acute kidney injury after surgery 3 was 2.40 times greater if acute kidney injury was present after surgery 1 or 2 (95% CI, 1.26-4.56; p = 0.008) after adjusting for confounders. The time between surgeries was not significantly associated with acute kidney injury (p = 0.85). CONCLUSIONS: In a heterogeneous population of pediatric patients with congenital heart disease undergoing multiple cardiopulmonary bypass surgeries, odds of acute kidney injury after a third surgery was increased by the presence of acute kidney injury after prior procedures. Time between surgery did not play a role in increasing odds of acute kidney injury. Further studies in a larger multicenter investigation are necessary to confirm these findings.

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Se presentó una gestante de 26 años de edad, mestiza, con antecedente de salud aparente evaluada en consulta de asesoramiento genético comunitaria con riesgo incrementado por exposición a teratógenos y mutágenos, que en la ecografía fetal del segundo trimestre se identificaron alteraciones cardiovasculares que pudieron estar relacionadas con anomalía de Ebstein. Este diagnóstico fue confirmado en el Centro Provincial de Genética Médica de Guantánamo y, posteriormente, en Santiago de Cuba. A pesar de ser una cardiopatía congénita poco frecuente, se consideraron: la intensificación de las acciones en la atención prenatal precoz de las gestantes en la comunidad, la realización y cumplimiento de los programas prenatales existentes en nuestro país, principalmente los ultrasonidos prenatales que continúan siendo el examen complementario de elección y ofrecer el asesoramiento genético específico en las familias afectadas(AU)

A 26-year-old mixed-race pregnant woman with a history of apparent health evaluated in a community genetic counseling clinic with increased risk due to exposure to teratogens and mutagens was presented. Fetal echography of the second trimester identified cardiovascular alterations that may have been related to Ebstein's anomaly. Diagnosis that was confirmed in the Provincial Center of Medical Genetics of Guantanamo later was confirmed in Santiago de Cuba. Despite being a rare congenital heart disease, the following were considered: the intensification of the actions in the prenatal care of pregnant women in the community, the realization and fulfillment of the prenatal programs existing in our country, mainly the prenatal ultrasounds that continue being the complementary examination of choice and offer specific genetic counseling in affected families(AU)

A presentou-se uma gestante de 26 anos de idade, mestiza, com antecedente de saúde aparente avaliada em consulta de assessoramento genético comunitária com risco incrementado por exposição a teratógenos e mutágenos, que na ecografia fetal do segundo trimestre se identificaram alterações cardiovasculares que puderam estar relacionadas com anomalia de Ebstein. Diagnóstico que foi confirmado no Centro Provincial de Genética Médica de Guantánamo e, posteriormente, em Santiago de Cuba. Apesar de ser uma cardiopatía congênita pouco frequente, consideraram-se: a intensificação das ações na atenção prenatal precoz das gestantes na comunidade, a realização e cumprimento dos programas prenatales existentes em nosso país, principalmente os ultrasonidos prenatales que continuam sendo o exame complementar de eleição e oferecer o assessoramento genético específico nas famílias afetadas(AU)

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BACKGROUND: Delayed sternal closure (DSC) after cardiac surgery is a therapeutic option in the treatment of the severely impaired heart in pediatric cardiac surgery. METHODS: A single-center retrospective review of all bypass surgeries performed over a 10-year period (2003-2012). RESULTS: Of a total of 2325 patients registered in our database, the DSC group included 259 cases (11%), and the remaining 2066 cases (89%) constituted the control group (PSC). RACHS-1 risk was higher for the DSC group (74% had a score of 3 or 4) than for the PSC group (82% had a score of 2 or 3). The most frequent diagnosis for the DSC group was transposition of the great arteries (28%). We found out that hemodynamic instability was the main indication observed in patients aged ≤ 8 years (63%), while bleeding was the principal indication for patients aged ≥ 8 years (94%) (p ≤ 0.001). The average time between surgery and sternal closure was 2.3 ± 1.4 days. Overall mortality rates were higher for patients of the DSC group (22%) than for the PSC group (8.7%) (OR: 0.4 (95% CI: 0.4 to 0.5), p < 0.05). There were six patients with DSC who developed mediastinitis (2.3%). The risk of mediastinitis was significantly higher when DSC was performed 4 days after the primary surgery. CONCLUSIONS: DSC is an important management strategy for congenital cardiac surgery in infants and children. The prolonged sternal closure time is associated with an increased rate of postoperative mediastinitis.

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INTRODUCTION: Congenital heart diseases are functional and structural alterations of the heart, circulatory system, and large vessels, which develop during cardiac embryogenesis. These defects are significant causes of morbidity and mortality in children worldwide. OBJECTIVE: To determine the prevalence of congenital heart diseases in Bogotá, Colombia, through the period 2001 to 2014 and evaluate the trend in time. MATERIALS AND METHODS: We analyzed 405,408 births from the Programa de Vigilancia y Seguimiento de Niños con Anomalías Congénitas de la Secretaría Distrital de Salud de Bogotá. We calculated the prevalence of congenital heart diseases through a daily registry of births, the clinical evaluation, and the work-up. Congenital heart diseases were classified as isolated, complex or associated. We estimated the prevalence per year and the mortality in the first hours of life. RESULTS: The total prevalence of congenital heart diseases was 15.1 per 10,000 newborns; however, we obtained values above 20 per 10,000 newborns in the previous 3 years. Of the newborns analyzed, 46% were females, 53.16% were males, and 0.33% undetermined. Out of the newborns with congenital anomalies, 397 had congenital heart diseases, 142 of which were classified as associated and 74 as complex. CONCLUSION: Congenital heart diseases have a significant impact on childhood morbidity and mortality. Although the prevalence continues to be low compared to other countries, this could be due to under registry nationwide. There was an increase in prevalence during the previous three years, which might be explained by the implementation of local and national programs. Therefore, we suggest continuing the development and promotion of such programs aimed at increasing screening and registration of these anomalies.

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Resumen Introducción. Las cardiopatías congénitas son alteraciones estructurales y funcionales del corazón, del sistema circulatorio y de los grandes vasos, que se desarrollan durante la embriogénesis cardiaca. A nivel mundial, dichos defectos son una causa importante de morbilidad y mortalidad en la población infantil. Objetivo. Determinar la prevalencia de cardiopatías congénitas en Bogotá durante el periodo de 2001 a 2014 y evaluar la tendencia en el tiempo. Materiales y métodos. Se analizóla informaciónde 405.408 recién nacidos registrados en el Programa de Vigilancia y Seguimiento de Niños con Anomalías Congénitas de la Secretaría de Salud de Bogotá y se calculó la prevalencia de las cardiopatías congénitas mediante un registro diario de nacimientos y su evaluación clínica y paraclínica. Las cardiopatías se clasificaron en aisladas, complejas y asociadas. Se halló la prevalencia por año y se evaluó la mortalidad en las primeras horas de vida. Resultados. La prevalencia de cardiopatías congénitas fue de 15,1 por cada 10.000 recién nacidos en todo el período, pero se evidenciaron valores por encima de 20 por 10.000 en los tresaños anteriores. Del total de recién nacidos evaluados, 46 % correspondió al sexo femenino, 53,16 % al sexo masculino y 0,33 % a sexo indeterminado. De los nacidos con malformaciones, 397 cardiopatías se clasificaron como aisladas, 142 se asociaron con otras malformaciones extracardiacas y 74 se consideraron complejas. Conclusiones. Las cardiopatías tienen gran impacto en la salud de la población infantil y, aunque la prevalencia es menor que en otros países, ello puede deberse al subregistro a nivel nacional. Se notó un aumento en la prevalencia en los tresaños anteriores, lo cual puede deberse a la implementación de los programas de vigilancia nacional y local. Sugerimos continuar desarrollando programas que incrementen e incentiven la tamización y el registro de estas enfermedades.

Abstract Introduction: Congenital heart diseases are functional and structural alterations of the heart, circulatory system, and large vessels, which develop during cardiac embryogenesis. These defects are significant causes of morbidity and mortality in children worldwide. Objective: To determine the prevalence of congenital heart diseases in Bogotá, Colombia, through the period 2001 to 2014 and evaluate the trend in time. Materials and methods: We analyzed 405,408 births from the Programa de Vigilancia y Seguimiento de Niños con Anomalías Congénitas de la Secretaría Distrital de Salud de Bogotá. We calculated the prevalence of congenital heart diseases through a daily registry of births, the clinical evaluation, and the work-up. Congenital heart diseases were classified as isolated, complex or associated. We estimated the prevalence per year and the mortality in the first hours of life. Results: The total prevalence of congenital heart diseases was 15.1 per 10,000 newborns; however, we obtained values above 20 per 10,000 newborns in the previous 3 years. Of the newborns analyzed, 46% were females, 53.16% were males, and 0.33% undetermined. Out of the newborns with congenital anomalies, 397 had congenital heart diseases, 142 of which were classified as associated and 74as complex. Conclusion: Congenital heart diseases have a significant impact on childhood morbidity and mortality. Although the prevalence continues to be low compared to other countries, this could be due to under registry nationwide. There was an increase in prevalence during the previous three years, which might be explained by the implementation of local and national programs. Therefore, we suggest continuing the development and promotion of such programs aimed at increasing screening and registration of these anomalies.

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Resumen: Objetivo: Describir la evolución clínica de pacientes pediátricos operados por cardiopatías congénitas en la UMAE de Yucatán. Métodos: Encuesta descriptiva sobre expedientes de pacientes pediátricos intervenidos quirúrgicamente por cardiopatía congénita del 1 de noviembre de 2011 al 30 de noviembre de 2013. Resultados: Las cardiopatías más frecuentes fueron la persistencia del conducto arterioso (37.6%) y la transposición de grandes vasos. La estancia en cuidados intensivos fue de 3 días (mediana). El 11.76% fallecieron por choque séptico (44.4%) en la mayoría de los casos. Las complicaciones más frecuentes fueron sepsis (5.9%), síndrome de bajo gasto (4.7%), paro cardíaco, bloqueo AV y taquicardia ventricular (2.4% cada uno). Existe una correlación positiva moderada entre las complicaciones trans y posquirúrgicas y la sobrevida o muerte del paciente. Conclusiones: El volumen de pacientes quirúrgicos es menor comparado con centros de referencia de cirugía cardiovascular. Existe una marcada tendencia a la realización de cirugías correctivas y paliativas en patologías específicas en pacientes con riesgos agregados o con «mala¼ anatomía cardiaca que impiden la corrección total de primera intención. Se deben realizar estudios epidemiológicos y clínicos prospectivos para conocer el comportamiento de las cardiopatías congénitas atendidas en la región. © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. Este es un artículo Open Access bajo la licencia CC BY-NC-ND (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Abstract: Objective: To describe the clinical course of paediatric patients undergoing surgery for congenital heart disease in UMAE of Yucatan. Methods: Descriptive review was performed on the records of paediatric patients undergoing surgery for congenital heart disease from 1 November 2011 to 30 November 2013. Results: The most frequent heart diseases were persistent ductus arteriosus (37.6%) and trans- position of the great vessels. The median intensive care stay was 3 days. Mortality was 11.76%, with septic shock (44.4%) in most cases. The most frequent complications were sepsis (5.9%), low cardiac output syndrome (4.7%), cardiac arrest, and AV block and ventricular tachycardia (2.4% each). There was a moderate positive correlation between surgical complications and survival or death. Conclusions: The number of surgical patients is lower compared to reference centres for cardiovascular surgery. There is a marked tendency to perform corrective and palliative surgeries in specific disease in patients with added risk or 'bad' cardiac anatomy that prevent full correction at the first attempt. Prospective epidemiological and clinical studies should be conducted to understand the behaviour of congenital heart diseases treated in the region. © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. This is an open access article under the CC BY-NC-ND license (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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OBJECTIVE: To describe the clinical course of paediatric patients undergoing surgery for congenital heart disease in UMAE of Yucatan. METHODS: Descriptive review was performed on the records of paediatric patients undergoing surgery for congenital heart disease from 1 November 2011 to 30 November 2013. RESULTS: The most frequent heart diseases were persistent ductus arteriosus (37.6%) and transposition of the great vessels. The median intensive care stay was 3 days. Mortality was 11.76%, with septic shock (44.4%) in most cases. The most frequent complications were sepsis (5.9%), low cardiac output syndrome (4.7%), cardiac arrest, and AV block and ventricular tachycardia (2.4% each). There was a moderate positive correlation between surgical complications and survival or death. CONCLUSIONS: The number of surgical patients is lower compared to reference centres for cardiovascular surgery. There is a marked tendency to perform corrective and palliative surgeries in specific disease in patients with added risk or 'bad' cardiac anatomy that prevent full correction at the first attempt. Prospective epidemiological and clinical studies should be conducted to understand the behaviour of congenital heart diseases treated in the region.

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La frecuencia del síndrome de Down es fuertemente dependiente de la edad materna y la incidencia varia según las edades maternas. Esta incidencia puede diferir de un país a otro de acuerdo a los factores sociales y ambientales predominantes en tales países. Este estudio pretende documentar la prevalencia de defectos cardíacos congénitos en pacientes con síndrome de Down, identificar la asociación entre la edad materna y la frecuencia de enfermedades cardíacas congénitas en estos sujetos. Métodos: El estudio fue prospectivo, cruzado-sectorial y consecutivo, involucrando a sujetos de dos centros los cuales eran un hospital terciario hospital vado y un centro cardiológico principal. Los niños con características clínicas de síndrome de Down fueron sometidos a ecocardiografías transtorácicas efectuadas por un cardiólogo. Resultados: Un total de 195 pacientes con síndrome de Down cuyas edades abarcaban entre 1 día a 12 años, fueron estudiados. La edad media + SD fue 241.03 + 529.71 días y la relación masculino a femenino de 1.04.1. El promedio de la edad materna iba desde los 19 a los 44 años con una edad media +SD de 32.36 + 5.43 años . Hasta el 16.9 % tenían un corazón estructuralmente normal. El defecto más común fue el defecto septal aurículoventricular 24.1 % . No hubo una asociación significativa entre la edad materna y la frecuencia de defectos cardíacos congénitos en estos sujetos. Conclusión: La incidencia de enfermedad cardíaca congénita es alta en los sujetos con síndrome de Down. Los defectos aurículoventriculares fueron los defectos cardíacos más comunes en estos sujetos. La incidencia de defectos cardíacos congénitos en los sujetos fue decreciendo de la edad materna de 36 años y por encima. La evaluación cardíaca rutinaria incluyendo la ecocardiografía se recomienda a todos los pacientes con síndrome de Down.

Summary The occurrence of Down syndrome is strongly dependent on maternal age and incidence varies at various maternal ages. This incidence may differ from one country to another according to the social and environmental factors predominant in such countries. The study aims to document the prevalence of congenital heart defects in patients with Down syndrome, identify the association between maternal age and occurrence of congenital heart disease among these subjects. Methods. The study was prospective, cross-sectional and consecutive, involving subjects from two centers which were, a tertiary hospital a private hospital and a major cardiology center. Children with clinical features of Down syndrome had transthoracic echocardiography done by a cardiologist. Results. A total of 195 patients with Down syndrome with their ages between 1 day to 12 years. The mean age + SD were 241.03 + 529.71 days and male to female ratio of 1.04.1. The mother´s age range was from 19 to 44 years with a mean age of + SD of 32.36+ 5.43 years. Up to 16.9 % had structurally normal heart. The most common heart defect was atrioventricular septal defect 24.1 %. There was no significant association between maternal age and occurrence of congenital heart defects in the subjects. Conclusion. The incidence of congenital heart disease is high among subjects with Down syndrome. Atrioventricular defects were the commonest cardiac defect among the subjects. The incidence of congenital heart defect in the subjects was decreasing from maternal age 36 years and above. Routine cardiac evaluation including echocardiography is recommended for all patients with Down syndrome

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OBJECTIVES: To evaluate the relationship between extracorporeal membrane oxygenation center volume and mortality in children undergoing heart operations using propensity score matching in a multiinstitutional cohort. DESIGN: Post hoc analysis of data from an existing national database, Pediatric Health Information System. Propensity score matching was performed to 1-1-1 match patients in low-volume (0-30 cases per year), medium-volume (31-50 cases per year), and high-volume (> 50 cases per year) categories. We tested the sensitivity of our findings by repeating the primary analyses using traditional statistical techniques (traditional regression-based methods and covariate adjustment using propensity score). SETTING: Forty-two children's hospitals across the Unites States. PATIENTS: Patients 18 years old or younger receiving extracorporeal membrane oxygenation before or after pediatric heart operation at a Pediatric Health Information System participating hospital (2004-2013) were included. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 3,502 from 42 hospitals qualified for inclusion. Using propensity score matching, 1,962 patients were matched 1-1-1 to compare the three volume categories (654 patients in each category). Overall mortality was 1,493 patients (43%). Before matching and adjustment, low- and medium-volume centers were associated with higher mortality (low versus high volume: unadjusted odds ratio, 1.99; 95% CI, 1.68-2.36; p < 0.001). After matching, there was no significant association between center volume and mortality in unadjusted and adjusted analyses (low versus high volume: unadjusted odds ratio, 1.06; 95% CI, 0.85-1.32; p = 0.62 and adjusted odds ratio, 0.97; 95% CI, 0.63-1.50; p = 0.90). This relationship remained similar for analyses using traditional statistical techniques (regression adjustment, low versus high volume: adjusted odds ratio, 1.23; 95% CI, 0.80-1.89; p = 0.35 and covariate adjustment using propensity score, low versus high volume: adjusted odds ratio, 1.16; 95% CI, 0.77-1.74; p = 0.49). CONCLUSIONS: We demonstrated no relationship between extracorporeal membrane oxygenation center volume and mortality. Further analyses are needed to evaluate this relationship.

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Copy number variation studies of known disorders have the potential to improve the characterization of clinical phenotypes and may help identifying candidate genes and their pathways. The authors described a child with congenital heart disease, microcephaly, facial dysmorphisms, developmental delay, learning difficulties, and behavioral problems. There was initially a clinical suspicion of 22q11.2 deletion syndrome (22q11.2 DS), but molecular cytogenetic analysis (array genomic hybridization [aGH]) showed the presence of a de novo 3.6-Mb interstitial microdeletion in 8p23.1. The main features of 8p23.1 DS include congenital heart disease and behavioral problems, in addition to minor dysmorphisms and mental delay. Therefore, this article highlights the application of aGH to investigate 8p23.1 deletion in nonconfirmed 22q11.2 DS patients presenting neurobehavioral disorders, congenital cardiopathy, and minor dysmorphisms.

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BACKGROUND: Children who have unhealthy lifestyles are predisposed to develop hypertension, dyslipidemia and other complications. The epidemic of obesity is also affecting children with congenital heart disease. The aim of this study is to estimate the prevalence of obesity and describe associated risk factors, including family history in children with congenital heart disease. METHODS: A cross-sectional study with 316 children and adolescents with congenital heart disease seen in an outpatient clinic of a reference hospital. Collected sociodemographic data included family history of chronic disease, dietary habits, laboratory tests (total cholesterol, HDL and LDL/cholesterol, triglycerides, fasting glucose, CRP, hematocrit and hemoglobin), and anthropometric assessment. Anthropometric data of the caregivers was self-reported. RESULTS: The prevalence of excess weight was 26.9%. Altered levels of total cholesterol were observed in 46.9%, of HDL in 32.7%, LDL in 23.6% and of triglycerides levels in 20.0%. A higher frequency of family history of obesity (42.6%; p = 0.001), dyslipidemia (48.1%; p = <0.001), diabetes (47.4%; p = 0.002), hypertension (39.2%; p = 0.006) and ischemic disease (43.7%; p = 0.023), as well as significantly higher values of triglycerides (p = 0.017), glycemia (p = 0.004) and C-reactive protein (p = 0.002) were observed among patients with excess weight. CONCLUSION: The presence of modifiable risk factors and the variables associated to excess weight in this population was similar to that described in the literature for children without congenital disease. As these children already present the risks associated to heart disease, it is particularly important to promote a healthy lifestyle in this group.

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