Biomarcadores associados à defechos clínicos pós-cirurgia cardíaca em terapia intensiva pediátrica / Biomarcadores relacionados con resultados clínicos en el posoperatorio de cirugía cardíaca en cuidados intensivos pediátricos / Biomarkers associated with postoperative cardiac surgery clinical outcomes in pediatric intensive care

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.

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.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica / La base de datos mundial de cirugía cardíaca pediátrica y congénita: una colaboración con el Registro Nacional de Cirugía Cardiaca Pediátrica (RENACCAPE)

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.

Epidemiología de las cardiopatías congénitas en Bogotá, Colombia, entre 2001 y 2014: ¿mejoría en la vigilancia o aumento en la prevalencia? / Epidemiology of congenital heart diseases in Bogotá, Colombia, from 2001 to 2014: Improved surveillance or increased prevalence?

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.

Morbilidad posquirúrgica en pacientes pediátricos operados por cardiopatías congénitas en la UMAE de Yucatán / Post-surgical morbidity in paediatric patients undergoing surgery for congenital heart disease in the UMAE of Yucatan, Mexico

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/).

Frecuencia de enfermedad cardíaca congénita en niños con Síndrome de Down: ¿cuál es la influencia de la edad materna? / 1 OCCURRENCE OF CONGENITAL HEART DISEASE AMONG CHILDREN WITH DOWN SYNDROME: WHAT IS THE INFLUENCE OF MATERNAL AGE ?

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

Laparoscopic colectomy in an adult with single ventricle physiology: Anesthetic implications and management.

Increasing numbers of adult patients with complex congenital heart conditions are presenting for noncardiac surgery later in life. These disorders can present challenges for surgical and anesthesia providers. Specifically, single ventricle lesions offer anatomic and physiologic concerns during the perioperative period. Single ventricle physiology represents a delicate balance between systemic and pulmonary blood flow. Any alterations in blood flow through these systems can produce undesirable hemodynamic changes, especially during the perioperative period. We present a case of an adult patient with a single left ventricle who presented for laparoscopic total colectomy due to inflammatory bowel disease. His abnormal anatomy coupled with the hemodynamic disruptions caused by laparoscopy presented significant anesthetic challenges. We highlight the anesthetic concerns of single ventricle physiology, specifically pertaining to laparoscopic surgery. We provide recommendations for safely managing these patients perioperatively. With detailed preoperative evaluation and close hemodynamic monitoring during the perioperative period, these patients can experience successful surgical and anesthetic outcomes.

Induced apnea enhances image quality and visualization of cardiopulmonary anatomic during contrast enhanced cardiac computerized tomographic angiography in children.

Objective: The purpose of our study was to determine the effect of induced apnea on quality of cardiopulmonary structures during computerized tomographic (CT) angiography images in children with congenital heart diseases. Methods: Pediatric patients with congenital heart defects undergoing cardiac CT angiography at our facility in the past 3 years participated in this study. The earlier patients underwent cardiac CT angiography without induced apnea and while, later, apnea was induced in patients, which was followed by electrocardiogram gated cardiac CT angiography. General anesthesia was induced using sleep dose of intravenous propofol. After the initial check CT, on request by the radiologist, apnea was induced by the anesthesiologist by administering 1 mg/kg of intravenous suxamethonium. Soon after apnea ensued, the contrast was injected, and CT angiogram carried out. CT images in the “apnea group” were compared with those in “nonapnea group.” After the completion of the procedure, the patients were mask ventilated with 100% oxygen till the spontaneous ventilation was restored. Results: We studied 46 patients, of whom 36 with apnea and yet another 10 without. The quality of the image, visualization of structures such as cardiac wall, outflow tracts, lung field, aortopulmonary shunts, and coronary arteries were analyzed and subjected to statistical analysis (Mann–Whitney U, Fischer’s exact test and Pearson’s Chi‑square test). In the induced apnea group, overall image quality was considered excellent in 89% (n = 33) of the studies, while in the “no apnea group,” only 30% of studies were excellent. Absent or minimal motion artifacts were seen in a majority of the studies in apnea group (94%). In the nonapnea group, the respiratory and body motion artifacts were severe in 50%, moderate in 30%, and minimal in 20%, but they were significantly lesser in the apnea group. All the studied parameters were statistically significant in the apnea group in contrast to nonapnea group (P < 0.000). Conclusion: The image quality of cardiac CT angiography greatly improves, and motion artifact significantly decreases with the use of induced apnea in pediatrics patients being evaluated for congenital heart disease. This technique poses no additional morbidity of significance.

Three-dimensional echocardiographic assessment of atrial septal defects.

Echocardiography provides a useful tool in the diagnosis of many congenital heart diseases, including atrial septal defects, and aids in further delineating treatment options. Although two-dimensional echocardiography has been the standard of care in this regard, technological advancements have made three-dimensional echocardiography possible, and the images obtained in this new imaging modality are able to accurately portray the morphology, location, dimensions, and dynamic changes of defects and many other heart structures during the cardiac cycle.

Impact of the International Quality Improvement Collaborative on outcomes after congenital heart surgery: A single center experience in a developing economy.

Background: The International Quality Improvement Collaborative (IQIC) for Congenital Heart Surgery in Developing Countries was initiated to decrease mortality and major complications after congenital heart surgery in the developing world. Objective: We sought to assess the impact of IQIC on postoperative outcomes after congenital heart surgery at our institution. Methods: The key components of the IQIC program included creation of a robust worldwide database on key outcome measures and nurse education on quality driven best practices using telemedicine platforms. We evaluated 1702 consecutive patients ≤18 years undergoing congenital heart surgery in our institute from January 2010-December 2012 using the IQIC database. Preoperative variables included age, gender, weight at surgery and surgical complexity as per the RACHS-1 model. The outcome variables included, in- hospital mortality, duration of ventilation, intensive care unit (ICU) stay, bacterial sepsis and surgical site infection. Results: The 1702 patients included 771(45.3%) females. The median age was 8 months (0.03-216) and the median weight was 6.1Kg (1-100). The overall in-‑hospital mortality was 3.1%, Over the three years there was a significant decline in bacterial sepsis (from 15.1%, to 9.6%, P < 0.001), surgical site infection (11.1% to 2.4%, P < 0.001) and duration of ICU stay from 114(8-999) hours to 72 (18-999) hours (P < 0.001) The decline in mortality from (4.3% to 2.2%) did not reach statistical significance. Conclusions: The inclusion of our institution in the IQIC program was associated with improvement in key outcome measures following congenital heart surgery over a three year period.

Adolescents and adults with congenital heart diseases in Oman

The aim of our study was to examine the spectrum, demographics, and mortality rate among adolescents and adults with congenital heart diseases [CHD] in Oman. Data was collected retrospectively from the Royal Hospital, Muscat, electronic health records for all patients with a diagnosis of CHD aged 13 years and above. Data was analyzed according to the type of CHD and in-hospital mortality was assessed using Kaplan-Meier survival analysis. A total of 600 patients with CHD were identified, among them 145 [24%] were aged 18 years or below. The median age was 24 years. The majority of patients had a simple form of CHD. Atrial and ventricular septal defects together constituted 62.8% of congenital heart diseases. Most patients were clustered in Muscat [32%] and the Batinah regions [31.1%] of Oman. Patients with tetralogy of Fallot and Fontan had shorter survival time than recorded in the published literature. Mostly simple forms of CHD in younger patients was observed. The survival rate was significantly shortened in more complex lesions compared to simple lesions. A national data registry for CHD is needed to address the morbidities and mortality associated with the disease

Frequency of congenital heart diseases in neonatology section of a tertiary care hospital Peshawar

To determine the frequency of common congenital heart diseases [CHD] in children presenting to Pediatrics, Department of Lady Reading Hospital, Peshawar. Cross sectional descriptive study. This study was conducted in the Pediatrics and Neonatology Department of Lady, Reading hospital Peshawar, from 14[th] July 2010 to 14[th] July 2011. A total of 481 patients, using 3.1% propotion of ASD in cases with CHD, 95% Confidence interval, 1.55% margin of error, under WHO software formula for sample size determination. Children from 0-18 years of age clinically diagnosed cases with their echocardio-graphy which was also suggestive of CHD were included in this study. Postoperative cases of CHD as they require different kind of management plan and Confirmed cases of CHD were excluded. Patients with acyanotic CHD were 416 [86.48%] and with cyanotic CHD were 65 [13.52%]. Cases of ventricular septal defect [VSD] were 190 [39.5%] and amongst them 120 [63.16%] were males and 70 [36.8%] were females. Cases of atrial septal defect [ASD] were 146 [30.35%] and amongst them 63 [43.15%] were males and 83 [56.84%] were female. Thus, there was a female preponderance of ASD patients as compared to VSD. As for patent ductus arteriosus [PDA] there were 80 [16.63%] cases and of transposition of great arteries [TGA] were 30 [6.23%]. Whereas cases of tetrology of fallot [TOF] were 35 [7.27%]. Congenital heart defects [CHD] are among the most common birth defects and are the leading cause of birth defect-related deaths. VSD is the commonest acyanotic CHD whereas TOF is the commonest cyanotic heart disease

Modified Blalock Taussig shunt: Comparison between neonates, infants and older children.

Objective: The aim was to compare various pre-and post-operative parameters and to identify the predictors of mortality in neonates, infants, and older children undergoing Modifi ed Blalock Taussig shunt (MBTS). Materials and Methods: Medical records of 134 children who underwent MBTS over a period of 2 years through thoracotomy were reviewed. Children were divided into three groups-neonates, infants, and older children. For analysis, various pre-and post-operative variables were recorded, including complications and mortality. Results: The increase in PaO2 and SaO2 levels after surgery was similar and statistically signifi cant in all the three groups. The requirement of adrenaline, duration of ventilation and mortality was signifi cantly higher in neonates. The overall mortality and infant mortality was 4.5% and 8%, respectively. Conclusion: Neonates are at increased risk of complications and mortality compared with older children. Age (<30 days), weight (<3 kg), packed red blood cells transfusion >6 ml/kg, mechanical ventilation >24 h and post shunt increase in PaO2 (PDiff) <25% of baseline PaO2 are independent predictors of mortality in children undergoing MBTS.

Assessment of carotid body tumor and its association with tetralogy of fallot: Effect of the chronic hypoxia

This is a rare combined presentation of Tetralogy of Fallot and carotid body tumor [CBT]. Hypotheses and further discussion provides data for the development of CBT as a response to chronic hypoxemia. This present study demonstrates and discusses such an occurrence

Congenital Heart Disease in the Niger Delta region of Nigeria : a Four-Year Prospective Echocardiographic Analysis : Cardiovascular Topic

Introduction : Echocardiographic evaluation remains the gold standard for the diagnosis of structural cardiac disease. No previous prospective studies have been done on the prevalence of congenital heart disease (CHD) in the Niger Delta area. This study was done to determine the frequency and pattern of congenital heart disease; using echocardiography as a diagnostic tool. Methods : All patients presenting to the Paediatric Cardiology clinics of two centres; the University of Port Harcourt Teaching Hospital and the Paediatric Care Hospital between April 2009 and March 2013; were recruited and all had echocardiography performed.Results : Prevalence of CHD in this study was 14.4 per 1 000 children; 277 (83.4) of the patients had acyanotic CHD and 55 (16.6) had cyanotic CHD. Ventricular septal defect and tetralogy of Fallot were the commonest acyanotic and cyanotic heart defects; respectively.Conclusion : The high prevalence of CHD in this study is the highest in the country and Africa; and may be attributable to the increased oil spillage and gas flaring from petroleum exploitation in this region

Características epidemiológicas e genéticas associadas à gravidade da bronquiolite viral aguda pelo vírus sincicial respiratório / Epidemiological and genetic characteristics associated with the severity of acute viral bronchiolitis by respiratory syncytial virus

OBJETIVO: avaliar os fatores epidemiológicos e genéticos associados à gravidade da Bronquiolite Viral Aguda (BVA) pelo Vírus Sincicial Respiratório (VSR). FONTE DOS DADOS: foram utilizados descritores "bronchiolitis", "risk factor", "genetics" e "respiratory syncytial virus" e todas as combinações entre eles, nas bases de dados PubMed, SciELO e Lilacs publicados após o ano de 2000 e que incluíram indivíduos menores de dois anos de idade. SÍNTESE DOS DADOS: foram encontrados 1.259 artigos e lidos seus respectivos resumos. Destes foram selecionados 81 que avaliaram fatores de risco para a gravidade da BVA para leitura na íntegra, e foram incluídos os 60 estudos mais relevantes. Os fatores epidemiológicos associados com a gravidade da BVA pelo VSR foram: prematuridade, tabagismo passivo, baixa idade, ausência de aleitamento materno, doença pulmonar crônica, cardiopatia congênita, sexo masculino, etnia, coinfecção viral, baixo peso na admissão hospitalar, tabagismo materno na gestação, dermatite atópica, ventilação mecânica no período neonatal, antecedente materno de atopia e/ou asma na gestação, estação do nascimento, baixo nível socioeconômico, síndrome de Down, poluição ambiental, morar em altitude acima de 2.500 metros do nível do mar e parto cesariana. Em contrapartida, algumas crianças com BVA grave não apresentam nenhum desses fatores de risco. Neste sentido, estudos recentes têm verificado a influência de fatores genéticos relacionados à gravidade da BVA pelo VSR. Polimorfismos dos genes TLRs, RANTES, JUN, IFNA5, NOS2, CX3CR1, ILs e VDR têm-se mostrado associados com a evolução mais grave da BVA pelo VSR. CONCLUSÃO: a gravidade da BVA pelo VSR é um fenômeno dependente da interação entre variáveis epidemiológicas, ambientais e genéticas em seus diferentes graus de interação.

OBJECTIVE: to assess the epidemiological and genetic factors associated with severity of acute viral bronchiolitis (AVB) by respiratory syncytial virus (RSV). DATA SOURCE: the key words ''bronchiolitis'', ''risk factor'', ''genetics'' and ''respiratory syn-cytial virus'', and all combinations among them were used to perform a search in the PubMed,SciELO, and Lilacs databases, of articles published after the year 2000 that included individualsyounger than 2 years of age. DATA SYNTHESIS: a total of 1,259 articles were found, and their respective summaries were read. Of these, 81 were selected, which assessed risk factors for the severity of AVB, and were read in full; the 60 most relevant studies were included. The epidemiologic factors associated with AVB severity by RSV were prematurity, passive smoking, young age, lack of breastfeeding, chronic lung disease, congenital heart disease, male gender, ethnicity, viral coinfection, low weight at admission, maternal smoking during pregnancy, atopic dermatitis, mechanical ventilation in the neonatal period, maternal history of atopy and/or asthma during pregnancy, season of birth, low socioeconomic status, Down syndrome, environmental pollution, living at an altitude > 2,500 meters above sea level, and cesarean section birth. Conversely, some children with severe AVB did not present any of these risk factors. In this regard, recent studies have verified the influence of genetic factors on the severity of AVB by RSV. Polymorphisms of the TLRs, RANTES, JUN, IFNA5, NOS2, CX3CR1, ILs, and VDR genes have been shown to be associated with more severe evolution of AVB by RSV. CONCLUSION: the severity of AVB by RSV is a phenomenon that depends on the varying degrees of interaction among epidemiological, environmental, and genetic variables.

Características cardiovasculares y perinatales de la mujer embarazada con cardiopatía congénita o adquirida y del recién nacido / Cardiovascular and perinatal characteristics of pregnant women with congenital or acquired heart disease and characteristics of the newborn", "_i": "

Introducción: El embarazo y el parto por sí mismos exponen riesgos importantes a la mujer con cardiopatía, más aún cuando ésta es de origen congénito. En instituciones especializadas en cardiología donde se otorga atención durante el parto y puerperio inmediato, el conocimiento de sus características permite enfocar la atención de enfermería cardiovascular a la prevención secundaria de la mujer y al producto de alto riesgo. Objetivo: Comparar las características cardiovasculares y perinatales de la mujer embarazada con cardiopatía congénita o adquirida y del recién nacido. Material y métodos: Es un estudio comparativo, retrospectivo y transversal (2006-2011). Muestra por conveniencia (n = 76), se incluyeron expedientes clínicos de mujeres con cardiopatía, hospitalizadas en una institución de tercer nivel, que cursaron con embarazo. Datos recolectados con instrumento ex professo, analizados con frecuencias, porcentajes, medias, prueba de Pearson, Spearman, χ2 y t Student; p ‹ 0.05 como significativo. Resultados: 53.3% mujeres con cardiopatía adquirida; edad promedio 26.6 ± 6.2 años; semanas de gestación 36.1 ± 2.2; 90.8% tuvo control prenatal, siendo semejante en ambos grupos (χ2 = 0.948, gl = 1, p = 0.442). El 34.7% interrumpió su embarazo por agravamiento cardiovascular; 76.3% tuvo una clase funcional I; 63.3% de recién nacidos fueron niñas; 5.3% con defecto al nacimiento. A menor edad de la primera relación sexual se observa un embarazo más temprano (r = 0.833, p = 0.000); sin embargo, el grado académico no se relaciona en este aspecto (rs = 0.052, p = 0.658). Conclusiones: Las características de la mujer con cardiopatía congénita y cardiopatía adquirida que se embarazan son semejantes; sin embargo, precisan del consejo pre-concepcional para su planificación, así como un control prenatal realizado por las enfermeras especializadas y el equipo multidisciplinario, para su éxito.

Introduction: Pregnancy and childbirth are themselves significant risks for women with heart disease, even more if it is of congenital origin. In cardiology specialist institutions, where care is given during labor and immediate postpartum, knowledge of its features allows to focus the attention of nursing to cardiovascular secondary prevention of women and to the high-risk product. Objective: To compare the cardiovascular and perinatal characteristics of pregnant women with congenital or acquired heart disease and those of the newborn. Material and methods: Comparative, retrospective, cross-sectional study (2006-2011). This convenience sample (n = 76) included medical records of women hospitalized in a tertiary institution, who had gone through pregnancy disease. Data collected with an ex profeso instrument, analyzed with frequencies, percentages, averages, Pearson test, Spearman, χ2, Student´s t, p ‹ 0.05 as significant. Results: 53.3% women with acquired heart disease, mean age 26.6 ± 6.2 years; weeks of gestation 36.1 ± 2.2, 90.8% had prenatal care, being similar in both groups (χ2 = 0.948, df = 1, p = 0.442). 34.7% discontinued their pregnancy because of worsening cardiovascular, 76.3% had a functional class I, 63.3% of infants were girls; 5.3% with birth defect. At a younger age of first sexual intercourse, earlier pregnancy (r = 0.833, p = 0.000) was observed, but the academic degree is not related in this regard (rs = 0.052, p = 0.658). Conclusions: The characteristics of women with congenital heart disease and acquired heart disease who become pregnant are similar, however, they require the pre-conception advice for planning and prenatal monitoring by specialist nurses and the multidisciplinary team for its success.

Segundo Consenso Clínico de la Sociedad Iberoamericana de Neonatología: manejo hemodinámico del recién nacido / Second Clinical Consensus of the Ibero-American Society of Neonatology: hemodynamic management of newborns

Este trabajo informa acerca del proceso y los resultados del Segundo Consenso Clínico de la SIBEN (Sociedad Iberoamericana de Neonatología), en el cual 80 neonatólogos de 23 países fueron invitados a participar y colaborar. Se desarrollaron varias preguntas de importancia clínico-fisiológica sobre el manejo hemodinámico del recién nacido. Los participantes fueron distribuidos en grupos, facilitando así la interacción y el trabajo conjunto, con la consigna de responder de tres a cinco preguntas mediante el análisis de bibliografía y factores locales. El Grupo de Consenso se reunió en Mar del Plata, Argentina, donde se llevaron a cabo diversas ponencias, debates y presentaciones. En total participaron 54 neonatólogos de 21 países, con el objetivo de desarrollar un consenso sobre aspectos que incluyeron conceptos y definiciones de inestabilidad hemodinámica, la fisiopatología del cuadro de compromiso hemodinámico, las estrategias terapéuticas recomendadas y el monitoreo hemodinámico. Se espera que esta experiencia internacional sirva como una iniciativa útil tanto para la búsqueda de futuros consensos como para reducir las disparidades existentes entre los tratamientos y resultados de los diferentes países de la Región.

This study reports on the process and results of the Second Clinical Consensus of the Ibero-American Society of Neonatology. Eighty neonatologists from 23 countries were invited to collaborate and participate in the event. Several questions of clinical-physiological importance in the hemodynamic management of newborns were addressed. Participants were divided into groups to facilitate interaction and teamwork, with instructions to respond to three to five questions by analyzing the literature and local factors. Meeting in Mar del Plata, Argentina, the Consensus Group served as a form for various presentations and discussions. In all, 54 neonatologists from 21 countries attended, with the objective of reaching a consensus on such matters as concepts and definitions of hemodynamic instability, the physiopathology of hemodynamic compromise, recommended therapy strategies, and hemodynamic monitoring. It is hoped that this international experience will serve as a useful initiative for future consensus building and reduction of the existing disparities among the countries of the Region in terms of treatment and outcomes.

Anestesia intraóssea em estudo hemodinâmico em criança cardiopata / Intraosseous anesthesia in hemodynamic studies in children with cardiopathy / Anestesia intraosea en estudio hemodinamico en nino cardiopata

JUSTIFICATIVA E OBJETIVOS: O acesso intraósseo (IO) tem sido utilizado com bons resultados em situações de emergência quando não há acesso venoso disponível para a administração de fluidos e fármacos. O objetivo do presente estudo foi avaliar se o acesso IO é uma técnica útil para a administração de anestesia e fluidos no estudo hemodinâmico quando é impossível obter acesso periférico. Este estudo foi realizado na Unidade de Hemodinâmica de um hospital universitário, com 21 lactentes que apresentavam doença cardíaca congênita agendados para estudo hemodinâmico diagnóstico. MÉTODOS: Este estudo comparou a efetividade do acesso IO em relação ao EV para a infusão de anestésicos (cetamina, midazolam e fentanil) e fluidos durante estudo hemodinâmico. Tempo de indução anestésica, duração do procedimento, tempo de recuperação da anestesia, hidratação e complicações das punções EV e IO foram comparados entre os grupos. RESULTADOS: O tempo de punção foi significativamente menor no grupo IO (3,6 minutos) do que no grupo EV (9,6 minutos). O tempo de início da ação do anestésico foi mais rápido no grupo EV (56,3 segundos) do que no grupo IO (71,3 segundos). Não foram observadas diferenças significativas entre os dois grupos em relação à hidratação (grupo EV 315,5 mL vs. grupo IO 293,2 mL) e o tempo de recuperação da anestesia (grupo IO 65,2 min vs grupo EV 55,0 min). O sítio da punção foi reavaliado após 7 e 15 dias, não apresentando sinais de infecção ou outras complicações. CONCLUSÕES: Os resultados demonstraram superioridade da infusão IO em relação ao tempo de punção. Devido à sua manipulação bastante fácil e à sua eficiência, a hidratação e a anestesia feitas através de infusão IO se revelaram satisfatórias para estudos hemodinâmicos sem a necessidade de outros acessos.

BACKGROUND AND OBJECTIVES: Intraosseous (IO) access has been used with good results in emergency situations, when venous access is not available for fluids and drugs infusion. The objective of this study was to evaluate IO a useful technique for anesthesia and fluids infusion during hemodynamic studies and when peripheral intravascular access is unobtainable. The setting was an university hospital hemodynamics unit, and the subjects were twenty one infants with congenital heart disease enrolled for elective hemodynamic study diagnosis. METHODS: This study compared the effectiveness of IO access in relation to IV access for infusion of anesthetics agents (ketamine, midazolam, and fentanyl) and fluids during hemodynamic studies. The anesthetic induction time, procedure duration, anesthesia recovery time, adequate hydration, and IV and IO puncture complications were compared between groups. RESULTS: The puncture time was significantly smaller in IO group (3.6 min) that in IV group (9.6 min). The anesthetic onset time (56.3 second) for the IV group was faster than IO group (71.3 second). No significant difference between groups were found in relation to hydration (IV group, 315.5 mL vs IO group, 293.2 mL), and anesthesia recovery time (IO group, 65.2 min vs IV group, 55.0 min). The puncture site was reevaluated after 7 and 15 days without signs of infection or other complications. CONCLUSIONS: Results showed superiority for IO infusion when considering the puncture time of the procedure. Due to its easy manipulation and efficiency, hydration and anesthesia by IO access was satisfactory for hemodynamic studies without the necessity of other infusion access.

JUSTIFICATIVA Y OBJETIVOS: El acceso intraoseo (IO), se ha venido utilizando con buenos resultados en situaciones de emergencia, cuando no existe el acceso venoso disponible para la administracion de fluidos y farmacos. El objetivo del presente estudio es evaluar si el acceso IO es una tecnica util para la administracion de anestesia y de fluidos en el estudio hemodinamico cuando el acceso periferico es imposible de obtenerse. Ese estudio fue realizado en el laboratorio de hemodinamica de un hospital universitario, con 21 lactantes portadores de enfermedad cardiaca congenita que fueron seleccionados para un estudio hemodinamico diagnostico. MÉTODOS: Este estudio comparo la efectividad del acceso IO con relacion al EV para la infusion de anestesicos (quetamina, midazolam y fentanil), y de fluidos durante el estudio hemodinamico. El tiempo de induccion anestesica, la duracion del procedimiento, el tiempo de recuperacion de la anestesia, la adecuada hidratacion y las complicaciones de las punciones EV e IO se compararon entre los grupos. RESULTADOS: El tiempo de puncion fue significativamente menor en el grupo IO (3,6 minutos) que en el grupo EV (9,6 minutos). El tiempo de inicio de la accion de la anestesia fue mas rapido en el grupo EV (56,3 segundos) que en el grupo IO (71,3 segundos). No se observaron diferencias significativas entre los dos grupos con relacion a la hidratacion (grupo EV 315,5 mL vs. grupo IO 293,2 mL), y sobre el tiempo de recuperacion de la anestesia (grupo IO 65,2 min vs, grupo EV 55,0 min). El sitio de la puncion se evaluo nuevamente despues de 7 a 15 dias, y no presento senales de infeccion u otras complicaciones. CONCLUSIONES: Los resultados comparativos arrojaron una superioridad de la infusion IO con relacion al tiempo de puncion. Debido a su eficiencia y manipulacion bastante facil, la hidratacion y la anestesia que se hicieron por medio de la infusion IO demostraron ser satisfactorias para los estudios hemodinamicos sin la necesidad de otros accesos.