Dificuldades de deglutição nas diferentes formas de oferta de alimentação em lactentes cardiopatas congênitos: uma revisão sistemática / Swallowing difficulties in the different forms of food supply in congenital heart disease infants: a systematic review / Dificultades para tragar em las diferentes formas de su ministro de alimentos em lactantes com cardiopatias congénitas: una revisión sistemática

Introdução: A forma de alimentação mais segura nos bebês cardiopatas pode ser um desafio para escolha da equipe multiprofissional. Objetivo: Identificar as principais dificuldades de deglutição nas diferentes formas de oferta de alimentação em lactentes cardiopatas congênitos. Métodos: A questão norteadora foi: "Quais as principais dificuldades de deglutição nas diferentes formas de oferta de alimentação em lactentes cardiopatas congênitos?". A população foi delimitada como lactentes cardiopatas, considerando amamentação como exposição de interesse e alimentação em mamadeira considerado grupo comparação. Dificuldades de deglutição foram consideradas desfecho. Foram selecionados artigos sem restrição de idioma, independentemente do ano de publicação até abril de 2019, que apresentassem no título, resumo ou corpo do artigo relação com o objetivo da pesquisa e os critérios de elegibilidade, com delineamento observacional. Após a extração dos dados, as medidas foram transformadas em percentagem, e descritas em uma síntese qualitativa. Resultados: Foram encontrados 828 artigos ao total, sendo que após análises, foram incluídos 11 artigos ao total. As principais dificuldades apresentadas pelos lactentes cardiopatas em seio materno foram: tosse, engasgo, cianose, queda da saturação periférica de oxigênio e incoordenação entre sucção, respiração e deglutição. As dificuldades de deglutição mais encontradas na oferta de seio materno foram: tosse, engasgo, cianose, queda de saturação, incoordenação entre sucção-respiração-deglutição, fadiga, escape oral, tempo prolongado de alimentação, vedamento-labial inadequado, preensão inadequada do bico, e ausculta cervical alterada. Conclusão: Os lactentes cardiopatas apresentam dificuldades de deglutição tanto em seio materno quanto em mamadeira, sendo observada maior frequência de apresentações das dificuldades, com mamadeira. (AU)

Introduction: The safest way of feeding babies with heart disease can be a challenge for the multidisciplinary team to choose. Objective: To identify the main swallowing difficulties in the different forms of feeding in infants with congenital heart disease. Methods: The guiding question was: "What are the main swallowing difficulties in the different forms of feeding in infants with congenital heart disease?". The population was defined as infants with heart disease, considering breastfeeding as exposure of interest and bottle feeding considered a comparison group. Swallowing difficulties were considered the outcome. Articles without language restriction were selected, regardless of the year of publication until April 2019, which presented in the title, abstract or body of the article a relationship with the objective of the research and the eligibility criteria, with an observational design. After data extraction, the measurements were transformed into percentages and described in a qualitative synthesis. Results: A total of 828 articles were found, and after analysis, 11 articles were included in total. The main difficulties presented by infants with heart disease at the mother's breast were coughing, choking, cyanosis, drop in peripheral oxygen saturation and incoordination between sucking, breathing and swallowing. The swallowing difficulties most found in the offer of the mother's breast were: cough, choking, cyanosis, drop in saturation, incoordination between sucking-breathing-swallowing, fatigue, oral leakage, prolonged feeding time, inadequate lip sealing, inadequate nipple grip, and altered cervical auscultation. Conclusion: Infants with heart disease have swallowing difficulties both in the mother's breast and in the bottle, with a higher frequency of presentations of difficulties being observed with the bottle. (AU)

Introducción: La forma más segura de alimentación en bebés com enfermidades del corazón puede ser um desafío para el equipo multidisciplinario para elegir. Objetivo: Identificar las principales dificultades deglutorias em las diferentes formas de alimentación en lactantes com cardiopatías congénitas. Métodos: La pregunta orientadora fue: "¿Cuáles son las principales dificultades deglutorias em las diferentes formas de alimentación en lactantes com cardiopatías congénitas?" La población se definió como lactantes com cardiopatías, considerando la lactancia materna como exposición de interés y la alimentación com biberón considerada una grupo de comparación. Las dificultades para tragar se consideraron el desenlace. Se seleccionaron artículos sin restricción de idioma, independentemente del año de publicación hasta abril de 2019, que presentaran em el título, resumen o cuerpo del artículo relación com el objetivo de la investigación y los criterios de elegibilidad, com um diseño observacional. Después de la extracción de datos, las medidas se transformaron em porcentajes y se describieron en una sínteses cualitativa. Resultados: Se encontraron un total de 828 artículos, y después del análisis, se incluyeron 11 artículos en total. Las principales dificultades que presentaron los lactantes com cardiopatia em el pecho materno fueron: tos, ahogo, cianosis, caída de la saturación periférica de oxígeno y falta de coordinación entre la succión, la respiración y la deglución. Las dificultades de deglución más encontradas em la oferta del pecho de la madre fueron: tos, ahogo, cianosis, descenso de la saturación, descoordinación entre succión-respiración-deglución, fatiga, escape oral, tiempo de alimentación prolongado, sellado labial inadecuado, agarre inadecuado del pezón y auscultación cervical alterada. Conclusión: Los lactantes com cardiopatia presentan dificultades para la deglución tanto em el pecho materno como em el biberón, observándose una mayor frecuencia de presentaciones de dificultades con el biberón. (AU)

Hipertensión pulmonar en cardiopatías congénitas del adulto / Pulmonary hypertension in adult congenital heart disease / Hipertensão pulmonar na cardiopatia congênita do adulto

Introducción: la hipertensión pulmonar asociada a cardiopatías congénitas del adulto implica un amplio espectro de situaciones clínicas complejas de difícil manejo para el cardiólogo clínico. Revisión: se repasarán los principales aspectos fisiopatológicos relacionados con el desarrollo de hipertensión pulmonar en los diferentes grupos de cardiopatías congénitas, así como los criterios para establecer el diagnóstico. Se discutirá el tratamiento guiado por perfil de riesgo y los grupos farmacológicos disponibles en nuestro medio. Por último, se discutirán grupos especiales, como el síndrome de Eisenmenger y la embarazada con cardiopatía e hipertensión pulmonar. Conclusión: es importante tener presente de todas formas que la hipertensión pulmonar en este escenario puede tener un mal pronóstico (como en el síndrome de Eisenmenger), por lo cual se deben realizar los mayores esfuerzos para hacer un diagnóstico precoz y tratamiento oportuno de estos pacientes.

Introduction: pulmonary hypertension associated with congenital heart disease in adults involves a wide spectrum of complex clinical situations that are difficult to manage for the clinical cardiologist. Review: the main pathophysiological aspects related to the development of pulmonary hypertension in the different groups of congenital heart disease will be reviewed, as well as the criteria to establish the diagnosis. Treatment guided by risk profile and the pharmacological groups available in our setting will be discussed. Finally, special groups such as Eisenmenger syndrome and pregnant women with heart disease and pulmonary hypertension will be discussed. Conclusion: it is important to keep in mind, however, that pulmonary hypertension in this scenario could have a poor prognosis (for example in Eisenmenger syndrome), which is why every effort should be made to make an early diagnosis and timely treatment of these patients.

Introdução: a hipertensão pulmonar associada à cardiopatia congênita em adultos envolve um amplo espectro de situações clínicas complexas e de difícil manejo para o cardiologista clínico. Revisão: serão revistos os principais aspectos fisiopatológicos relacionados ao desenvolvimento da hipertensão pulmonar nos diferentes grupos de cardiopatias congênitas, bem como os critérios para estabelecer o diagnóstico. Serão discutidos o tratamento guiado pelo perfil de risco e os grupos farmacológicos disponíveis em nosso meio. Por fim, serão discutidos grupos especiais como síndrome de Eisenmenger e gestantes com cardiopatia e hipertensão pulmonar. Conclusão: de qualquer forma, é importante ter em mente que a hipertensão pulmonar nesse cenário pode ter um prognóstico ruim (como na síndrome de Eisenmenger), razão pela qual todo esforço deve ser feito para o diagnóstico precoce e tratamento oportuno desses pacientes.

Asociación del complejo ring-sling con pig bronchus: a propósito de un caso pediátrico / Ring-sling complex association with pig bronchus: a pediatric case report

El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

Aumento do trabalho miocárdico desperdiçado como indicador de lesão coronariana significativa / Increased wasted myocardial work as an indicator of significant coronary lesion

Introdução: A avaliação dos índices de trabalho miocárdico global em condições basais pode ser útil para a estratificação clínica de pacientes com suspeita de obstrução coronariana. Objetivo: Correlacionar o valor do índice de trabalho miocárdico global e a presença de lesões obstrutivas coronarianas significativas. Método: Estudo transversal, com pacientes encaminhados para cinecoronarioangiografia eletiva. Foi realizado ecocardiograma com obtenção das medidas para cálculo do valor do trabalho miocárdico, sendo avaliada a presença de lesões obstrutivas coronarianas significativas à cinecoronarioangiografia. Resultados: A amostra foi composta de 30 pacientes, com a idade média de 64,2±12,8 anos, sendo a maioria do sexo masculino (63,3%), dos quais 68,4% apresentaram lesões obstrutivas coronarianas significativas. O índice de trabalho miocárdico global foi de 1.876mmHg%±253,8 no grupo com lesões obstrutivas coronarianas significativas e de 2.054,2mmHg%±417,3 naqueles sem lesões significativas (p=0,089). O trabalho miocárdio construtivo global nos pacientes sem lesões obstrutivas coronarianas significativas foi maior (2.329,3mmHg%±462,9) do que naqueles com lesões obstrutivas coronarianas significativas (2.109,5mmHg%±332,3; p=0,064). O trabalho miocárdio desperdiçado global foi maior nos pacientes com lesões obstrutivas coronarianas significativas (103,7mmHg%±47,1 versus 68,3mmHg%±33,8; p=0,038). O ponto de corte de 115mmHg% foi aquele com a melhor área sob a curva (0,625), com sensibilidade de 83,3%. Conclusão: O aumento do trabalho miocárdio desperdiçado global se correlacionou com a presença de lesões obstrutivas coronarianas significativas em nossa amostra.(AU)

Introduction: The assessment of global myocardial work indices under baseline conditions may be useful for the clinical stratification of patients with suspected coronary obstruction. Objective: To correlate the value of global myocardial work indices and the presence of significant obstructive coronary lesions. Method: Cross-sectional study, with patients referred for elective coronary angiography. An echocardiogram was performed to obtain measurements to calculate the value of myocardial work and evaluated the presence or presence of significant obstructive coronary lesions at coronary angiography. Results: The sample consisted of 30 patients, with a mean age of 64.2±12.8 years, the majority being male (63.3%), of which 68.4% had significant obstructive coronary lesions. The global myocardial work indices was 1,876mmHg%±253.8 in the group with significant obstructive coronary lesions and 2,054.2mmHg%±417.3 in those without significant lesions (p=0.089). Global constructive myocardial work in patients without significant obstructive coronary lesions was higher (2,329.3mmHg%±462.9) than in those with significant obstructive coronary lesions (2,109.5mmHg%±332.3; p=0.064). Global wasted myocardial work was higher in patients with significant obstructive coronary lesions (103.7mmHg%±47.1 versus 68.3mmHg%±33.8; p=0.038). The cutoff point of 115 mmHg% was the one with the best area under the curve (0.625), with a sensitivity of 83.3%. Conclusion: The increase in global wasted myocardial work correlated with the presence of significant obstructive coronary lesions in our sample. (AU)

Impact of composite clinical worsening events on outcome of patients with pulmonary arterial hypertension associated with congenital heart disease / 中华心血管病杂志

Objective: To explore the impact of composite clinical worsening (cCW) events and its components on the prognosis of patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH). Methods: This is a retrospective study. Patients who were diagnosed with CHD-PAH in Beijing Anzhen Hospital between January 2007 and July 2018, were included, and their baseline clinical data including demographic, clinical manifestations and New York Heart Association (NYHA) classification were collected retrospectively. All-cause deaths and clinical worsening events were recorded, which included syncope, PAH related hospitalization, NYHA classification deterioration and ≥ 2 PAH related clinical symptoms (dyspnea, hemoptysis, edema, chest pain, palpitations, cyanosis) appearance/progress. Three kinds of cCW events were defined: cCW1 (included PAH related hospitalization, NYHA classification deterioration), cCW2 (increased syncope on the basis of cCW1) and cCW3 (increased ≥ 2 PAH related clinical symptoms appearance/progress on the basis of cCW2). The Kaplan-Meier survival curve was used to analyze the long-term survival of the included patients. Univariate and multivariate Cox regression models were used to evaluate the impact of cCW events and their components on the risk of all-cause mortality. Results: A total of 525 patients with CHD-PAH were included in this study. The median age at diagnosis was 20.7 (11.2, 30.3) years. There were 43.8% children (<18 years), and 68.8% female patients. There were 431 patients (82.1%) with NYHA classification II. A total of 180 patients had PAH symptoms at diagnosis. The median follow-up time was 4.5 (2.6, 6.7) years. Forty-seven patients (9.0%) died during the follow-up period. Survival rates at 1, 5 and 10 years after diagnosis of PAH were 98.0%, 89.9% and 84.4%, respectively. Cox multivariate analysis showed that NYHA classification deterioration (HR=3.901, 95%CI 1.863-8.169, P<0.001), ≥2 PAH symptoms appearance/progress (HR=4.458, 95%CI 1.870-10.625, P<0.001), PAH-related hospitalization (HR=4.058, 95%CI 1.851-8.896, P<0.001) and syncope (HR=11.313, 95%CI 4.860-26.332, P<0.001) were independent predictors of increased risk of death. All 3 kinds of cCW events were significantly associated with the significantly increased risk of death, and cCW2 was highly predictive to increased risk of death (HR=15.476, 95%CI 4.346-37.576, P<0.001). Conclusions: The overall long-term prognosis of CHD-PAH patients in this study is relatively good. cCW events and its components (NYHA classification deterioration, ≥2 PAH symptoms occurrence/worsening, PAH-related hospitalization and syncope) have adverse influence on all-cause death in this patient cohort.

Clinical factors affecting the early postoperative prognosis of infants suffering surgery for congenital heart diseases: A single-center experience of 511 patients / 中南大学学报(医学版)

OBJECTIVES@#The integrated model of prenatal diagnosis and postnatal treatment for congenital heart disease (CHD) leads to an increasing number of operation in infants. This study aims to reveal the risk factors for postoperative early mortality and delayed recovery in infants less than 3 months old, who underwent surgical treatment for CHD in the Department of Cardiovascular Surgery, Second Xiangya Hospital, Central South University during the past 5 years.@*METHODS@#Clinical variables were collected via medical records. Delayed recovery was defined as the time of postoperative intubation, or cardiac intensive cure unit (CICU) stay, or hospital stay longer than its third quartile. Risk factors for early postoperative prognosis and the odds ratio (OR) were analyzed with logistic regression analysis.@*RESULTS@#A total of 511 infants underwent surgical treatment for CHD from January 2016 to June 2020 were retrospectively reviewed, including 217 (42.5%) infants with complex CHD. The median age was 60 days (3 hours-90 days); and median weight was 4.5 (1.7- 8.4 kg). There were 26 postoperative mortalities, making the incidence at 5.1%, including 5 (5/294, 0.7%) mortalities in patients with uncomplicated CHD, and 21 (9.6%) mortalities in patients with complex CHD. Based on multivariable analysis, risk factors for postoperative mortality were diagnosis of complex CHD (OR=5.53, P<0.001), weight under 4.0 kg (OR=9.86, P<0.001), preoperative symptoms (OR=3.17, P=0.012), and emergency operation (OR=11.66, P<0.001). The median time for postoperative intubation, CICU stay, and hospital stay were 21.0 (0.3-979.0) hours, 3.0 (0.5-91.0) days, and 11.5 (3.0-105.0) days, respectively. A total of 177 (34.6%) infants delayed recover, with risk factors including diagnosis of complex CHD (OR=3.41, P=0.001), weight under 4.0 kg (OR=4.55, P<0.001), and preoperative symptoms (OR=3.91, P<0.001).@*CONCLUSIONS@#Surgical treatment for infants (<3 months) with CHD is still a challenge, particularly for infants with complex CHD and weight under 4.0 kg. We can improve the prognosis of CHD treatment in infants by establishing the integrated model of prenatal diagnosis and postnatal treatment to choose the most suitable time window, avoid symptoms before surgery, and reduce emergency operation.

Congenital Heart Disesase: A Retrospective Analysis from a Tertiary Referral Centre in Portugal

Abstract Background: Congenital heart disease is the leading cause of mortality among all congenital malformations. Objectives: To evaluate the incidence of congenital heart diseases in a central maternity hospital in Portugal from January 2003 to December 2018 and to determine survival in the first year of life. Methods: Retrospective analysis of newborns diagnosed with congenital heart diseases within 72 hours after birth. Malformations were divided according to pathophysiology. Cumulative survival analysis was performed by the Kaplan-Meier test. Stastical significance was set at p <0.05. Results: A total of 297 newborns with cardiac malformation was recorded among 47,198 live births (incidence of 6:1000), 16% associated with extra-cardiac disease. The most frequent congenital heart diseases were left-to-right shunt lesions (n = 216), followed by cyanotic (n = 41), acyanotic obstructive (n = 31) and miscellaneous (n = 9). Seventy (24%) patients had prenatal diagnosis, 88% of them cyanotic defects, and a positive association was found between prenatal diagnosis and mortality (p <0.001). Coarctation of the aorta was associated with gestational diabetes (p = 0.014). Atrial septal defect was more common in females (p = 0.02). Mortality rate due to heart disease was 3.4%. Patients with cyanotic disease, 99%, 97%, 97%, respectively, for patients with left-to-right shunt lesions, and 97%, 97%,97% for those with obstructive lesion cases. Conclusion: The incidence of congenital heart disease was 6:1000, mostly left-to-right shunt lesions. Heart disease accounted for only half of deaths, and cyanotic diseases have a high nonspecific mortality rate.

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

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

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

Cardiopatías congénitas, tratamiento quirúrgico y sus complicaciones en población pediátrica del Hospital Vicente Corral Moscoso, 2017 - Agosto 2019 / Congenital heart diseases, surgical treatment and complications in pediatric sample of Hospital Vicente Corral Moscoso, 2017-August 2019.

INTRODUCCIÓN: El corazón es el órgano más comúnmente afectado por anormalidades congénitas, con una incidencia de 0.8 por cada 100 nacidos vivos. Cerca de dos tercios de todos los procedimientos son en la actualidad realizados antes del año de edad, lo que mejora la sobrevida y la calidad de vida. Este estudio busca determinar cuáles son las principales intervenciones quirúrgicas realizadas para tratar las cardiopatías congénitas y sus complicaciones. MATERIALES Y MÉTODOS: Se realizó un estudio observacional, descriptivo de corte transversal; con 70 pacientes pediátricos diagnosticados y tratados quirúrgicamente por cardiopatías congénitas. Los datos fueron tomados de las historias clínicas mediante un formulario. El análisis estadístico se realizó utilizando el programa SPSS versión 15. RESULTADOS: La mediana de la edad fue de 1.1 años, el 60% fueron de sexo femenino. El 90% de las cardiopatías fueron no cianógenas. El diagnóstico más frecuente fue la persistencia del conducto arterioso (58.57%), seguido de la comunicación interventricular (12.86%). Según el tipo de procedimiento el 58.57% se realizaron para cierre de persistencia del conducto arterioso y un 12.86% fueron reparaciones quirúrgicas para cierre de comunicación interventricular. La mediana de estadía en la unidad de cuidados intensivos fue de 4 días y la mediada de estadía en la sala general de 5 días. La principales complicaciones observadas en esta población pediátrica sometida a un procedimiento quirúrgico fueron: la neumonía (11.4%) y la sepsis de origen no especificado (8.6%). CONCLUSIÓN: Los tratamientos para las cardiopatías congénitas se realizaron a edades tempranas (Media =2.5 ± 3.2 años). Más de la mitad de los procedimientos quirúrgicos realizados para cardiopatías quirúrgicas fueron para corregir la persistencia del conducto arterioso y la principal complicación fue la neumonía.

BACKGROUND: The heart is the most commonly affected organ by congenital diseases, with and incidence of 0.8 per 100 newborns. Nearly two thirds of all the surgical procedures are now a days performed before the first year of life, improving survival rate and life quality. This study aims to determine the frequency of the surgical interventions performed to treat congenital heart diseases and its complications. METHODS: An observational, descriptive cross sectional study was carried out; with 70 pediatric patients diagnosed and surgically treated for congenital heart diseases. The data was collected from the patient's medical records using a form. Statistical analysis was performed using SPSS version 15 software. RESULTS: The median age was 1.1 years, 60% of the sample were women. 90% of the heart diseases were non-cyanogenic. The most frequent diagnosis was: persistence of the arterial duct (58.57%), followed by interventricular communication (12.86%). The type of procedures corresponds to the heart disease, thus 58.57% were performed for closure of arterial duct persistence and 12.86% were surgical repairs for closure of interventricular communication. The median stay in the intensive care unit was 4 days and the median stay in general hospitalization room was 5 days. The main complications in this pediatric population undergoing a surgical procedure were: pneumonia (11.4%) and sepsis of unspecified origin (8.6%). CONCLUSION: Treatment for heart diseases were performed at early ages (average age= 2.5±3.2). More than half of the surgical procedures for congenital heart disease were performed to correct the persistence of the ductus arteriosus, the main complication was pneumonia.

Avaliação da deglutição e indicadores clínicos associados em crianças após correção cirúrgica de doença cardíaca congênita / Evaluation of swallowing and associated clinical markers in infants after surgical repair of congenital heart disease

RESUMO Objetivo Identificar os marcadores clínicos associados às alterações da deglutição em crianças após a correção da doença cardíaca congênita e correlacionar esses marcadores às categorias da escala Risk Adjustment for Congenital Heart Surgery 1 (RACHS-1). Métodos Foi realizado um estudo retrospectivo observacional, utilizando a base de dados eletrônica do centro de pediatria para crianças admitidas em um hospital, em decorrência de doença cardíaca congênita. Foram coletados dados da avaliação de deglutição e a presença de disfagia foi classificada de acordo com a Dysphagia Management Staging Scale (DMMS). Foram incluídos os dados demográficos, os marcadores clínicos e o risco de mortalidade, de acordo com a RACHS-1. Resultados A amostra final do estudo foi composta por 108 pacientes. Os achados mais relevantes foram: os sinais mais graves de disfagia estiveram associados ao aumento da permanência no hospital (p=0,005); ao maior número e tempo de intubações orotraqueais (p=0,022 e 0,005, respectivamente); ao maior tempo entre a admissão hospitalar e a avaliação da deglutição (p=0,003); ao maior tempo entre o procedimento cirúrgico e a avaliação da deglutição (p=0,043) e ao maior número de sessões fonoaudiológicas para remoção da via alternativa de alimentação e retorno seguro para via oral (p<0,001). Não foram encontradas correlações entre o risco de mortalidade das crianças e os sinais alterados na avaliação da deglutição. Conclusão Os resultados do estudo contribuem para o conhecimento atual de que crianças com doenças cardíacas que precisam de correção cirúrgica apresentam dificuldades alimentares e necessitam atendimento hospitalar prolongado. Não houve correlação entre as categorias da escala RACHS-1 e os sinais de alteração na avaliação clínica da deglutição.

ABSTRACT Purpose To identify clinical markers that are associated to swallowing alterations in infants after surgical repair of congenital heart disease (CHD) and to correlate these markers to the categories on the Risk Adjustment for Congenital Heart Disease 1 (RACHS-1) scale. Methods Using the Pediatric Center Database System we conducted a retrospective, observational cohort study on children admitted to the pediatric hospital unit due to CDH. We collected data on specific parameters of a clinical swallowing assessment (SA) and dysphagia was classified according to the Dysphagia Management Staging Scale. We also included demographic and clinical markers and patient´s risk of mortality was determined by using the RACHS-1. Results The final study sample consisted of 108 patients. Important findings were: the more severe signs of dysphagia are associated to an increased length of hospital stay (p=0.005); an increased number and duration of orotracheal intubation (p=0.022 and 0.005 respectively); an increased time between hospital admission and SA (p=0.003); an increased time between the surgical procedure and swallowing assessment (0.043); and an increased number of SLP sessions to remove alternate feeding methods and warrant safe oral feeding (p<0.001). No correlations were observed between the infant's risk of mortality and the altered sings on the clinical swallowing assessment. Conclusion The data from this study contributes to the current knowledge that children with heart condition requiring heart surgery in the first month of life have high risk of presenting feeding difficulties and will require prolonged hospital care. No correlation was observed between the categories on RACHS-1 and the altered signs on the clinical swallowing assessment.

Primary peritoneal drainage in neonates with necrotizing enterocolitis associated with congenital heart disease: a single experience in a Brazilian tertiary center

Necrotizing enterocolitis (NEC) is a common condition in preterm infants. The risk factors that contribute to NEC include asphyxia, apnea, hypotension, sepsis, and congenital heart diseases (CHD). The objective of this study was to evaluate the association between the treatment (surgery or drainage) and unfavorable outcomes in neonates with NEC and congenital heart diseases (NEC+CHD). A 19-year retrospective cohort study was conducted (2000-2019). Inclusion criterion was NEC Bell II stage. Exclusion criteria were associated malformation or genetic syndrome and those who did not undergo echocardiography or had a Bell I diagnosis. We included 100 neonates: NEC (n=52) and NEC+CHD (n=48). The groups were subdivided into NEC patients undergoing surgery (NECS, n=31), NEC patients undergoing peritoneal drainage (NECD, n=19), NEC+CHD patients undergoing surgery (NECCAS, n=21), and NEC+CHD patients who were drained (NECCAD, n=29). Multivariate analysis was performed to estimate the relative risk of death and the length of stay. Covariates were birth weight and gestational age. The group characteristics were similar. The adjusted relative risk of death was higher in the drainage groups [NECD (Adj RR=2.70 (95%CI: 1.47; 4.97) and NECCAD (Adj RR=1.97 (95%CI: 1.08; 3.61)], and they had the shortest time to death: NECD=8.72 (95%CI: 3.10; 24.54) and NECCAD=5.32 (95%CI: 1.95; 14.44). We concluded that performing primary peritoneal drainage in neonates with or without CHD did not improve the number of days of life, did not decrease the risk of death, and was associated with a higher mortality in newborns with NEC and clinical instability.

Insuficiencia Mitral Severa secundaria a "Cleft" Mitral Aislado: revisión a partir de un caso clínico / Severe Mitral Insufficiency secondary to Isolated Mitral Cleft: Review from a clinical case

Resumen El "cleft" mitral es un defecto congénito raro que se encuentra comúnmente asociado a otras patologías no obstante, en raras ocasiones puede encontrarse como un defecto aislado. Dicho hallazgo es de suma importancia desde el punto de vista clínico ya que es una causa reparable de insuficiencia mitral, sin necesidad de someterse a reemplazo valvular. Su diagnóstico es primariamente mediante ecocardiografía, en la que el uso del 3D como técnica permite la correcta delimitación de la extensión de la lesión, así como la valoración a detalle del resto del aparato valvular mitral. Presentamos un caso de Insuficiencia Mitral severa en la infancia de causa no clara en un inicio, en la cual, por medio de ecocardiografía transtorácica 3D se logró identificar una hendidura aislada de la valva anterior como etiología y descartar otras posibles etiologías y/o mecanismos.

Abstract Severe Mitral Insufficiency secondary to Isolated Mitral Cleft: Review from a clinical case Severe mitral valve reguritacion secondary to an Isolated cleft of the mitral valve: a review based on a clinical case Mitral Cleft is a rare congenital defect that is commonly associated with others pathologies; nevertheless, in rare occasions it can be found as an isolated defect. This finding is paramount from clinical perspective as it is a reparable defect and not necessarily ends in valvular replacement. Its assessment is primarily by transthoracic echocardiography with the addition of 3D technology that provides a more detailed delimitation of the extent of the lesion, as well as detailed assessment of the whole mitral valve apparatus. In this article, we present a case of severe mitral regurgitation without a clear ethiology at glance, but posteriorly identified with the aid of transthoracic 3D echocardiography, as an isolated mitral cleft and also, allowed to rule out other possible etiologies and mechanisms.

Hernia diafragmática congénita: predictores posnatales de mortalidad / Congenital diaphragmatic hernia: postnatal predictors of mortality

La hernia diafragmática congénita (HDC) es una enfermedad de baja prevalencia, con elevada morbimortalidad. Los factores pronósticos posnatales, durante el primer día de vida, son útiles para la toma de decisiones.Objetivos. Determinar la capacidad predictiva de los factores posnatales ecocardiográficos, clínicos y bioquímicos de mortalidad en los recién nacidos con HDC durante el primer día de vida.Método. Estudio observacional analítico de cohorte retrospectiva. Se incluyeron los pacientes con HDC, en forma consecutiva, desde marzo de 2012 a noviembre de 2018. Se analizaron como predictores el índice de oxigenación (IO), valor más alto de presión parcial de dióxido de carbono en sangre (pCO2), puntaje de gravedad SNAPPE II, ecocardiograma, dosaje de NT-pro péptido natriurético B (NT-proPNB), todos medidos en el primer día de vida.Resultados. La población fue de 178 pacientes con HDC. La sobrevida, del 75 %. El 24 % recibió oxigenación por membrana extracorpórea. La presencia precoz de hipertensión pulmonar sistémica o suprasistémica no mostró capacidad predictiva (OR 2,2; IC 95 %: 0,8-8), p = 0,1. NT-proPNB tampoco mostró buena discriminación (área bajo la curva (ABC) 0,46, p = 0,67). El IO, SNAPPE II y el valor más alto de pCO2 mostraron buena discriminación, ABC IO 0,82, ABC SNAPPE II 0,86 y ABC pCO2 0,75, p < 0,001.Conclusión.SNAPPE II, IO y valor más alto de pCO2, medidos el primer día de vida, mostraron buena capacidad predictiva con respecto a la evolución; SNAPPE II fue superior al IO y al valor más alto de CO2.

Introduction: Congenital diaphragmatic hernia (CDH) prevalence is low while its associated morbidity and mortality rates are high. Postnatal prognostic factors on the first day of life are useful for predicting the outcome. Objectives: To determine the mortality predictive ability of postnatal echocardiographic, clinical, and biochemical factors among newborn infants with CDH in their first day of life. Method: Observational analytical study of a retrospective cohort. Patients with CDH were consecutively included between March 2012 and November 2018. On the first day of life, analyzed predictors were the oxygenation index (OI), the highest partial pressure of carbon dioxide (pCO2) level in blood, the SNAPPE II severity score, the echocardiography, and the N-terminal pro-B-type natriuretic peptide (NTproBNP) value. Results: The population consisted of 178 patients with CDH. Survival was 75 %. Extracorporeal membrane oxygenation was used in 24 %. The early onset of systemic or suprasystemic pulmonary hypertension showed no predictive ability (OR: 2.2, 95 % CI: 0.8-8), p = 0.1. NT-proBNP did not show good discrimination either (area under the curve [AUC]: 0.46, p = 0.67). The OI, SNAPPE II score, and the highest pCO2 level showed adequate discrimination power, AUC for OI: 0.82, AUC for SNAPPE II: 0.86, and AUC for pCO2: 0.75, p < 0.001. Conclusion: The SNAPPE II score, the OI, and the highest pCO2 level measured on the first day of life, showed a good predictive ability in terms of the course of the disease; the SNAPPE II score was better than the OI and the highest pCO2 level.

Coartación de aorta / Coarctation of the Aorta

Resumen La coartación de aorta es una cardiopatía congénita con altas tasas de morbilidad y mortalidad, que usualmente es subdiagnosticada a pesar de la disponibilidad de herramientas diagnósticas. El grado de severidad de las manifestaciones clínicas de la coartación de aorta va a depender del grado de obstrucción, así como de la presencia de defectos cardiacos y lesiones extracardíacas asociados. En la población pediátrica la modalidad terapéutica mayormente utilizada es la reparación quirúrgica; mientras que la angioplastia con balón y la colocación de una endoprótesis son menos utilizadas en esta población, ya que asocian mayor riesgo de reestenosis con la consecuente reintervención, estas técnicas son principalmente utilizadas en pacientes mayores. A pesar del éxito en la reparación de la coartación de aorta, los pacientes deben continuar un seguimiento estrecho a largo plazo, que incluye mediciones de la presión arterial de manera periódica, así como estudios por imagen de la estructura cardíaca, debido a la aparición tardía de complicaciones cardiovasculares asociadas.

Abstract Coarctation of the aorta is a congenital heart disease with high rates of morbidity and mortality, which is usually underdiagnosed despite the availability of diagnostic tests. The degree of severity of the clinical manifestations of coarctation of the aorta will depend on the level of obstruction, as well as the presence of cardiac defects and associated extracardiac lesions. In the pediatric population the most used therapeutic modality is surgical correction; while balloon angioplasty and stent placement are less used in this population, since they are associated with a greater risk of restenosis with the subsequent reintervention; these techniques are mostly used in older patients. Despite the success in the repair of coarctation of the aorta, patients should continue a close long-term follow-up, which includes periodic blood pressure measurements, as well as imaging studies that assess the cardiac structure, due to the late onset of associated cardiovascular complications.

Influência da cardiopatia congênita no desenvolvimento neuropsicomotor de lactentes / Influencia de la cardiopatía congénita en el desarrollo neuropsicomotor de los lactantes / Influence of congenital heart disease on the neuropsychomotor development of infants

RESUMO As cardiopatias congênitas (CC) estão entre as principais causas de morbimortalidade na primeira infância e os lactentes com essa condição podem apresentar atrasos no desenvolvimento neuropsicomotor (DNPM). O objetivo deste estudo foi avaliar a influência da CC no DNPM de lactentes. Trata-se de um estudo observacional com avaliação do desenvolvimento neuropsicomotor realizada pela Bayley Scales of Infant and Toddler Development (BSID-III). As condições maternas e clínicas dos lactentes foram verificadas no relatório de alta médica e na caderneta de saúde da criança, e a condição socioeconômica das famílias pelo Critério da Classificação Econômica Brasil. Para associar as variáveis clínicas e o DNPM foram utilizados o coeficiente de correlação de Spearman e o teste de razão de verossimilhança. Foram avaliados 18 lactentes, com predomínio do sexo feminino (72,2%). A maioria das mães (47,1%) possuía ensino médio completo ou superior incompleto, com média da idade de 27,2±5,5 anos. Houve correlação das escalas do BSID-III com as variáveis quantitativas analisadas: escala motora com o peso (p=0,02 e r=0,54) e com uso de oxigenoterapia (p=0,009 e r=−0,591); já para as variáveis qualitativas as associações foram entre: escala motora e condição socioeconômica (p=0,015), escala motora e comunicação interatrial - (CIA) (p=0,023) e escala da linguagem e CIA (p=0,038). A CC influenciou o DNPM, principalmente no aspecto motor. Além disso peso, diagnóstico de CIA, uso de oxigenoterapia e condição socioeconômica foram considerados como principais fatores de risco para o atraso no DNPM.

RESUMEN Las cardiopatías congénitas (CC) se encuentran entre las principales causas de morbimortalidad en la primera infancia, y los lactantes con esta afección pueden tener retrasos en el desarrollo neuropsicomotor (DNPM). El presente estudio tuvo el objetivo de evaluar la influencia de las CC en el DNPM de los lactantes. Este es un estudio observacional en el cual se evaluó el desarrollo neuropsicomotor utilizando la Bayley scales of infant and toddler development (BSID-III). Las condiciones maternas y clínicas de los lactantes se obtuvieron en el informe de alta médica y en la libreta de salud del niño, y el estado socioeconómico de las familias en el Criterio de Clasificación Económica de Brasil. Para asociar las variables clínicas y el DNPM, se utilizaron el coeficiente de correlación de Spearman y la prueba de razón de probabilidad. Se evaluaron a 18 lactantes, con un predominio del sexo femenino (72,2%). La mayoría de las madres (47,1%) tenían la secundaria completa o la educación superior incompleta, con una edad promedio de 27,2±5,5 años. Hubo una correlación entre las escalas BSID-III y las variables cuantitativas analizadas: escala motora con el peso (p=0,02 y r=0,54) y con el uso de oxigenoterapia (p=0,009 y r=−0,591); para las variables cualitativas, las asociaciones fueron entre: escala motora y estado socioeconómico (p=0,015), escala motora y comunicación interauricular (CIA) (p=0,023) y escala de lenguaje y CIA (p=0,038). Las CC influyeron en el DNPM, principalmente en el aspecto motor. Además, el peso, el diagnóstico de CIA, el uso de oxigenoterapia y el estado socioeconómico fueron considerados los principales factores de riesgo para el retraso en el DNPM.

ABSTRACT Congenital heart defects (CHD) are among the main causes of morbidity and mortality in infants who has this impairment may present delays in neuropsychomotor development (NPMD). This study assesses the influence of CHD on NPMD of infants. This is an observational study assessing neuropsychomotor development performed by Bayley Scales of Infant and Toddler Development - BSID-III. The Brazilian Economic Classification Criteria was used to verify the socioeconomic status of the families and also the maternal and infants' clinical conditions were verified in the medical discharge report and in the child's health handbook. For the association between the quantitative and qualitative variables with the NPMD, the Spearman's correlation coefficient and the likelihood ratio test were used. A total of 18 infants were assessed, with a predominance of females (72.2%). Most mothers (47.1%) had complete high school or incomplete higher education, with a mean age of 27.2±5.5 years. There was a correlation between the BSID-III scales and the quantitative variables analyzed: motor scale with weight (p=0.02 and r=0.54) and oxygen therapy (p=0.009 and r=−0.591); besides that, the qualitative variables correlation were: motor scale and socioeconomic condition (p=0.015), motor scale and Interatrial Communication - IAC (p=0.023) and language with IAC scales (p=0.038). CHD influences the delay of NPMD, mainly for motor aspect. Furthermore, weight, diagnosis of IAC, use of oxygen therapy and socioeconomic status were considered the main risk factors for the delay in NPMD.

Cardiopatías congénitas y desarrollo del cerebro fetal artículo de revisión / Congenital heart disease and fetal brain development: review

La enfermedad cardíaca congénita se ha asociado con alteraciones del neurodesarrollo. Niños y adolescentes con cardiopatía congénita presentan déficits en distintos dominios neurológicos (motor, cognitivo, lenguaje, rendimiento escolar y habilidades sociales). Históricamente se atribuyeron a una potencial injuria cerebral durante la cirugía cardíaca. Sin embargo, evidencia actual demuestra que RN de término ya presentan anormalidades cerebrales y retraso de la maduración (dismaduración) con alta prevalencia 28% (IC 95%, 18-40%) Entre ellas se reporta ventriculomegalia, agenesia cuerpo calloso, hipoplasia de vermis, hemorragia intraventricular, aumento espacio subaracnoídeo, alteraciones de la sustancia blanca (Leucomalacia periventricular), desarrollo cortical alterado y quistes periventriculares. El mecanismo más estudiado es la interferencia de la cardiopatía con el shunt fisiológico de derecha a izquierda por el foramen ovale, que asegura la llegada de la sangre mejor oxigenada primeramente al cerebro. Se discute el beneficio de potenciales intervenciones prenatales neuroprotectoras y el diagnóstico temprano de cardiopatías congénitas.

Congenital heart disease has been associated with neurodevelopmental disorders. Children and adolescents with congenital heart disease present deficits in different neurological domains (motor, cognitive, language, school performance and social skills). Historically they were attributed to a potential brain injury during cardiac surgery. However, current evidence shows that term newborns already present brain abnormalities and delay of maturation (dysmaturation) with high prevalence 28% (95% CI, 18-40%) Among them is reported ventriculomegaly, Corpus Callosum agenesis, hypoplastic vermis, intraventricular hemorrhage, subarachnoid space enlargement, white matter alterations (periventricular leukomalacia), altered cortical development and periventricular cysts. The most studied mechanism is the interference of heart disease with the physiological shunt from right to left by the foramen ovale, which ensures the arrival of the best oxygenated blood first to the brain. The benefit of potential neuroprotective prenatal interventions and early diagnosis of congenital heart diseases is discussed.