Síndrome de Eisenmenger secundario a comunicación interatrial tipo ostium secundum en mujer adulta / Eisenmenger Syndrome Associated with an ostium secundum Atrial Septal Defect in an Adult Woman

El síndrome de Eisenmenger es la forma más severa de presentación de hipertensión arterial pulmonar secundaria a defectos cardíacos congénitos no reparados, aunque su prevalencia es baja, continúa siendo un reto para los sistemas de salud de los países en vías de desarrollo por su complejidad en el manejo. Presentación del caso. Paciente femenina sin antecedentes médicos conocidos quien consulta por disnea relacionada a los esfuerzos y policitemia. Intervención terapéutica. Se realiza ecocardiograma transesofágico que arroja la presencia de defecto interatrial tipo ostium secundum e hipertensión arterial pulmonar severa, con cortocircuito de derecha a izquierda, se inicia oxigenoterapia y terapia farmacológica. Evolución clínica. Paciente permaneció ingresada presentando notable mejora a la disnea, se le dio de alta con referencia a la clínica de cardiopatías congénitas del adulto en Hospital Nacional Rosales.

Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension secondary to an unrepaired congenital heart disease. Despite the low prevalence, it remains a challenge for the public health service of developing countries due to the complexity of the treatment. Case presentation. A female patient without known medical history, who consults with dyspnea on exertion and polycythemia. Treatment. A transesophageal echocardiogram was performed, showing an ostium secundum atrial septal defect and severe pulmonary arterial hypertension with a right-left shunt. Supplemental oxygen was administrated and pharmacological treatment was started. Outcome. The patient presented remarkable clinical improvement to dyspnea, she was discharged with medical reference to the Adult Congenital Heart Disease clinic at Rosales National Hospital.

Prevalence of Critical Congenital Heart Disease Detected in the Pulse Oximetry Test in Asymptomatic Newborns, ≥35 Gestational Weeks, in a Maternity in Southern Brazil

Abstract Background Congenital heart diseases (CHD) are one of the most prevalent malformations, and the screening tests to identify critical congenital heart disease (CCHD) is the pulse oximetry test, with subsequent investigation and treatment. Objective To quantify positive pulse oximetry tests and verify the prevalence of CCHD detected by it in asymptomatic newborns, ≥35 gestational weeks, in a Brazilian maternity hospital. Methodology This is an observational, retrospective, quantitative, analytical and cross-sectional study, conducted from October 2020 to May 2022, in a maternity hospital in southern Brazil, through the collection of records of positive oximetry pulse tests, following the norms of screening CCHD test of the Brazilian Society of Pediatrics (SBP), after they were evaluated with echocardiography for confirmation or exclusion of CHD. Results A total of 5,667 newborns were evaluated in this study, according to the inclusion criteria; 0.17% (n = 10) had a positive pulse oximetry test. Regarding the results of the echocardiography of the neonates with a positive test, two were normal, seven were cases of patent foramen ovale (PFO), and one was a case (0,017%) with interatrial communication (IAC) diagnosis. In the same period, five neonates with CCHD were born, symptomatic, diagnosed by physical examination, and referred to neonatal intensive care units (NICU) before taking the neonatal cardiac screening (< 24 hours of life). Conclusion The prevalence of positive pulse oximetry tests was 0.17% and none CCHD was detected. Five cases of CCHD were born in this period, but they were diagnosed before the recommend time to perform the screening test.

Patients' prognosis with congenital heart disease followed by ten years: survival and associated factors / Prognóstico de pacientes com cardiopatia congênita seguidos por dez anos: sobrevida e fatores associados

ABSTRACT Objective: To evaluate the prognosis and influence of associated factors in patients with congenital heart disease admitted for the first time to the Intensive Care Unit of the Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, especially those factors associated with death. Methods: Patients were prospectively and consecutively allocated over a period of one year (August 2005 to July 2006). Now, 15 years after the initial selection, we collected data from these patients in the database of the Cytogenetics Laboratory of the Universidade Federal de Ciências da Saúde de Porto Alegre and in the medical records of the hospital. Results: Of the 96 patients, 11 died and 85 were alive until 20 years old. Four patients died in the Intensive Care Unit. The survival probability up to 365 days of life was 95.8%. The survival assessment identified that the deaths occurred mainly before the patients completed one thousand days of life. We found that complex heart disease was independently associated with an odds ratio of 5.19 (95% confidence interval — CI:1.09-24.71; p=0.038) for death. Conclusions: Knowledge about the factors that interfere with the prognosis can be crucial in care practice planning, especially considering that congenital heart disease is an important cause of mortality in the first year of life.

RESUMO Objetivo: Avaliar o prognóstico e a influência de fatores associados em pacientes com cardiopatia congênita internados pela primeira vez na Unidade de Terapia Intensiva do Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, principalmente aqueles fatores associados ao óbito. Métodos: Os pacientes foram alocados prospectiva e consecutivamente por um período de um ano (agosto de 2005 a julho de 2006). Agora, 15 anos após a seleção inicial, coletamos dados desses pacientes no banco de dados do Laboratório de Citogenética da Universidade Federal de Ciências da Saúde de Porto Alegre e nos prontuários do hospital. Resultados: Dos 96 pacientes, 11 faleceram e 85 permaneceram vivos até completar 20 anos. Quatro pacientes morreram na Unidade de Terapia Intensiva. A probabilidade de sobrevida até 365 dias de vida foi de 95,8%. A avaliação da sobrevida identificou que os óbitos ocorreram principalmente antes de os pacientes completarem mil dias de vida. Verificamos que a doença cardíaca complexa foi independentemente associada a um odds ratio de 5,19 (intervalo de confiança — IC95% 1,09-24,71; p=0,038) para morte. Conclusões: O conhecimento dos fatores que interferem no prognóstico pode ser fundamental no planejamento da prática assistencial, principalmente considerando-se que as cardiopatias congênitas são importante causa de mortalidade no primeiro ano de vida.

Lactate and Lactate Clearance Are Predictive Factors for Mortality in Patients with Extracorporeal Membrane Oxygenation

ABSTRACT Introduction: Findings of inadequate tissue perfusion might be used to predict the risk of mortality. In this study, we evaluated the effects of lactate and lactate clearance on mortality of patients who had undergone extracorporeal membrane oxygenation (ECMO). Methods: Patients younger than 18 years old and who needed venoarterial ECMO support after surgery for congenital heart defects, from July 2010 to January 2019, were retrospectively analyzed. Patients successfully weaned from ECMO constituted Group 1, and patients who could not be weaned from ECMO were in Group 2. Postoperative clinics and follow-ups of the groups including mortality and discharge rates were evaluated. Results: There were 1,844 congenital heart surgeries during the study period, and 55 patients that required ECMO support were included in the study. There was no statistically significant difference between the groups regarding demographics and operative variables. The sixth-, 12th-, and 24th-hour lactate levels in Group 1 were statistically significantly lower than those in Group 2 (P=0.046, P=0.024, and P<0.001, respectively). There were statistically significant differences regarding lactate clearance between the groups at the 24th hour (P=0.009). The cutoff point for lactate level was found as ≥ 2.9, with 74.07% sensitivity and 78.57% specificity (P<0.001). The cutoff point for lactate clearance was determined as 69.44%, with 59.26% sensitivity and 78.57% specificity (P=0.003). Conclusion: Prognostic predictive factors are important to initiate advanced treatment modalities in patients with ECMO support. In this condition, lactate and lactate clearance might be used as a predictive marker.

Patau and Edwards Syndromes in a University Hospital: beyond palliative care / Síndrome de Edwards e Patau em um hospital universitário: além do paliativo

ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

RESUMO Objetivo: Descrever a população de recém-nascidos com síndrome de Patau (T13) e Edwards (T18) portadores de cardiopatias congênitas, que permaneceram em Unidades de Terapia Intensiva (UTI) de um complexo hospitalar quaternário, com relação a conduta cirúrgica ou não, cuidados paliativos e seus desfechos. Métodos: Série de casos de pacientes internados entre janeiro de 2014 a dezembro de 2018, com análise dos prontuários de portadores de T13 ou T18 que permaneceram internados em UTI que recebem neonatos nesse hospital quaternário. Utilizou-se análise estatística descritiva. Resultados: Foram identificados 33 prontuários para análise — 27 T18 (81,8%) e seis T13 (18,2%); 64% do sexo feminino e 36% do sexo masculino. Oito foram prematuros, nascidos com 30 a 36 semanas (24,2%), e apenas quatro nasceram com mais de 2500 g (12,1%). Quatro pacientes foram submetidos a cirurgia cardíaca e um deles foi a óbito. A mortalidade intra-hospitalar foi de 83% para T13 e 59% para T18. A maioria apresentava outras malformações e foi submetida a outras cirurgias. Cuidados paliativos foram oferecidos a 54% dos pacientes. A mediana do tempo de hospitalização para T18 e T13 foi respectivamente de 29 dias (variação: 2-304) e 25 dias (13-58). Conclusões: Pacientes com T13 e T18 cursam com alta morbimortalidade e longa permanência hospitalar em UTI. São necessários estudos multicêntricos para melhor análise de aspectos importantes para a criação de protocolos que, buscando proporcionalidade terapêutica, tragam melhor qualidade de vida para os pacientes e suas famílias.

Fatores de Risco para Infecção da Ferida Operatória em Pacientes Submetidos à Cirurgia Cardíaca Pediátrica / Risk Factors for Surgical Site Infection in Patients Undergoing Pediatric Cardiac Surgery

Resumo Fundamento A infecção do sítio cirúrgico (ISC) é uma importante complicação no pós-operatório de cirurgia cardíaca pediátrica associada ao aumento da morbimortalidade. Objetivos Identificar fatores de risco para a ISC após cirurgias cardíacas para correção de malformações congênitas. Métodos Este estudo caso-controle incluiu 189 pacientes com um ano completo e 19 anos e 11 meses, submetidos à cirurgia cardíaca em hospital universitário terciário de cardiologia de janeiro de 2011 a dezembro de 2018. Foi realizado registro e análise de dados pré, intra e pós-operatórios. Para cada caso foram selecionados dois controles, conforme o diagnóstico da cardiopatia e cirurgia realizada em um intervalo de até 30 dias para minimizar diferenças pré e/ou intraoperatórias. Para a análise dos fatores de risco foi utilizado o modelo de regressão binária logística. Significância estatística definida como valor de p<0,05. Resultados O estudo incluiu 66 casos e 123 controles. A incidência de ISC variou de 2% a 3,8%. Fatores de risco identificados: faixa etária de lactentes (OR 3,19, IC 95% 1,26 - 8,66, p=0,014), síndrome genética (OR 6,20, IC 95% 1,70 - 21,65, p=0,004), RACHS-1 categorias 3 e 4 (OR 8,40, IC 95% 3,30 - 21,34, p<0,001), o valor da proteína C reativa (PCR) de 48 horas pós-operatórias foi demonstrado como fator protetor para esta infecção (OR 0,85, IC 95% 0,73 - 0,98, p=0,023). Conclusão Os fatores de risco identificados não são variáveis modificáveis. Vigilância e medidas preventivas contínuas são fundamentais para reduzir a infecção. O papel do PCR elevado no pós-operatório foi fator protetor e precisa ser melhor estudado.

Abstract Background Surgical site infection is an important complication after pediatric cardiac surgery, associated with increased morbidity and mortality. Objectives We sought to identify risk factors for surgical site infection after pediatric cardiac surgeries. Methods A case-control study included patients aged between 1 year and 19 years and 11 months of age, submitted to cardiac surgery performed at a tertiary cardiac center from January 1 st , 2011, through December 31, 2018. Charts were reviewed for pre-, intra, and postoperative variables. We identified two randomly selected control patients with the same pathophysiological diagnosis and underwent surgery within thirty days of each index case. Univariate and multivariate logistic regression analyses were performed to identify risk factors. Statistical significance was defined as p<0.05. Results Sixty-six cases and 123 controls were included. Surgical site infection incidence ranged from 2% to 3.8%. The following risk factors were identified: Infant age (OR 3.19, 95% CI 1.26 to 8.66, p=0.014), presence of genetic syndrome (OR 6.20, CI 95% 1.70 to 21.65, p=0.004), categories 3 and 4 of RACHS-1 (OR 8.40, CI 95% 3.30 to 21.34, p<0.001), 48 h C-reactive protein level range was detected as a protective factor for this infection (OR 0.85, 95% CI 0.73 to 0.98, p=0.023). Conclusions The risk factors defined in this study could not be modified. Therefore, additional surveillance and new preventive strategies need to be implemented to reduce the incidence of surgical site infection. The increased CRP in the postoperative period was a protective factor that needs further understanding.

Nursing guidelines for caregivers of children with congenital heart disease after discharge: Integrative Review / Directrices de enfermería para cuidadores de niños con cardiopatía congénita tras el alta: Revisión Integradora / Orientações de enfermagem para cuidadores de crianças com cardiopatia congênita após a alta: Revisão Integrativa

Objective. To identify the nursing guidelines for caregivers of children with congenital heart disease (CHD) after hospital discharge. Methods. This is an integrative literature review of articles published between 2016 and 2022. In order to select the studies, the controlled descriptors "Nursing Care", "Nursing", "Heart Defects, Congenital", "Caregivers" and "Child" were used in four scientific databases ­ LILACS, SCIELO, PUBMED and BDENF. Results. The current integrative literature review analyzed 11 articles from the original sample. The main nursing care issues are those related to nutrition, oral health, leisure and physical activity, care with medication and the surgical wound, as well as the need to offer support to these children's families. The authors emphasize that nurses are present at various moments in a child's life, including at birth, but the approach to CHD is scarce in Invest Educ Enferm. 2023; 41(3): e05Nursing guidelines for caregivers of children with congenitalheart disease after discharge: Integrative Reviewtheir basic training as nurses, as well as in their professional practice, and there is a shortage of continuing education proposals for the care of children with CHD. Conclusion. The study showed that nursing guidelines are focused on basic care and family support for these children. Lastly, this study highlighted the important role of nurses in terms of consolidating guidelines on the care needs of these children.

Objetivo. Identificar las orientaciones de enfermería para los cuidadores de niños con cardiopatía congénita (CC) después del alta hospitalaria. Métodos. Se trata de una revisión bibliográfica integradora de los artículos publicados entre 2016 a 2022. Se utilizaron los descriptores controlados "Nursing Care", "Nursing","Heart Defects, Congenital", "Caregivers" y "Child" para seleccionar los estudios en cuatro bases de datos científicas - LILACS, SCIELO, PUBMED y BDENF. Resultados. Se analizaron 11 artículos de la muestra original en esta revisión. Los principales tipos de cuidados de enfermería son los aspectos relacionados con la nutrición, la salud bucodental, el ocio y la actividad física, los cuidados con la medicación y la herida quirúrgica, así como la necesidad de ofrecer apoyo a las familias de estos niños. Los autores enfatizan que, aunque, las enfermeras están presentes en diversos momentos de la vida del niño, incluyendo el nacimiento, el abordaje de la ECC es escaso en su formación básica como enfermeras, así como en su práctica profesional, y faltan propuestas de formación continua en el cuidado de estos niños. Conclusión. El estudio mostró que las orientaciones de enfermería se centran en los cuidados básicos y en el apoyo familiar a niños con ECC. Por último, este estudio se evidenció el importante papel de las enfermeras en la consolidación de las orientaciones para las necesidades de atención de estos niños.

Objetivo. Identificar as orientações de enfermagem para cuidadores de crianças com cardiopatias congênitas (CC) após alta hospitalar. Métodos. Trata-se de uma revisão integrativa da literatura de artigos publicados entre 2016 e 2022. Para seleção dos estudos, foram utilizados os descritores controlados "Nursing Care", "Nursing", "Heart Defects, Congenital", "Caregivers"), y "Child" em duas bases de dados científicos ­ LILACS, SCIELO, PUBMED e BDENF. Resultados. Na presente revisão integrativa da literatura, foram analisados onze artigos da amostra original. Os principais cuidados de enfermagem são os relacionados com à alimentação, saúde bucal, lazer e atividade física, cuidados com medicamentos e com a ferida operatória, como também a necessidade de oferecer apoio à família dessas crianças. Os autores ressaltam que o enfermeiro está presente em diversos momentos da vida da criança, inclusive, no nascimento, porém a abordagem sobre CC é escassa na sua formação básica como enfermeiro, assim como em sua atuação profissional, percebendo-se escassez de propostas de educação continuada para o cuidado de crianças com CC. Conclusão. O estudo apontou que as orientações de enfermagem estão voltadas ao cuidados básicos da assistência e ao apoio familiar dessas crianças. Por fim, foi evidenciado nessa pesquisa o importante papel do enfermeiro para a consolidação das orientações das necessidades voltadas ao cuidar dessas crianças.

Prevalencia de defectos congénitos folato-sensibles en la provincia de Villa Clara / Prevalence of folate-sensitive birth defects in Villa Clara province

Introducción: Los defectos congénitos son un problema global que anualmente afecta a 7,9 millones de recién nacidos, los cuales constituyen la primera causa de muerte en los países desarrollados, y la segunda en países en vías de desarrollo, como es el caso de Cuba. Objetivos: Determinar la prevalencia de defectos congénitos folato-sensibles entre nacidos vivos, nacidos muertos e interrupciones electivas del embarazo. Métodos: Se realizó un estudio descriptivo y transversal en la provincia de Villa Clara donde se incluyeron todos los casos con defectos congénitos folato-sensibles diagnosticados entre 2013 y 2018. Resultados: Las cardiopatías congénitas conotruncales, el síndrome Down y los defectos del tubo neural fueron los fenotipos clínicos más prevalentes. La tasa de prevalencia ajustada fue de 5,79 por 1 000 nacimientos. Se constató una mayor tasa de prevalencia entre nacidos muertos y se interrumpió el 43,26 % de los embarazos con defectos diagnosticados prenatalmente. Las mayores tasas de prevalencia ajustada de cardiopatías y hendiduras labiopalatinas se identificaron en los municipios de Corralillo y Quemado de Güines, mientras que Placetas tuvo las mayores tasas de defectos del tubo neural y síndrome Down, y Manicaragua las de gastrosquisis. Conclusiones: La mayor frecuencia de defectos congénitos entre nacidos muertos puede estar en relación con la gran expresividad variable de los defectos estudiados, donde se incluyen fenotipos clínicos de gravedad. En los diferentes municipios con elevadas tasas de prevalencia de estos defectos están involucrados diferentes factores de riesgo ambientales que actúan sobre un genotipo que predispone a estos defectos congénitos.

Introduction: congenital defects are a global problem, annually affecting 7.9 million newborns. They constitute the leading cause of death in developed countries and the second one in developing countries like Cuba. Objective: to determine the prevalence of folate-sensitive birth defects among live births, stillbirths and elective pregnancy terminations. Methods: a descriptive and cross-sectional study was carried out in Villa Clara province, where all cases with folate-sensitive birth defects diagnosed between 2013 and 2018 were included. Results: conotruncal congenital heart diseases, Down syndrome and neural tube defects were the most prevalent clinical phenotypes. The adjusted prevalence rate was 5.79 per 1,000 births. A higher prevalence rate was found among stillbirths and 43.26% of pregnancies with prenatally diagnosed defects were terminated. The highest rates of adjusted prevalence of cleft lip and palate and heart diseases were identified in Corralillo and Quemado de Güines municipalities, while Placetas had the highest rates of neural tube defects and Down syndrome, as well as Manicaragua those of gastroschisis. Conclusions: the higher frequency of congenital defects among stillbirths may be related to a highly variable expressivity of the studied defects, where serious clinical phenotypes are included. Different environmental risk factors acting on a genotype that predisposes these congenital defects are involved in the different municipalities with high prevalence rates of these defects.

Cardiopatia congênita e percepções e sentimentos maternos / Congenital heart disease and maternal perceptions and feelings / Cardiopatías congénitas y percepciones y sentimientos maternos

Introdução: A Cardiopatia Congênita (CC) é uma doença crônica, caracterizada por anormalidades estruturais e funcionais no sistema cardiocirculatório, podendo ocorrer por fatores genéticos, mutações, alterações cromossômicas ou mesmo ter uma origem multifatorial. Estudos discutem sobre a possibilidade da CC criar um ambiente estressor para a criança e sua família, sobretudo para sua mãe, por ser o elemento da família que, geralmente, assume o acompanhamento e a execução dos cuidados com a criança. Objetivo: Identificar e descrever a percepção e sentimentos maternos acerca da doença do filho, suas dificuldades, o impacto da doença na qualidade de vida da família e suas angústias diante do futuro. Método: Pesquisa qualitativa, transversal, descritiva, com coleta de dados feita entre os meses de Dezembro de 2022 e Fevereiro de 2023, por meio de entrevistas semiestruturadas realizadas por ligação telefônica, gravada, com 13 mães de crianças com CC. O processo de análise foi orientado pela análise de conteúdo do tipo temática. Resultado: O estresse está presente na fala da maior parte das mães. Essas falas trazem à cena o fato de que este se condensa especialmente nos períodos iniciais do processo: descoberta da doença do filho, notícia da(s) cirurgia(s), responsabilidade pelos cuidados que se prolongam, isolamento materno. Conclusão: O medo da morte, do futuro e do desenvolvimento da criança são fantasmas que também circulam nas manifestações maternas e expressam a dificuldade em antecipar aos seus filhos uma subjetividade, condição de base para o desenvolvimento geral adequado. (AU)

Introduction: Congenital heart disease (CHD) is a chronic disease, characterized by structural and functional abnormalities in the cardiocirculatory system, which may occur due to genetic factors, mutations, chromosomal alterations, or even have a multifactorial origin. Studies discuss the possibility of CC creating a stressful environment for the child and his family, especially for his mother, as she is the family member who generally takes on the monitoring and execution of care for the child. Objective:To identify and describe maternal perceptions and feelings about their child's illness, their difficulties, the impact of the disease on the family's quality of life, and their anxieties about the future. Method:Qualitative, cross-sectional, descriptive research, with data collection carried out between December 2022 and February 2023, through semi-structured interviews conducted by telephone, recorded, with 13 mothers of children with CC. The analysis process was guided by thematic content analysis. Result:Stress is in the speech of most mothers. These statements bring to the fore the fact that stress is condensed especially in the early stages of the process: discovery of the child's illness, news of the surgery(s), responsibility for prolonged care, and maternal isolation. Conclusion: Fear of death, the future and the child's development are ghosts that also circulate in maternal manifestations and express the difficulty in anticipating their children, a basic condition for adequate general development. (AU)

Introducción: La cardiopatía congénita (CC) es una enfermedad crónica, caracterizada por anomalías estructurales y funcionales en el sistema cardiocirculatorio, que pueden deberse a factores genéticos, mutaciones, alteraciones cromosómicas o incluso tener un origen multifactorial. Los estudios discuten la posibilidad de que el CC genere un ambiente estresante para el niño y su familia, especialmente para su madre, ya que es ella la que generalmente asume el seguimiento y ejecución del cuidado del niño. Objetivo: Identificar y describir las percepciones y sentimientos maternos sobre la enfermedad del hijo, sus dificultades, el impacto de la enfermedad en la calidad de vida de la familia y sus angustias sobre el futuro. Método: Investigación cualitativa, transversal, descriptiva, con recolección de datos realizada entre diciembre de 2022 y febrero de 2023, a través de entrevistas semiestructuradas realizadas por teléfono, grabadas, con 13 madres de niños con CC. El proceso de análisis fue guiado por el análisis de contenido temático. Resultado: El estrés está presente en el habla de la mayoría de las madres. Estas declaraciones traen a la luz el hecho de que el estrés se condensa especialmente en las primeras etapas del proceso: descubrimiento de la enfermedad del niño, noticia de la(s) cirugía(s), responsabilidad por cuidados prolongados, aislamiento materno. Conclusión: El miedo a la muerte, al futuro y al desarrollo del niño son fantasmas que también circulan en las manifestaciones maternas y expresan la dificultad de anticiparse a sus hijos, condición básica para un adecuado desarrollo general. (AU).

Atención de enfermería en preescolar con atresia pulmonar en oxigenación con membrana extracorpórea: caso exitoso / Nursing Care in Kindergarten with Pulmonary Atresia in Extracorporeal Membrane Oxygenation: a Succesful Case / Cuidados de enfermagem em uma criança pré-escolar com atresia pulmonar em oxigenação por membrana extracorpórea: caso de sucesso

Introducción. La atresia pulmonar con comunicación interventricular es una cardiopatía compleja que enmarca grandes desafíos en su etapa pre y postquirúrgica; el uso del soporte vital extracorpóreo con membrana de oxigenación restablece la oxigenación y perfusión al organismo para permitir recuperación y complementar estudios. El objetivo de este caso clínico es determinar la atención de enfermería en la fase aguda post quirúrgica. Se expondrá usando el modelo teórico de Dorothea Orem: teorías de déficit de autocuidado y teoría de sistemas. Esta cardiopatía es la forma más severa de la tetralogía de Fallot. Tiene una incidencia del 2% entre todas las cardiopatías. Metodología. Es el caso de una preescolar con atresia pulmonar con comunicación interventricular, se describe los procesos realizados desde el diagnóstico, la intervención percutánea y reparo quirúrgico, así como el manejo de lesión residual en el marco del uso de diferentes tecnologías. Se resalta como elemento clave el uso del soporte con oxigenación con membrana extracorpórea usada como puente a decisión. Resultados. El plan de atención de enfermería en esta fase crítica logró los resultados planteados como la adecuada perfusión y oxigenación, recuperación de la función ventricular, estabilización hemodinámica para ser llevada al reparo de la lesión residual. Este novedoso soporte fue implementado dos veces durante la misma hospitalización y con resultados exitosos. Conclusiones. Caso de difícil manejo con terapias convencionales, pero con aplicación de una atención integral de enfermería; el uso de tecnología y de diversas especialidades permitió un egreso de la menor sin complicaciones. Palabras clave: Atresia Pulmonar; Oxigenación por membrana extracorpórea; Atención de Enfermería; Unidades de Cuidado Intensivo Pediátrico; Cardiopatías Congénitas.

Introduction. The pulmonary atresia with ventricular septal defect is a complex heart disseas that possess great challenges in pre and post-surgical stages; the use of vital support extracorporeal with membrane oxygenation restores oxygen and perfusion to the body to allow recovery and complement studies. The objetive of this case report is to determinate the nurse attention in the acute post quirulgical phase. It will be presented using Dorothea Orem's theoretical model: theories of self-care deficits and systems theory. This heart disease is the most severe form of tetralogy Fallot. It has an incidence of 2% among all heart diseases. Methodology. This is the case of a kindergarten with pulmonary atresia with ventricular septal defect, the processes carried out from diagnosis, percutaneous intervention and surgical repair are described, as well as the management of residual injury within the framework of the use of different technologies. The use of extracorporeal membrane oxygenation support used as a decision bridge is highlighted as a key element. Results. The nursing care plan in the critical phase achieved the results proposed as adequate perfusion and oxygenation, recovery of the ventricular function, hemodynamic stabilization to be carried out to repair the residual injury. This newfangled support was implemented twice during the same hospitalization with sucessful result. Conclusions. Case report with struggle managment with conventional therapies but with the application of comprehensive nursing care; the use of technology and the work of various specialities allowed the minor to be discharged without complications. Keywords: Pulmonary Atresia; Extracorporeal Membrane Oxygenation; Nursing Care; Intensive Care Units, Pediatric; Heart Defects, Congenital.

Introdução. A atresia pulmonar com comunicação interventricular é uma cardiopatia complexa que apresenta grandes desafios em sua fase pré e pós-cirúrgica. O uso de suporte de vida extracorpóreo com membrana de oxigenação restaura a oxigenação e a perfusão do corpo para permitir a recuperação e complementar os estudos. O objetivo deste caso clínico é determinar os cuidados de enfermagem na fase aguda pós-cirúrgica. Será apresentado utilizando o modelo teórico de Dorothea Orem: teorias do déficit de autocuidado e teoria de sistemas. Esta doença cardíaca é a forma mais grave de tetralogia de Fallot. Tem uma incidência de 2% entre todas as doenças cardíacas. Metodologia. É o caso de uma criança em idade pré-escolar com atresia pulmonar com comunicação interventricular, são descritos os processos realizados desde o diagnóstico, intervenção percutânea e reparação cirúrgica, bem como o manejo da lesão residual no âmbito da utilização de diferentes tecnologias. Destaca-se, como elemento-chave, a utilização de suporte com oxigenação por membrana extracorpórea como ponte para a decisão. Resultados. O plano de cuidados de enfermagem nesta fase crítica alcançou os resultados propostos como perfusão e oxigenação adequadas, recuperação da função ventricular, estabilização hemodinâmica a ser realizada para o reparo da lesão residual. Este novo suporte foi implementado duas vezes durante a mesma hospitalização e com resultados bem-sucedidos. Conclusões. Caso de difícil manejo com terapias convencionais, mas com aplicação de cuidados integrais de enfermagem, o uso da tecnologia e de diversas especialidades permitiu que a criança recebesse alta sem complicações. Palavras-chave: Atresia Pulmonar; Oxigenação por Membrana Extracorpórea; Cuidados de Enfermagem; Unidades de Terapia Intensiva Pediátrica; Cardiopatias Congênitas.

Influence of Congenital Heart Defect Types over Cardiopulmonary Bypass, Aortic Cross-Clamping, and Intensive Care Unit Length of Stay and Their Association with Genetic Factors

Aim: Surgical correction of congenital heart defects (CHD) often requires interruption of blood flow through cardiopulmonary bypass (CPB) and aortic cross-clamping (ACC), for which duration(s) are considered to be prognostic factors, along with intensive care unit (ICU) length of stay (ICULOS). The aim of this study was to evaluate these surgical prognostic factors in pediatric patients with different types of CHD regarding their type of lesion and associated genetic factors. Study Design: Cross-sectional cohort study with 307 pediatric patients. Place and Duration of Study: Pediatric Intensive Care Unit (ICU) of Hospital da Criança Santo Antônio, in Porto Alegre/RS, Brazil, from 2006-2009 (3 years) Methodology: After inclusion criteria, we studied 266 pediatric patients admitted for the first time in a reference cardiac pediatric ICU from Southern Brazil following cardiac surgery. Intraoperative prognostic factors such as duration of CPB, ACC and ICULOS, in addition to dysmorphological and cytogenetic examinations were compiled and analyzed. P-values of <0.05 were considered significant. Results: CPB time was associated to four outflow tract defects (Tetralogy of Fallot [ToF], transposition of the great arteries [TGA], double outlet right ventricle, and truncus arteriosus [TA]), atrioventricular septal defect, and hypoplastic left heart syndrome (HLHS) (P < 0.001). ACC duration was associated with three outflow tract defects (ToF, TGA, and TA) and HLHS (P < 0.001). Moreover, CPB and ACC times showed an association with cyanotic and complex heart defects, as well as prolonged ICULOS (P < 0.001). There was no relationship between these prognostic factors and syndromic aspects or cytogenetic findings. Conclusions: CHD type has an impact over CPB and ACC duration and ICULOS, whereas genetic factors are not associated with those prognostic factors.

El ancho de distribución eritrocitaria en el pronóstico de corrección quirúrgica de cardiopatías congénitas / Red cell distribution width in the prognosis of surgical correction of congenital heart diseases

Resumen Objetivo: Evaluar la capacidad del ancho de distribución eritrocitaria (ADE) para predecir la mortalidad en niños sometidos a cirugía cardiovascular en la Fundación Hospital Infantil Napoleón Franco Pareja, en Colombia. Método: Estudio analítico de corte transversal retrospectivo que incluyó 45 individuos de 0 a 17 años operados de cardiopatía congénita. Se aplicaron la escala RACHS-1 (Risk Adjustment in Congenital Heart Surgery) y variables de laboratorio, incluyendo el ADE. La asociación entre el ADE y la mortalidad se determinó mediante análisis por curva ROC y correlación rho de Spearman. Resultados: Un ADE superior al 15.52% representó 1.6 veces más riesgo, comparado con los individuos por debajo de ese valor (intervalo de confianza del 95%: 1.01-2.6; p = 0.034). Los valores del ADE no se correlacionaron con los días de estancia hospitalaria ni con las complicaciones. El ADE prequirúrgico y el puntaje RACHS-1 fueron significativamente mayores en el grupo de mortalidad. La relación entre el ADE prequirúrgico y el puntaje RACHS-1 fue significativa. Conclusiones: En nuestro estudio, el ADE prequirúrgico presentó un poder moderado para discriminar la mortalidad perioperatoria en la corrección quirúrgica de cardiopatías congénitas. Se precisan más estudios con mayor tamaño de muestra.

Abstract Objective: To evaluate the capacity of red cell distribution width (RDW) to predict mortality in children undergoing cardiovascular surgery at the Fundación Hospital Infantil Napoleón Franco Pareja, in Colombia. Method: Retrospective cross-sectional analytical study that included 45 individuals aged 0 to 17 years operated for congenital heart disease. The RACHS-1 (Risk Adjustment in Congenital Heart Surgery) scale and laboratory variables including the RDW were applied. The association between RDW and mortality was determined by ROC curve analysis and Spearman's rho correlation. Results: An RDW greater than 15.52% represented 1.6 times more risk, compared to individuals below that value (95% confidence interval: 1.01-2.6; p = 0.034). The RDW values did not correlate with days of hospital stay or complications. The preoperative RDW and RACHS-1 score were significantly higher in the mortality group. The relationship between presurgical RDW and the RACHS-1 score was significant. Conclusions: In our study, the preoperative RDW had moderate power to discriminate perioperative mortality in the surgical correction of congenital heart disease. More studies with a larger sample size are required.

Coração em Criss-Cross: um relato de caso / Criss-Cross Heart: a case report

Coração em criss-cross (ou coração entrecruzado) foi descrito pela primeira vez em 1974. Trata-se de uma malformação cardíaca congênita, rara, ocorrendo 8 casos a cada 1.000.000 de crianças, e representando somente 0,1% das malformações congênitas. Os métodos diagnósticos de escolha são o ecocardiograma transtorácico, a ressonância magnética cardíaca (RMC), a angiotomografia (TC) e, eventualmente, o cateterismo cardíaco. Neste relato, descreve-se o caso de um recém-nascido com coração em criss-cross somado à dupla via de saída do ventrículo direito (VD), com vasos mal posicionados, além de comunicação interatrial (CIA), comunicação interventricular (CIV), displasia de valva tricúspide e veia cava superior esquerda persistente. Não se sabe a etiologia exata dessa malformação, mas parece ocorrer pela rotação dos ventrículos em seu eixo longitudinal, não acompanhada das rotações atrial e das valvas atrioventriculares (AV). Esse movimento produz uma alteração das vias de entrada dos ventrículos, determinando que o VD se posicione em plano superior e o esquerdo em plano inferior. Apesar de ainda não se saber a exata causa dessa anomalia, acredita-se que uma alteração genética possa estar levando a esses casos: a mutação do gene Cx43. O diagnóstico do caso em questão foi dado pela ecocardiografia transtorácica e da TC de aorta e artérias pulmonares, que mostraram, além do criss-cross, outras alterações, como dupla via de saída do VD, CIA e CIV amplas.(AU)

Criss-cross heart was first described in 1974. It is a rare congenital heart malformation that occurs in 8 cases per 1,000,000 children, and represents only 0.1% of congenital malformations. The diagnostic methods of choice are transthoracic echocardiography, cardiac magnetic resonance (CMR), computed tomography angiography (CT) and, sometimes, cardiac catheterization. This report describes the case of a newborn with a criss-cross heart in addition to double-outlet right ventricle (RV), with poorly positioned vessels, in addition to atrial septal defect (ASD), interventricular septal defect, tricuspid valve dysplasia and persistent left superior vena cava. The exact etiology of this malformation is not known, but it seems to occur due to rotation of the ventricles in their longitudinal axis, not accompanied by rotation of the atrial and atrioventricular (AV) valves. This movement produces abnormal ventricular inlets, determining that the RV be positioned on a superior plane and the left ventricle on an inferior plane. Although the exact cause of this anomaly is still unknown, it is believed that a genetic abnormality may be leading to these cases: mutation of the Cx43 gene. Diagnosis of the case concerned was given by transthoracic echocardiography and computed CT of the aorta and pulmonary arteries, which showed, in addition to the criss-cross heart, other abnormalities, such as double-outlet RV, large ASD and ventricular septal defect (VSD).(AU)

Cuidado de enfermería al paciente pediátrico con tetralogía de Fallot intervenido quirúrgicamente: una revisión de alcance / Nursing care of the surgically operated pediatric patient with Tetralogy of Fallot: a scoping review

Resumen Introducción: los niños con tetralogía de Fallot afrontan grandes desafíos durante su etapa posoperatoria, por lo cual es necesario que el personal de enfermería reconozca los mecanismos involucrados en este proceso con el fin de contribuir a que estos alcancen un nivel de adaptación fisiológico y psicosocial integrado. Objetivo: establecer las intervenciones de enfermería para niños en posoperatorio paliativo o correctivo de tetralogía de Fallot. Materiales y método: a través de la metodología propuesta por Fawcett et al. y el Instituto Joanna Briggs, se realizó una búsqueda en diez bases de datos y motores de búsqueda de artículos publicados del 2014 al 2019 utilizando dos fórmulas: Pediatric AND Tetralogy of Fallot AND Postoperative y Pediatric AND Tetralogy of Fallot AND Postoperative AND Psychosocial Adaptation. Resultados: se obtuvieron 1.901 estudios, de los cuales 56 cumplieron todos los criterios de elegibilidad. Los resultados se estructuraron según el plan de atención de enfermería y se enmarcaron en los modos de adaptación y necesidades del modelo de adaptación de Callista Roy. Con la información obtenida, se construyeron doce diagnósticos NANDA correspondientes al modo de adaptación fisiológico y once al modo psicosocial. Conclusión: existe información relevante que permite establecer las intervenciones de cuidado desde el campo de acción para enfermería en este contexto de unidad de cuidado intensivo pediátrico, ya que se obtuvieron intervenciones para abordar todas las necesidades que el modelo epistemológico plantea.

Abstract Introduction: children with tetralogy of Fallot face great challenges during their postoperative stage, so it is necessary for nurses to recognize the mechanisms involved in this process, in order to help them reach an integrated physiological and psychosocial level of adaptation. Objective: to establish nursing interventions for children in palliative or corrective postoperative tetralogía de Fallot. Materials and method: using the methodology proposed by Fawcett et al. and the Joanna Briggs Institute, we searched 10 databases and search engines for articles published from 2014 to 2019 using 2 formulas: Pediatric AND Tetralogy of Fallot AND Postoperative; and Pediatric AND Tetralogy of Fallot AND Postoperative AND Psychosocial Adaptation. Results: 1.901 studies were obtained, of which 56 met all eligibility criteria. The results were structured according to the nursing care plan, and were framed within the modes of adaptation and needs of Callista Roy’s adaptation model. With the information obtained, 12 NANDA diagnoses corresponding to the physiological mode of adaptation and 11 to the psychosocial mode were constructed. Conclusion: There is relevant information that allows us to establish care interventions from the field of action for nursing in this paediatric intensive care unit context, as interventions were obtained to address all the needs that the epistemological model proposes.

Direct Anastomosis of Persistent Left Superior Vena Cava to Right Superior Vena Cava in a Pediatric Patient with Tetralogy of Fallot: an Alternative Technique

ABSTRACT The presence of persistent left superior vena cava to the left atrium connection without an innominate vein may give rise to technical challenges during intracardiac repair. In this report, the end-to-side anastomosis technique of the persistent left superior vena cava to the right superior vena cava is discussed in a patient with tetralogy of Fallot associated with persistent left superior vena cava draining directly into the left atrium. A successful end-to-side anastomosis between the persistent left superior vena cava and the right superior vena cava was performed and short-term anastomosis patency was documented via angiography.

Based on Delphi method improved early graded rehabilitation nursing model for postoperative children with congenital heart disease / 中国实用护理杂志

Objective:To improve the early graded rehabilitation nursing model suitable for postoperative children with congenital heart disease, providing reference for related research and clinical practice.Methods:Searched databases like JBI, PubMed, Medline, CINAHL, CNKI, Wanfang Data and related websites for information on postoperative rehabilitation of children with congenital heart disease. We improved the first draft model with qualitative interview results and used Delphi method to conduct two rounds of consultation for 16 experts from 6 provinces and cities to further test the scientific and feasibility of the model.Results:The early graded rehabilitation nursing model for postoperative children with congenital heart disease includes 4 first-level items, 15 second-level items and 48 third-level items. The 4 first-level items are the evaluation of the early graded rehabilitation nursing model, the grading standard of the early graded rehabilitation nursing model, the implementation of the early graded rehabilitation nursing model, the effect evaluation and health education. Experts′ response rates were 100% in the 2 rounds, experts′ authority coefficient were 0.82 and 0.84 respectively, and the Kendall′ s W rank-order correlation coefficients of all levels of indicators were 0.188-0.246, 0.223-0.287 (all P<0.01). Conclusions:The improved early graded rehabilitation nursing model for postoperative children with congenital heart disease is scientific, pertinence and safe, which can provide guidance for clinical rehabilitation nursing practice.

Paternal occupational exposures and infant congenital heart defects in the Japan Environment and Children's Study

BACKGROUND@#Few prospective studies have investigated the association between paternal occupational exposures and risk of infant congenital heart defects (CHDs). We investigated the associations between paternal occupational exposures, frequency of use, and concurrent or sequential exposure to a mixture of compounds and the risk of infant CHDs.@*METHODS@#Our study examined 28,866 participants in the Japan Environment and Children's Study. Logistic regression analysis was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs) associated with paternal occupational exposures during the 3 months until pregnancy was noticed after adjustment for potential confounding factors of the infant CHDs. CHD diagnosis was ascertained from medical record.@*RESULTS@#In total, 175 were diagnosed with infant CHDs. The number of fathers who were exposed to the following substances at least once a month were: 11,533 for photo copying machine/laser printer, 10,326 for permanent marker, 8,226 for soluble paint/inkjet printer, 6,188 for kerosene/petroleum/benzene/gasoline, 4,173 for organic solvents, 3,433 for chlorine bleach/germicide, 2,962 for engine oil, 2,931 for insecticide, 2,460 for medical sterilizing disinfectant, 1,786 for welding fumes, 1,614 for dyestuffs, 1,247 for any products containing lead-like solder, 986 for herbicide, 919 for radiation/radioactive substances/isotopes, 837 for lead-free solder, 341 for microbes, 319 for formalin/formaldehyde, 301 for agricultural chemical not listed above or unidentified, 196 for general anesthetic for surgery at hospital, 171 for anti-cancer drug, 147 for chromium/arsenic/cadmium, 88 for mercury and 833 for other chemical substances. Paternal occupational exposure regularly to photo copying machine or laser printer and soluble paint/inkjet printer were associated with higher risks of infant CHDs: the adjusted ORs (95%CIs) were 1.38 (1.00-1.91) and 1.60 (1.08-2.37), respectively. The higher risks were also observed for occasional exposure to engine oil, any products containing lead-like solder lead-free solder, and microbes; the adjusted ORs (95%CIs) were 1.68 (1.02-2.77), 2.03 (1.06-3.88), 3.45 (1.85-6.43), and 4.51, (1.63-12.49), respectively.@*CONCLUSIONS@#Periconceptional paternal occupational exposure was associated with a higher risk of infant CHDs. Further studies using biomarkers of the association between paternal occupational exposure and infant CHDs are warranted.