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.

Role of echocardiography in assessing copy number variation in fetal congenital heart disease / 中华围产医学杂志

Objective:This study examines the application of echocardiography in the prenatal diagnosis of copy number variation (CNV) associated with fetal congenital heart disease (CHD).Methods:A retrospective analysis was conducted on 447 singleton pregnancies from Quanzhou Maternal and Child Care Hospital (Quanzhou Children's Hospital) from January 2019 to August 2022. These individuals underwent echocardiographic assessments suggestive of fetal CHD and subsequently received invasive prenatal diagnoses. Comprehensive karyotype analysis and chromosome microarray analysis (CMA) were performed for each case. The discrepancies in the chromosomal abnormality detection were analyzed between the results produced by CMA and karyotype analysis. Furthermore, differences in the detection of pathogenic copy number variation (pCNV) between the two methods in CHD cases with diverse cardiac phenotypes, including the presence or absence of extracardiac structural malformations, the type, and quantity of cardiac structural anomalies, were explored. Statistical analysis was conducted using the Chi-square test. Results:Compared with conventional karyotype analysis, CMA demonstrated a higher detection rate of fetal chromosomal abnormalities [10.5% (47/447) vs. 20.6% (92/447), χ 2=161.56, P<0.001]. In terms of distinct cardiac phenotypes, CHD cases with extracardiac structural anomalies displayed an escalated pCNV detection rate in comparison to isolated CHD cases [11.4% (45/394) vs. 32.1% (17/53), χ 2=16.68, P<0.001]. Within the cardiac structural anomaly subgroups, increased pCNV detection rates were observed in the septal defect subgroup, conotruncal malformation subgroup, and left ventricular malformation subgroup [18.4%(29/158), 25.9%(7/27), and 25.0%(7/28) vs. 7.6%(16/210); χ 2=9.15, 9.68, and 8.55, respectively, all P<0.05]. The CMA-identified pCNV correlated with CHD included 22q11.2 deletions/duplications in eight cases, 4p16.3 deletions in two cases, 11q23.3 microduplications in two cases, 1q21.1 microdeletions/microduplications in two cases, 4q28.3 microduplications in one case, and 10p15.3 microdeletions in one case. Conclusions:CMA technology exhibited an enhanced ability to detect pCNV in fetuses with CHD. Echocardiography can guide targeted CMA screening, thereby facilitating prenatal genetic assessment of CHD.

Prenatal ultrasonic diagnosis and genetic analysis of fetal congenital heart diseases / 中国医学影像技术

Objective To explore the genetic characteristics of fetuses with congenital heart diseases(CHD)diagnosed by prenatal ultrasound.Methods Data of 613 singletons with prenatal ultrasonic diagnosed CHD were retrospectively analyzed.The cardiac structural abnormalities were classified into 8 types.Whole-exome sequencing(WES)was performed for 40 fetuses since chromosomal karyotyping analysis and/or chromosomal microarray analysis(CMA)showed benign copy number variations(CNV)or variants of uncertain significance(VUS).Results Among 613 fetuses,479 fetuses underwent both chromosomal karyotyping analysis and CMA,genomic abnormalities were detected in 60 fetuses(60/479,12.53％).Among 134 fetuses underwent only CMA,genomic abnormalities were found in 4 fetuses(4/134,2.99％).According to results of chromosomal karyotyping analysis and/or CMA,abnormalities were noticed in 40 fetuses(40/568,7.04％)among 568 fetuses with isolated CHD,while in 15 fetuses(15/45,33.33％)among 45 fetuses with non-isolated CHD,respectively.Abnormality detection rate of chromosomal karyotyping analysis and/or CMA in fetuses with complex CHD(10/41,24.39％)was higher than that in fetuses with non-complex CHD(54/572,9.44％).Among complex CHD fetuses,abnormality detection rate was the highest in fetuses with conotruncal defect(CTD)combined with malformation of venous system(4/13,30.77％),while among fetuses with non-complex CHD,situs inversus viscerum had the highest detection rate(1/4,25.00％).Among 40 fetuses chromosomal karyotyping analysis and/or CMA showed benign CNV or VUS,WES indicated pathogenic CNV/likely pathogenic CNV(P/LP)in 3 fetuses,VUS in 3 fetuses and benign CNV in 34 fetuses.Conclusion Fetuses with CHD,especially extracardiac malformations had possibilities of genomic abnormalities.Fetuses with CTD combined with malformation of venous system had higher possibilities of genomic abnormalities.Compared with CMA alone,chromosomal karyotyping analysis combined with CMA was helpful for detecting genomic abnormalities.

Clinical value of prospective ECG-gated high-pitch CT scanning in the diagnosis of pediatric congenital heart disease / 中华放射医学与防护杂志

Objective:To investigate the clinical value of prospective ECG-gated high-pitch protocol scanning of third generation DSCT in the diagnosis of pediatric congenital heart disease (CHD).Methods:A total of 243 children with confirmed CHD who were expected to undergo surgical treatment were prospectively collected and randomly divided evenly into 3 groups, with first group for prospective ECG-gated high-pitch scanning in third generation DSCT (Flash 3rd), second group for prospective ECG-gated high-pitch scanning in second generation DSCT (Flash 2nd) and third group for prospective sequential scanning in third generation DSCT (Sequence 3rd). The SD value and SNR of aortic root and pulmonary artery of each child were recorded. The 5-point system is adopted with subjective scoring. Based on the result of operation, the diagnosis accuracy in 3 groups was analyzed. Results:The E values in Flash 3rd, Flash 2nd and Sequence 3rd group were 0.24 (0.19, 0.27), 0.11 (0.10, 0.14) and 0.44 (0.39, 0.48) mSv ( H=207.04, P<0.05), respectively. Subjective scores of group Flash 3rd and Sequence 3rd were significantly higher than that of group Flash 2nd [4 (4, 4) vs. 4(3, 4) vs. 3(3, 3), H=124.05, P<0.05] and no difference between these two groups. SD value of aortic root and pulmonary artery of group Flash 3rd and Sequence 3rd were significantly lower than that of group Flash 2nd( H= -40.27-33.38, P<0.05). SNR of aortic root and pulmonary artery of group Flash 3rd was significantly higher than that of group Flash 2nd and Sequence 3rd ( H=-0.90-51.42, P<0.05). Diagnosis accuracy of intracardiac malformation for group Flash 2nd was significantly lower than that of Flash 3rd and Sequence 3rd (77.7%, 90.9%, 88.9%, K=9.36, P<0.05), and there was no significant difference between the latter two groups. There was no difference in diagnosis accuracy of extracardiac malformation among 3 groups (88.6%, 94.8%, 92.2%, K=3.11, P=0.21). Conclusions:The prospective ECG-gated high-pitch scanning in third generation DSCT can take into account radiation dose and image quality, which has important clinical value in the diagnosis of CHD.

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.

Factors associated with the indication of neonatal echocardiography in the investigation of congenital heart diseases / Factores asociados a la indicación de la ecocardiografía neonatal en la investigación de cardiopatías congénitas / Fatores associados à indicação de ecocardiografia neonatal na investigação de cardiopatias congênitas

ABSTRACT Objective: To analyze the factors associated with the indication of echocardiography for the investigation of congenital heart disease among newborns. Method: Retrospective sectional study through the collection of 848 medical records of patients admitted to maternity hospitals in Rio de Janeiro-Brazil, respecting the time frame from September to December 2022. Results: The average age of mothers was 26.5±6.3 years; 52.7% were classified as brown. The average age of the newborns was 3.5±5.6 days. Maternal variables: gestational age (OR=6.93, CI:3.76-12.80), number of gestational risk factors (1.90: 1.47-2.45) and number of medications (1. 97: 1.40-2.77); and neonatal variables: age (1.07: 1.03-1.02), prematurity (10.55: 5.29-21.03) and number of risk factors (2.62: 2.03-3 .38) were significantly associated with the indication for echocardiography (p<0.001). Conclusion: It is concluded that the different maternal and neonatal variables, gestational age, number of gestational risk factors, number of medications, age, prematurity and number of risk factors, respectively, showed a significant association for the indication of echocardiography. Therefore, the identification of these factors will enable the investigation of congenital heart disease at an opportune time among newborns.

RESUMEN Objetivo: Analizar los factores asociados a la indicación de la ecocardiografía para la investigación de cardiopatías congénitas en recién nacidos. Método: Estudio seccional retrospectivo a través de la recolección de 848 historias clínicas de pacientes ingresadas en maternidades de Río de Janeiro-Brasil, respetando el período de septiembre a diciembre de 2022. Cálculos del odds ratio, intervalo de confianza del 95% en el nivel de significancia de 5%. Resultados: La edad promedio de las madres fue de 26,5±6,3 años; El 52,7% fueron clasificados como pardos. La edad promedio de los recién nacidos fue de 3,5±5,6 días. Variables maternas: edad gestacional (OR=6,93, IC:3,76-12,80), número de factores de riesgo gestacional (1,90: 1,47-2,45) y número de medicamentos (1,97: 1,40-2,77); y variables neonatales: edad (1,07: 1,03-1,02), prematuridad (10,55: 5,29-21,03) y número de factores de riesgo (2,62: 2,03-3,38) se asociaron significativamente con la indicación de ecocardiografía (p<0,001). Conclusión: Se concluye que las diferentes variables maternas y neonatales, edad gestacional, número de factores de riesgo gestacional, número de medicamentos, edad, prematuridad y número de factores de riesgo, respectivamente mostraron asociación significativa para la indicación de ecocardiografía. Por lo tanto, la identificación de estos factores permitirá investigar las cardiopatías congénitas en un momento oportuno entre los recién nacidos.

RESUMO Objetivo: Analisar os fatores associados à indicação de ecocardiografia para a investigação de cardiopatia congênita entre recém-nascidos. Método: Estudo seccional retrospectivo por meio da coleta em 848 prontuários de pacientes internados em maternidades no Rio de Janeiro-Brasil, respeitando o recorte temporal de setembro a dezembro de 2022. Realizados cálculos da razão de chance, intervalo de confiança de 95% em nível de significância de 5%. Resultados: A média de idade das mães foi de 26,5±6,3 anos; 52,7% foram classificadas como pardas. A idade média dos recém-nascidos foi de 3,5±5,6 dias.As variáveis maternas: idade gestacional (RC=6,93, IC:3,76-12,80), número de fatores de risco gestacional (1,90: 1,47-2,45) e número de medicamentos (1,97: 1,40-2,77); e as variáveis neonatais: idade (1,07: 1,03-1,02), prematuridade (10,55: 5,29-21,03) e número de fatores de risco (2,62: 2,03-3,38) se associaram significativamente à indicação de ecocardiografia (p<0,001). Conclusão: Conclui-se que as distintas variáveis materna e neonatal,idade gestacional, número de fatores de risco gestacional número de medicamentos, idade, prematuridade e números de fatores de risco, respectivamente apresentaram associação significativa para a indicação da ecocardiografia. Logo, a identificação desses fatores possibilitará a investigação de cardiopatia congênita em momento oportuno entre os recém-nascidos.

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.

Análise de custo-efetividade do teste de oximetria de pulso na triagem de cardiopatias congênitas / Cost-effectiveness analysis of the pulse oximetry test in screening for congenital heart diseases

Resumo: Introdução: Trata-se de uma tese desenvolvida na Pós-graduação em Enfermagem, na Linha de Pesquisa de Políticas e Práticas de Saúde, Educação e Enfermagem, de maneira a agregar valor à atividade de monitoramento de recém-nascidos, abrangendo a esfera mundial. Seu caráter inovador e sua relevância são fortes, considerando haver cenários de não realização da Triagem de Oximetria de Pulso (TOP) ou de desenvolvimento de técnica incorreta, de maneira que poderá favorecer a ampliação de sua aplicabilidade. Aponta-se que as Cardiopatias Congênitas (CC) são as anomalias de maior incidência nos recém-nascidos, com impacto na Mortalidade Infantil. Seu diagnóstico precoce está relacionado aos melhores desfechos e à sustentabilidade do sistema de saúde. O TOP comprova a importância do impacto social do diagnóstico para a real prevenção dos desfechos mórbidos, das mortes, e dos custos em saúde. Objetivos: Determinar a acurácia do TOP para triagem de CC nas primeiras 48 horas de vida, conforme especificidade e sensibilidade; e, desenvolver análise de custo-efetividade do TOP para triagem de CC. Método: Realizada revisão sistemática de acurácia de teste diagnóstico conforme as recomendações do Jonna Briggs Institute. O protocolo está registrado na plataforma PROSPERO - CRD42021256286 - e, publicado em periódico. Foram selecionados estudos com recém-nascidos sem o diagnóstico prévio de CC, independentemente da idade gestacional ao nascimento, que realizaram o TOP entre as primeiras 48h após o nascimento, em comparação ao exame físico realizado por profissional da saúde, ou estudos que não apresentaram comparadores e sim o diagnóstico de interesse, as CC. Para a seleção dos estudos utilizaram-se bases de dados e a literatura cinzenta. A Análise de Custo-Efetividade foi realizada conforme diretriz do Ministério da Saúde e, sob a perspectiva do SUS - Sistema Único de Saúde. Os dados de custo aplicados foram os disponibilizados em bases de dados nacionais. O modelo foi de Árvore de Decisão, com horizonte temporal de um ano, considerando quatro intervenções: 1) sem o TOP; 2) TOP sem reteste; 3) TOP com 1 reteste e 4) TOP com 2 reteste. Resultados: Na Revisão Sistemática foram incluídos 29 estudos, obtendo-se população total de 388.491 recém-nascidos. O TOP demonstrou sensibilidade de 47% (IC 95%: 43% a 50%) e, especificidade de 98% (IC 95%: 98% a 98%). Em estudos que apresentavam retestes e que incluíram recém-nascidos prematuros foram realizadas análises dos subgrupos, conforme o tempo de nascimento para realização do teste entre 24-48h. Na análise de custo-efetividade evidenciou-se que não realizar o TOP culminou com a estratégia de custo mais elevado, de R$ 85,41 por paciente. Os recém-nascidos triados com TOP + dois retestes possuem uma redução de custo entre R$ 0,21 a R$ 38,78 por paciente, conforme o protocolo aplicado. Conclusão: Para o diagnóstico precoce de CC, o TOP se configura como um teste de moderada sensibilidade, alta especificidade e custo-efetivo. Apresenta relação com o diagnóstico precoce, e contribui com a referência oportuna para atenção em serviço de saúde especializado e de qualidade, desde que na esfera de gestão estejam estabelecidos os acessos. Esta pesquisa é replicável considerando o rigor metodológico, assim como seu desenvolvimento e apresentação.

Abstract: Introduction: This is a thesis developed in the Graduate Program in Nursing, in the Research Line of Health, Education and Nursing Policies and Practices, in order to add value to the activity of monitoring newborns, covering the sphere worldwide. Its innovative character is high, as well as its relevance, considering that there are scenarios of non-performance of Pulse Oximetry Screening (POS), or of development of an incorrect technique, thus favoring the expansion of its applicability. It is pointed out that Congenital Heart Defects (CHD) are the anomalies with the highest incidence in newborns, with an impact on Infant Mortality. Its early diagnosis is related to better outcomes and sustainability of the health system. The POS proves the importance of the social impact of the diagnosis, for the real prevention of morbid outcomes, deaths, and health costs. Objectives: To determine the accuracy of the POS for CHD screening in the first 48 hours of life, according to specificity and sensitivity; and, develop a costeffectiveness analysis of the POS for CHD screening. Method: A systematic review of diagnostic test accuracy was carried out according to the Jonna Briggs Institute recommendations. The protocol is registered on the PROSPERO platform - CRD42021256286 - and published in a journal. Selected studies with newborns without a previous diagnosis of CHD, regardless of gestational age at birth, that performed the POS within the first 48 hours after birth, compared to the physical examination performed by a health professional or studies that did not present comparators, and the diagnosis of interest was CHD. For the selection of studies, databases and gray literature were used. The Cost-Effectiveness Analysis was carried out according to the guidelines of the Ministry of Health and from the perspective of the UHS - Unified Health System. The cost data applied were those available in national databases. The model was a Decision Tree, with a time horizon of one year, considering four interventions: 1) without the POS; 2) POS without retest; 3) POS with 1 retest and 4) POS with 2 retests. Results: The Systematic Review included 29 studies, resulting in a total population of 388,491 newborns. The POS showed a sensitivity of 47% (CI 95%: 43% to 50%) and specificity of 98% (CI 95%: 98% to 98%). In studies that presented retests and that included premature newborns, subgroup analyzes were performed, according to the time of birth for the test to be performed between 24-48 hours. In the cost-effectiveness analysis, it was shown that not performing the POS resulted in the highest cost strategy, R$ 85.41 per patient. Newborns screened with TOP + two retests have a cost reduction of between R$ 0.21 and R$ 38.78 per patient, depending on the protocol applied. Conclusion: For the early diagnosis of CHD, the POS is configured as a test of moderate sensitivity, high specificity and cost-effective. It is related to early diagnosis, and contributes to the timely referral for care in specialized and quality health services, as long as the accesses are established in the management sphere. This research is replicable considering the clarity of its development and presentation.

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.

Research progress of radiation protection in interventional diagnosis and treatment of pediatric heart disease / 中国基层医药

Interventional diagnosis and treatment of heart disease is the gold standard to evaluate the anatomy and physiology of children with congenital heart disease. It plays an important role in the treatment of congenital heart disease. However, ionizing radiation is inevitably harmful to the health of children and surgery operators to varying degrees. More and more attention has been paid by surgery operators to children's unique characteristics, protective awareness and skills. This paper reviews recent literature regarding the application, radiation hazards, and research status of interventional surgery in children with congenital heart disease, which hope to help people to better understand the importance of ionizing radiation protection.

Prenatal echocardiographic features and outcomes of congenital ventricular outpouching in ten fetuses / 中华围产医学杂志

Objective:To summarize the echocardiographic features and outcomes in fetuses with congenital ventricular outpouching (CVO).Methods:This retrospective study enrolled ten fetuses diagnosed with CVO by fetal echocardiography in the Affiliated Hospital of Qingdao University and Qingdao Women and Children's Hospital from January 2015 to April 2022. Clinical data were analyzed, including echocardiographic features, other intracardiac and extracardiac malformations, karyotypes, and pregnancy outcomes. Data were analyzed by descriptive statistics.Results:All ten cases were single, including eight ventricular diverticula and two ventricular aneurysms. Five cases had the anomaly in the left ventricular and the other five in the right. Five cases were isolated malformations, and the other five were complicated by other intra- or extracardiac malformations. A pathogenic copy number variation was detected in one case. Three pregnancies were terminated, and one was lost to follow-up. The other six fetuses were born alive and showed no obvious clinical symptoms or abnormalities in growth and development during 3-70 months of follow-up. The right ventricular diverticulum spontaneously disappeared in one case. One case with the right ventricular aneurysm was also diagnosed with noncompaction of the left ventricular myocardium by echocardiography at six months.Conclusions:Fetal CVO presents with typical echocardiographic features and can be diagnosed prenatally. Regular follow-up during pregnancy is recommended to observe the sizes of outpouchings and the occurrence of complications in fetuses with CVO after excluding other structural and chromosomal abnormalities to avoid unnecessary termination. Attention should also be paid to postnatal follow-up.

Nonsense variation of OTUD5 gene in a fetus with X-linked multiple congenital anomalies- neurodevelopmental syndrome / 中华围产医学杂志

We report a fetus presented with complex cardiac malformations, pulmonary atresia with ventricular septal defect, detected by fetal echocardiography at 17 +4 weeks. The pregnancy was terminated after routine counseling and genetic tests were performed on umbilical cord of the induced fetus and peripheral blood samples of the parents. Whole-exome sequencing identified a novel maternally-inherited and likely pathogenic variation hemizygous nonsense variant, c.1651C>T (p.Gln551*) in the OTUD5 gene (NM_017602.3), which was confirmed by subsequent Sanger sequencing. The fetus was finally diagnosed as X-linked multiple congenital anomalies-neurodevelopmental syndrome.

Genetic etiology of fetal conotruncal defects and significance of copy number variation sequencing and whole exome sequencing: analysis of 196 cases / 中华围产医学杂志

Objective:To investigate the genetic etiology of fetal conotruncal heart defects (CTDs) and to evaluate the performance of copy number variation sequencing (CNV-seq) and whole exome sequencing (WES) in identifying the genetic etiology.Methods:This retrospective study involved 196 fetuses diagnosed with CTDs by fetal echocardiography in Beijing Anzhen Hospital, Capital Medical University from June 2017 to December 2021. CNV-seq was performed to screen for chromosomal abnormalities [aneuploidy and copy number variations (CNVs)] in the fetuses and their parents, and then WES was performed if CNV-seq was negative. The diagnostic yields of genetic abnormalities [aneuploidy+CNVs+single nucleotide variations (SNVs)] for different types of CTDs were compared using Chi-square test. Results:CNV-seq revealed 54 cases (27.6%, 54/196) with chromosomal abnormalities, including 14 (7.1%, 14/196) aneuploidies, 39 (19.9%, 39/196) CNVs and one aneuploidy complicated by CNVs. Together with another 13 fetuses with pathogenic or likely pathogenic SNVs detected by WES among the rest 142 cases whose CNV-seq results were negative, the total detection rate of genetic abnormalities was 34.2% (67/196). WES increased the diagnostic yield for CTDs by 9.2% (13/142). There was significant difference in the diagnostic yields for different types of CTDs ( χ2=20.31, P=0.002). The diagnostic yield was relatively high for interrupted aortic arch of type B, absent of the pulmonary valve -type of tetralogy of Fallot (9/10 and 8/12), but low for transposition of the great arteries (12.5%, 5/40). Conclusions:CNVs are the common genetic abnormalities in fetal CTDs, and SNVs are also detected in some cases. It is recommended that all fetuses with CTDs should undergo genetic testing. CNV-seq should be used in combination with WES if possible to improve the identification of genetic etiology and provide reference for genetic counseling.

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis

ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

Atividades de Vida Diária, Atividade Física, Aptidão Física e Qualidade de Vida em Crianças com Cardiopatia Congênita: Um Estudo de Caso-Controle / Activities of Daily Living, Physical Activity, Physical Fitness and Quality of Life in Children with Congenital Heart Disease: A Case-Control Study

Resumo Fundamento Apesar dos relatos de redução da aptidão física em crianças com cardiopatia congênita (CC), não foram realizadas avaliações específicas de desempenho para atividades de vida diária. Objetivos O objetivo foi comparar as atividades de vida diária, qualidade de vida, postura, aptidão física e níveis de atividade física entre crianças com CC e controles saudáveis (CS). Métodos O estudo incluiu 30 crianças, de 6 a 14 anos, com diagnóstico de CC moderada ou grave e 30 consideradas CS pareadas por idade e sexo. Os dados sociodemográficos e clínicos dos participantes foram registrados. Todos os participantes realizaram diversos testes: teste de TGlittre-P para atividades de vida diária; teste de caminhada de 6 minutos (TC6M) para capacidade funcional; bateria de testes Fitnessgram para aptidão física; dinamômetro de mão para medir a força de preensão; pedômetro para medir a atividade física; além disso, a criança e os pais completaram o Pediatric Quality of Life Inventory (PedsQL) para avaliação da qualidade de vida, além de análises posturais. Valores de p < 0,05 foram considerados estatisticamente significativos. Resultados Indivíduos com CC apresentaram um tempo de conclusão do teste TGlittre-P mais longo e uma distância de TC6M mais curta em comparação com o CS (TGlittre-P: CC 3,45 [3,24-4,02] min vs. CS 3,10 [2,57-3,23] min, TC6M: CC 514,00 [412,50-566,00] m vs. CS 591,50 [533,00-631,00] m). Para o grupo CC, os resultados dos testes de sit-ups, flexões, elevação do tronco e sentar e alcançar, dentro da bateria do Fitnessgram, além de força de preensão, postura e qualidade de vida foram menores do que os do grupo CS. Os níveis de atividade física foram semelhantes entre os grupos. Conclusões O desempenho das atividades de vida diária, a capacidade funcional, a aptidão física, a postura e a qualidade de vida de crianças com CC moderada e grave foram afetados em comparação com seus pares saudáveis.

Abstract Background Despite reports of reduced physical fitness in children with congenital heart disease (CHD), no specific performance evaluations for activities of daily living have been conducted. Objectives The aim was to compare the activities of daily living, quality of life, posture, physical fitness and physical activity levels of children with CHD with healthy controls (HC). Methods The study included 30 children aged 6-14 diagnosed with moderate or severe CHD and 30 age-sex-matched HC. The sociodemographic and clinical data of the participants were recorded. All participants went through several tests, namely the TGlittre-P test for activities of daily living, the 6-minute walk test (6MWT) for functional capacity, the Fitnessgram test battery for physical fitness, the hand dynamometer for measuring grip strength, the pedometer for measuring physical activity, and both the child and parents reported the Pediatric Quality of Life Inventory (PedsQL) for evaluating the quality of life, in addition to posture analyses. Values of p < 0.05 were considered statistically significant. Results Individuals with CHD had a longer TGlittre-P test completion time and a shorter 6MWT distance than HC (TGlittre-P: CHD 3.45 [3.24-4.02]min vs. HC 3.10 [2.57-3.23]min, 6MWT: CHD 514.00 [412.50-566.00]m vs. HC 591.50 [533.00-631.00]m). For the CHD group, sit-ups, push-ups, trunk lift, and sit-and-reach test scores within the Fitnessgram battery, grip strength, posture, and quality of life scores were lower than those for the HC group. Physical activity levels were similar in the groups. Conclusions The performance of activities of daily living, functional capacity, physical fitness, posture, and quality of life of children with moderate and severe CHD were affected compared to healthy peers.

Pulse oximetry test for screening congenital heart diseases: a systematic review / Prueba de oximetría de pulso para el screening de cardiopatías congénitas: revisión sistemática / Teste de oximetria de pulso para triagem de cardiopatias congênitas: revisão sistemática

ABSTRACT Objective: To determine the accuracy of the Pulse Oximetry Test (POT) in screening for Congenital Heart Diseases (CHD) in newborns in the first 48 hours of life. Method: Systematic review of diagnostic test accuracy with meta-analysis. The selection of studies was carried out in June 2021. Studies were selected with newborns, in a hospital or home environment, without a previous diagnosis of CHD, regardless of gestational age at birth, who underwent POT within the first 48 hours after birth. Registration on the PROSPERO platform - CRD42021256286. Results: Twenty-nine studies were included, totaling a population of 388,491 newborns. POT demonstrated sensitivity of 47% (95% CI: 43% to 50%) and specificity of 98% (95% CI: 98% to 98%). Subgroup analyses were carried out according to the different testing period, inclusion of retests in protocols and population of premature newborns. Conclusion: POT is a test with moderate sensitivity and high specificity. It is more effective when carried out within 24h - 48h of birth; in protocols that present retests, within two hours after the first measurement. It does not show satisfactory effectiveness for premature newborns.

RESUMEN Objetivo: Determinar la precisión de la Prueba de Oximetría de Pulso (POT) en el screening de Cardiopatías Congénitas (CC) en recién nacidos en las primeras 48 horas de vida. Método: Revisión sistemática de la precisión de las pruebas diagnósticas con metanálisis. La selección de estudios se realizó en junio de 2021. Se seleccionaron estudios con recién nacidos, en ambiente hospitalario o domiciliario, sin diagnóstico previo de CC, independientemente de la edad gestacional al nacer, a quienes se les realizó POT dentro de las primeras 48 horas después del nacimiento. Registro en la plataforma PROSPERO - CRD42021256286. Resultados: Se incluyeron 29 estudios, totalizando una población de 388.491 recién nacidos. POT demostró una sensibilidad del 47% (IC del 95%: 43% al 50%) y una especificidad del 98% (IC del 95%: 98% al 98%). Se realizaron análisis de subgrupos según los diferentes períodos de prueba, la inclusión de repruebas en los protocolos y la población de recién nacidos prematuros. Conclusión: POT es una prueba con sensibilidad moderada y especificidad alta. Es más eficaz cuando se realiza entre las 24 y 48 horas siguientes al nacimiento; en protocolos que presenten repruebas, dentro de las dos horas posteriores a la primera medición. No muestra una eficacia satisfactoria para los recién nacidos prematuros

RESUMO Objetivo: Determinar a acurácia do Teste de Oximetria de Pulso (TOP) na triagem de Cardiopatias Congênitas (CC) em recém-nascidos nas primeiras 48 horas de vida. Método: Revisão sistemática de acurácia de teste diagnóstico com metanálise. A seleção dos estudos foi realizada em junho de 2021. Foram selecionados estudos com recém-nascidos, em ambiente hospitalar ou domiciliar, sem o diagnóstico prévio de CC, independentemente da idade gestacional ao nascimento, que realizaram o TOP entre as primeiras 48h após o nascimento. Registro na plataforma PROSPERO - CRD42021256286. Resultados: Foram incluídos 29 estudos, somando uma população total de 388.491 recém-nascidos. O TOP demonstrou sensibilidade de 47% (IC 95%: 43% a 50%) e especificidade de 98% (IC 95%: 98% a 98%). Foram realizadas análises dos subgrupos conforme período de realização do teste diferente, inclusão de retestes nos protocolos e população de recém-nascidos prematuros. Conclusão: O TOP é um teste de moderada sensibilidade e alta especificidade. Apresenta maior efetividade quando realizado no intervalo entre 24h - 48h do nascimento; em protocolos que apresentem retestes, em até duas horas após a primeira medida. Não apresenta efetividade satisfatória para recém-nascidos prematuros.