Echocardiographic and Ultrasonographic Parameters Associated with Protein-losing Enteropathy in Patients with Fontan Physiology: a Systematic Review with Meta-analysis

Abstract Background: Fontan circulation can be associated with significant morbidity, especially Protein-Losing Enteropathy (PLE). Echocardiographic parameters can provide valuable diagnostic information about a patient's risk of developing PLE after Fontan surgery. Objectives: To describe echocardiographic/ultrasonographic parameters associated with PLE in patients after Fontan surgery through a systematic review with meta-analysis. Methods: A literature search was performed in electronic databases to identify relevant studies about echocardiographic parameters and PLE prediction in children after Fontan surgery. The search terms used were: "echocardiography", "ultrasonography", "Fontan," and "protein-losing enteropathy". A p < 0.05 was considered statistically significant. Results: A total of 653 abstracts were obtained from electronic databases and bibliographic references. From these, six articles met criteria to be included in the qualitative analysis and three in the quantitative (meta-analysis). The resistance index in the superior mesenteric artery was described in three studies, and the quantitative analysis showed statistical significance (p < 0.001). Other echocardiographic and ultrasonographic parameters were also described, albeit in single studies not allowing a meta-analysis. Conclusion: This systematic review with meta-analysis identified echocardiographic and ultrasonographic parameters related to PLE in patients with Fontan physiology. Vascular ultrasonography seems to play a prominent role in this aspect, but additional studies are needed to increase the degree of evidence.

Echocardiography Learning by Pediatricians While Screening Congenital Heart Disease with the Aid of Telemedicine.

Background: Congenital heart disease (CHD) affects 1% of all newborns and is a major public health problem. Most children with CHD benefit from early diagnosis to optimize management. However, in many places there are no specialized professionals to do so. Introduction: A screening method for CHD was developed between two states in Northeast Brazil. It was based upon the performance of an echocardiogram by local pediatricians with support of pediatric cardiologists through telemedicine. Objective: To determine if the continuous performance of examinations together with online supervision increased the level of understanding of the pediatricians about the echocardiograms. Methods: Pediatricians received online support to perform echocardiograms for 7 years (2012-2018). Although the project aimed to teach them to acquire images and send to the cardiologists for analysis and interpretation, they were encouraged to describe their own diagnostic impressions. The level of agreement between pediatricians and cardiologists was calculated. Results: A large number (n = 3,951) echocardiograms was analyzed. From them, 2,818 were classified as abnormal, inconclusive, or normal by both the pediatricians and the cardiologists. The 7-year analysis demonstrated an overall agreement of 63.7%. As for the final diagnosis, it was possible to compare 1,457 echocardiograms. The combined analysis of the 7 years demonstrated agreement in 62.2%. Discussion: The screening of CHD under online support led to more in-depth learning of echocardiography by the pediatricians. This approach potentialized the accuracy of the screening through the years. Conclusion: By enrolling in a telemedicine-based screening program, the pediatricians' degree of understanding of echocardiography increased considerably.

A Arte no Ensino da Cardiologia: Relato da Experiência do Uso de Massas Moldáveis no Aprendizado da Anatomia Normal e Patológica do Coração / Learning Cardiology through Art: Modeling Hearts to Understand Normal and Abnormal Anatomy

RESUMO INTRODUÇÃO A anatomia e a fisiologia do coração humano há muito tempo despertam curiosidade, dada a complexidade da morfologia cardíaca, a sua importância para a manutenção da vida e seu grande valor simbólico. O entendimento da fisiologia cardíaca e sua representação nas diversas modalidades artísticas têm ajudado até hoje na formação de profissionais de saúde que trabalham com cardiologia e também na comunicação com familiares. Este artigo apresenta a experiência de um especializando em um serviço de referência no ensino de cardiologia pediátrica, na construção de modelos de corações normais e patológicos em massa de modelar e massa de biscuit, e as implicações no processo de ensino-aprendizagem do grupo de preceptores e residentes da instituição. RELATO DE EXPERIÊNCIA Um residente do primeiro ano de cardiologia pediátrica, sem treinamento ou experiência prévia com artes plásticas, foi estimulado pela preceptoria do programa a moldar corações normais e com cardiopatias congênitas para fins de aprendizado da anatomia interna e externa do órgão. Os materiais utilizados foram massa de modelar e massa de biscuit. RESULTADOS Dez peças foram produzidas ao longo de um ano de especialização, com melhora progressiva nos aspectos artísticos e anatômicos. Entre os modelos patológicos, foram produzidas peças representativas de tetralogia de Fallot, transposição dos grandes vasos, persistência do canal arterial e comunicação interventricular. CONCLUSÃO A experiência proporcionou três efeitos positivos constatados pela equipe: maior entendimento da anatomia cardíaca normal e patológica por parte do especializando envolvido, o qual se refletiu, por exemplo, em melhor compreensão de imagens médicas, como ecocardiografia, tomografia computadorizada e ressonância magnética cardíacas, maior aproximação dos residentes do programa com as artes plásticas em uma especialidade que sempre teve uma relação muito estreita com o desenho e a escultura, e produção de modelos em três dimensões de estruturas normais e patológicas que podem ser usadas em aulas e encontros científicos para discussão da disciplina.

ABSTRACT INTRODUCTION The anatomy and physiology of the human heart has long captivated the curiosity of human beings, due to its complexity, its importance for maintaining life, and its great symbolic value. The understanding of cardiac physiology and its representations in different art forms has assisted in the training of health professionals, particularly those working in cardiology, as well as communication with families.This article presents the experience of a pediatric cardiology resident using modeling clays to build normal and abnormal heart models, and discusses the implications of this practice in the teaching-learning process among staff and trainees of the institution. CASE REPORT A first year paediatric cardiology resident, with no previous training or experience in the plastic arts, was encouraged by Head of Department to mould hearts with normal anatomy and with congenital heart disease for the purpose of teaching the internal and external anatomy of the organ. The materials used were plasticine and cold porcelain clay. RESULTS Ten anatomical pieces were produced over a one-year period, with progressive improvements in artistic and anatomical aspects. Among the pathological models, there were pieces demonstrating the tetralogy of Fallot, transposition of the great vessels, patent ductus arteriosus, and ventricular septal defect. CONCLUSION The experience lead to three positive effects noted by the medical team: a better understanding of normal and abnormal cardiac anatomy by the residents, evidenced by their rapid improvement in interpreting medical images such as echocardiography, computerized tomography and nuclear magnetic resonance images; greater access of residents to the plastic arts, in a medical specialty that has always benefited from close proximity with drawings and sculpture; and the production of very low-cost 3D models of normal and abnormal cardiac anatomy that can be used for lectures, clinical-surgical and scientific meetings, and teaching future students.

Development and evaluation of a rapid molecular diagnostic test for Zika virus infection by reverse transcription loop-mediated isothermal amplification.

The recent outbreak of Zika virus (ZIKV) disease caused an enormous number of infections in Central and South America, and the unusual increase in the number of infants born with microcephaly associated with ZIKV infection aroused global concern. Here, we developed a reverse transcription loop-mediated isothermal amplification (RT-LAMP) assay using a portable device for the detection of ZIKV. The assay specifically detected ZIKV strains of both Asian and African genotypes without cross-reactivity with other arboviruses, including Dengue and Chikungunya viruses. The assay detected viral RNA at 14.5 TCID50/mL in virus-spiked serum or urine samples within 15 min, although it was slightly less sensitive than reference real time RT-PCR assay. We then evaluated the utility of this assay as a molecular diagnostic test using 90 plasma or serum samples and 99 urine samples collected from 120 suspected cases of arbovirus infection in the states of Paraíba and Pernambuco, Brazil in 2016. The results of this assay were consistent with those of the reference RT-PCR test. This portable RT-LAMP assay was highly specific for ZIKV, and enable rapid diagnosis of the virus infection. Our results provide new insights into ZIKV molecular diagnostics and may improve preparedness for future outbreaks.

Gender differences in the prevalence of congenital heart disease in Down's syndrome: a brief meta-analysis.

BACKGROUND: Down's syndrome (DS) affects one per 700 live births and congenital heart disease (CHD) occurs in 40-60% of these patients. Contributing factors to the association between DS and CHD are being unraveled. Gender could be one of them. METHODS: We performed a meta-analysis of CHD prevalence in DS, separated by gender. Three search engines were used and 578 articles were reviewed. Twelve articles were included. RESULTS: Quantitative analysis showed a higher prevalence of CHD, particularly atrioventricular septal defects (AVSD), in female patients. No differences were found in others forms of CHD. CONCLUSION: CHD, particularly AVSD, are more common in the female gender of Down's syndrome patients.

Microcephaly in north-east Brazil: a retrospective study on neonates born between 2012 and 2015.

OBJECTIVE: To assess the number of children born with microcephaly in the State of Paraíba, north-east Brazil. METHODS: We contacted 21 maternity centres belonging to a paediatric cardiology network, with access to information regarding more than 100 000 neonates born between 1 January 2012 and 31 December 2015. For 10% of these neonates, nurses were requested to retrieve head circumference measurements data from delivery-room books. We used three separate criteria to classify whether a neonate had microcephaly: (i) the Brazilian Ministry of Health proposed criterion: term neonates (gestational age ≥ 37 weeks) with a head circumference of less than 32 cm; (ii) Fenton curves: neonates with a head circumference of less than -3 standard deviation for age and gender; or (iii) the proportionality criterion: neonates with a head circumference of less than ((height/2))+10) ± 2. FINDINGS: Between 1 and 31 December 2015, nurses obtained data for 16 208 neonates. Depending on which criterion we used, the number of neonates with microcephaly varied from 678 to 1272 (4.2-8.2%). Two per cent (316) of the neonates fulfilled all three criteria. We observed temporal fluctuations of microcephaly prevalence from late 2012. CONCLUSION: The numbers of microcephaly reported here are much higher than the 6.4 per 10 000 live births reported by the Brazilian live birth information system. The results raise questions about the notification system, the appropriateness of the diagnostic criteria and future implications for the affected children and their families. More studies are needed to understand the epidemiology and the implications for the Brazilian health system.

Impact of Telemedicine in the Screening for Congenital Heart Disease in a Center from Northeast Brazil.

OBJECTIVES: To describe the incidence of congenital heart disease before and after the establishment of a telemedicine screening program, in a reference center from Northeast Brazil. METHODS: This is a descriptive, retrospective and comparative study based on the institutional data from a reference center in perinatology for a period of 16 years. Institutional data were collected from a 16-year period (2001-15). Data were divided into two periods: prior to (2001-11) and after (2012-15) the establishment of a telemedicine screening program. RESULTS: After the implementation of the screening process, almost all kinds of heart disease showed a significant increase in their incidence (p < 0.05). With this, the incidence of major heart diseases approached those specified in developed regions. CONCLUSION: The implementation of a screening process model for congenital heart diseases can change the context of patients with congenital heart diseases in poor regions.

Diagnosis and management of Transposition of great arteries within a pediatric cardiology network with the aid of telemedicine: A case report from Brazil.

We present a case of a newborn from a remote, underserved area in the inland of Paraíba, a state from Northeast Brazil. She presented with clinical cyanosis at birth. With the aid of telemedicine, a neonatologist under online cardiology supervision performed a screening echocardiogram. The session established the diagnosis of simple transposition of the great vessels in the baby's first few hours of life. During the same telemedicine session, the necessary arrangements for transferal to a larger maternity center took place. The baby was maintained stable on prostaglandins and was subsequently transferred to a tertiary cardiac center in the neighboring State, Pernambuco. She underwent anatomical correction at day 10, presented no surgical or postoperative complications, and was discharged home at the age of 21 days. She is now over three years old and continues her follow-up care mostly at her hometown, with local pediatricians under online supervision by a cardiologist in a virtual outpatient clinic. The establishment of a Pediatric Cardiology Network, with the aid of telemedicine, can produce a major impact on the access to specialized health care for poor regions of developing countries.

A telemedicine network for remote paediatric cardiology services in north-east Brazil.

PROBLEM: Providing health care for children with congenital heart diseases remains a major challenge in low- and middle-income countries. APPROACH: In October 2011, the Government of Paraíba, Brazil, established a paediatric cardiology network in partnership with the nongovernmental organization Círculo do Coração. A cardiology team supervised all network activities, using the Internet to keep in contact with remote health facilities. The network developed protocols for screening heart defects. Echocardiograms were performed by physicians under direct online supervision of a cardiologist; alternatively, a video recording of the examination was subsequently reviewed by a cardiologist. Cardiovascular surgeons came to a paediatric hospital in the state capital once a week to perform heart surgeries. LOCAL SETTING: Until 2011, the State of Paraíba had no structured programme to care for children with heart disease. This often resulted in missed or late diagnosis, with adverse health consequences for the children. RELEVANT CHANGES: From 2012 to 2014, 73,751 babies were screened for heart defects and 857 abnormalities were identified. Detection of congenital heart diseases increased from 4.09 to 11.62 per 1000 live births (P < 0.001). Over 6000 consultations and echocardiograms were supervised via the Internet. Time to diagnosis, transfers and hospital stays were greatly reduced. A total of 330 operations were carried out with 6.7% (22/330) mortality. LESSONS LEARNT: Access to an echocardiography machine with remote supervision by a cardiologist improves the detection of congenital heart disease by neonatologists; virtual outpatient clinics facilitate clinical management; the use of Internet technology with simple screening techniques allows resources to be allocated more efficiently.

Impact of a Virtual Clinic in a Paediatric Cardiology Network on Northeast Brazil.

Introduction. Congenital heart diseases (CHD) affect approximately 1% of live births and is an important cause of neonatal morbidity and mortality. Despite that, there is a shortage of paediatric cardiologists in Brazil, mainly in the northern and northeastern regions. In this context, the implementation of virtual outpatient clinics with the aid of different telemedicine resources may help in the care of children with heart defects. Methods. Patients under 18 years of age treated in virtual outpatient clinics between January 2013 and May 2014 were selected. They were divided into 2 groups: those who had and those who had not undergone a screening process for CHD in the neonatal period. Clinical and demographic characteristics were collected for further statistical analysis. Results. A total of 653 children and teenagers were treated in the virtual outpatient clinics. From these, 229 had undergone a neonatal screening process. Fewer abnormalities were observed on the physical examination of the screened patients. Conclusion. The implementation of pediatric cardiology virtual outpatient clinics can have a positive impact in the care provided to people in areas with lack of skilled professionals.

Oximetria de pulso em triagem de cardiopatias congênitas: conhecimento e atuação do enfermeiro / Pulse oximetry in triaging congenital cardiopathies: the nurse's knowledge and role / Oximetría de pulso en selección de cardiopatías congénitas: conocimiento y actuación del enfermero

A oximetria de pulso nas unidades neonatais apresenta-se como um processo de triagem de cardiopatias congênitas graves. O estudo tem como objetivo avaliar o nível de informação e a atuação dos enfermeiros na oximetria de pulso realizada nos recém-nascidos de uma maternidade pública do município de João Pessoa, Paraíba. Trata-se de estudo descritivo com abordagem quanti-qualitativa, realizado com 13 enfermeiras assistenciais através de entrevista semiestruturada no período de janeiro a março de 2015. Os resultados mostraram que a maioria das enfermeiras tem conhecimento sobre o teste do coraçãozinho, a justificativa para realizá-lo, os parâmetros de normalidades da saturação de oxigênio, assim como as condutas que devem ser tomadas diante de um resultado alterado. No entanto, 84,6% referiram dificuldades na sua implantação em virtude de modificar a rotina da assistência de enfermagem. Acredita-se que o teste do coraçãozinho para triagem de cardiopatias congênitas traz bons resultados na captação precoce dessas malformações (AU).

Pulse oximetry in the neonatal units is presented as a process for triaging serious congenital cardiopathies. The study aims to assess the level of information, and the work, of the nurses in pulse oximetry undertaken on the newborns in a public maternity unit in the municipality of João Pessoa, Paraíba. It is a descriptive study with a quanti-qualitative approach, undertaken with 13 staff nurses through the use of a semistructured interview in January ­ March 2015. The results showed that the majority of the nurses have knowledge regarding the 'little heart test' (teste do coraçãozinho), the rationale for undertaking it, and the normal parameters for oxygen saturation, as well as the conducts to be taken in the event of a result outside these parameters. However, 84.6% mentioned difficulties in implementing it due to having to alter the routine of the nursing care. It is believed that the little heart test for triaging congenital cardiopathies brings good results in the early identification of these malformations (AU).

La oximetría de pulso en las unidades neonatales se presenta como un proceso de selección de cardiopatías congénitas severas. El estudio tiene como finalidad evaluar el nivel de información y la actuación de los enfermeros en la oximetría de pulso realizada en recién nacidos de una maternidad pública del municipio de João Pessoa, Paraíba. Es un estudio descriptivo con abordaje cuantitivo cualitativo, realizado con 13 enfermeras asistenciales a través de entrevista semiestructurada en el periodo de enero a marzo de 2015. Los resultados mostraron que la mayoría de las enfermeras tiene conocimiento sobre el test del corazoncito, la justificativa para realizarlo, los parámetros de normalidad de la saturación de oxígeno, así como las conductas delante de un resultado alterado. Sin embargo, 84,6% hablaron de dificultades en su implantación por el hecho de cambiarse la rutina de la asistencia de enfermería. Se cree que el test del corazoncito para selección de cardiopatías congénitas trae buenos resultados en la captación precoz de esas malas formaciones (AU).

Comparison of different screening methods for blood pressure disorders in children and adolescents / Comparação entre diferentes métodos de rastreamento para distúrbios da pressão arterial em crianças e adolescentes

OBJECTIVE: To compare different methods of screening for blood pressure disorders in children and adolescents. METHOD: A database with 17,083 medical records of patients from a pediatric cardiology clinic was used. After analyzing the inclusion and exclusion criteria, 5,650 were selected. These were divided into two age groups: between 5 and 13 years and between 13 and 18 years. The blood pressure measurement was classified as normal, pre-hypertensive, or hypertensive, consistent with recent guidelines and the selected screening methods. Sensitivity, specificity, and accuracy were then calculated according to gender and age range. RESULTS: The formulas proposed by Somu and Ardissino's table showed low sensitivity in identifying pre-hypertension in all age groups, whereas the table proposed by Kaelber showed the best results. The ratio between blood pressure and height showed low specificity in the younger age group, but showed good performance in adolescents. CONCLUSION: Screening tools used for the assessment of blood pressure disorders in children and adolescents may be useful to decrease the current rate of underdiagnosis of this condition. The table proposed by Kaelber showed the best results; however, the ratio between BP and height demonstrated specific advantages, as it does not require tables. .

OBJETIVO: Comparar diferentes métodos de rastreamento para distúrbios da pressão arterial em crianças e adolescentes. MÉTODO: Foi usado um banco de dados com 17.083 prontuários de pacientes de uma clínica de cardiologia pediátrica. Após análise dos critérios de inclusão e exclusão, 5.650 foram selecionados. Esses foram divididos em duas faixas etárias: entre cinco e 13 anos e entre 13 e 18 anos De acordo com a aferição, a pressão arterial era classificada como normal, pré-hipertensiva ou hipertensiva de acordo com guidelines recentes e os métodos de rastreamento selecionados. Posteriormente, foram calculadas a sensibilidade, especificidade e acurácia de cada um de acordo com o gênero e faixa etária. RESULTADOS: As fórmulas de Somu e a tabela proposta por Ardissino apresentaram baixa sensibilidade na identificação de pré-hipertensão em todas as faixas etárias, enquanto a tabela proposta por Kaelber apresentou os melhores resultados. A razão entre pressão arterial e altura apresentou baixa especificidade na faixa etária menor, mas apresentou bom desempenho em adolescentes. CONCLUSÃO: As ferramentas de rastreamento para distúrbios da pressão arterial em crianças e adolescentes podem ser úteis para diminuir o subdiagnóstico que ocorre atualmente nessa condição. A tabela proposta por Kaelber apresentou os melhores resultados, entretanto a razão entre PA e altura apresenta vantagens específicas, como a não necessidade de tabelas. .

Busca ativa por cardiopatias congênitas é factível? Experiência em oito cidades Brasileiras / Active search for congenital heart disease is possible? Experience from eight brazilian cities

Fundamentos: As cardiopatias congênitas são causa de alta morbimortalidade na faixa etária pediátrica. Em regiões em desenvolvimento, a falta de diagnóstico precoce pode acarretar a piora do quadro; uma busca ativa por cardiopatias congênitas pode colaborar na resolução dessa situação.Objetivo: Descrever os principais achados de uma busca ativa por cardiopatias na infância, realizada em oito cidades brasileiras do estado da Paraíba. Métodos: Estudo realizado em oito cidades brasileiras do estado da Paraíba. A busca ativa compreendeu duas fases. Na primeira, os centros de saúde locais realizaram a triagem de crianças com sintomas ou história clínica de doenças cardiovasculares. Na segunda fase, realizada uma consulta clínica com posterior realização de ecocardiograma para identificação de cardiopatias. As frequências obtidas foram analisadas. Variáveis categóricas foram comparadas utilizando-se o teste do qui-quadrado. Resultados: Atendidas 440 crianças. Destas, 192 (43,63%) apresentaram alterações ao ecocardiograma. As presenças de sopro e da síndrome de Down mostraram correlação significativa (p<0,05) com a cardiopatia congênita. A maioria dos casos era de cardiopatia congênita por shunt (64,1%), seguida pelos defeitos valvares (12,5%) e pelas cardiopatias obstrutivas acianogênicas (8,3%). Conclusão: A busca ativa realizada foi capaz de identificar um número expressivo de cardiopatias congênitas na infância, não reconhecidas previamente, e inserir esses pacientes nos serviços de saúde para o tratamento adequado, garantindo-lhes melhor qualidade de vida.

Background: Congenital heart defects are the cause of high morbidity and mortality in pediatric patients. In developing regions, the lack of early diagnosis may lead to worsening of the condition; an active search for congenital heart diseases can assist in resolving this situation. Objective: To describe the key findings of an active search for heart diseases in childhood, held in eight Brazilian cities in the state of Paraíba. Methods: A study conducted in eight Brazilian cities in the state of Paraíba. The active search included two phases. In the first, local health centers screened children with symptoms or a history of cardiovascular diseases. In the second phase, a clinical consultation with echocardiogram to identify diseases was conducted. The frequencies were then analyzed. Categorical variables were compared using the chi-square test. Results: 440 children were assisted. Of these, 192 (43.63%) had abnormalities on echocardiogram. The presence of heart sounds and Down’s syndrome showed a significant correlation (p <0.05) with the congenital heart disease. Most cases were congenital heart disease by shunt (64.1%), followed by valve defects (12.5%) and acyanogenic obstructive heart diseases (8.3%). Conclusion: The active search conducted was able to identify a significant number of congenital heart diseases in childhood not previously recognized and inserting these patients in health services for appropriate treatment, thus ensuring them a better quality of life.