Abstract Background In the pediatric population, syncope is mainly from vasovagal (VVS) origin. Its evaluation must be done by clinical methods, and the tilt test (TT) can contribute to the diagnosis. Objectives To analyze the clinical profile, Calgary and modified Calgary scores, response to TT and heart rate variability (HRV) of patients aged ≤ 18 years with presumed VVS. To compare the variables between patients with positive and negative responses to TT. Method Observational and prospective study, with 73 patients aged between 6 and 18 years, submitted to clinical evaluation and calculation of scores without previous knowledge of the TT. It was done at 70º under monitoring for HRV analysis. P-value < 0.05 was the statistical significance criterion. Results Median age was 14.0 years; 52% of participants were female, 72 had Calgary ≥ -2 (mean 1.80), and 69 had modified Calgary ≥ -3 (mean 1.38). Prodromes were observed in 59 patients, recurrence in 50 and trauma in 19. The response to TT was positive in 54 participants (49 vasovagal, with 39 vasodepressor responses), with an increase in the low frequency (LF) component and a decrease in the high frequency (HF) component (p < 0,0001). In the supine position, LF was 33.6 in females and 47.4 in normalized units for males (p = 0.02). When applying the operating characteristic curve for positive TT, there was no statistical significance for HRV and scores. Conclusion Most children and adolescents with a presumed diagnosis of VVS presented a typical clinical scenario, with a Calgary score ≥ -2, and a predominant vasodepressor response to TT. Greater sympathetic activation was observed in the supine position in males. Calgary scores and sympathetic activation did not predict the response to TT.

Resumo Fundamento A síncope, na população pediátrica, tem como sua principal causa, a vasovagal (SVV). Sua avaliação deve ser feita por métodos clínicos e o teste de inclinação (TI) pode contribuir para seu diagnóstico. Objetivos Analisar o perfil clínico, os escores de Calgary e de Calgary modificado, a resposta ao TI e a variabilidade da frequência cardíaca (VFC) de pacientes ≤ 18 anos de idade, com presumida SVV. Comparar as variáveis entre pacientes com resposta positiva e negativa ao TI. Método Estudo observacional e prospectivo, com 73 pacientes com idades entre 6 e 18 anos, submetidos à avaliação clínica e ao cálculo dos escores, sem o conhecimento do TI. Este foi feito a 70º sob monitoramento para análise da VFC. Valor-p < 0,05 foi considerado como o critério de significância estatística. Resultados A mediana de idade foi de 14,0 anos, sendo que 52% eram no sexo feminino, 72 apresentaram Calgary ≥ -2 (média 1,80) e 69 com Calgary modificado ≥ -3 (média 1,38). Ocorreram pródromos em 59 pacientes, recorrência em 50 e trauma em 19. A resposta ao TI foi positiva em 54 (49 vasovagal, com 39 vasodepressora), com aumento do componente de baixa frequência (BF) e diminuição da alta frequência (AF) (p < 0,0001). Na posição supina, o BF foi de 33,6 no sexo feminino e 47,4 em unidades normalizadas no sexo masculino (p = 0,02). Aplicando-se a curva de operação característica para TI positivo, não houve significância estatística para VFC e os escores. Conclusões A maioria das crianças e adolescentes com diagnóstico presumido de SVV apresentaram um cenário clínico típico, com escore de Calgary ≥ -2, e resposta vasodepressora predominante ao TI. Verificou-se uma maior ativação simpática na posição supina no sexo masculino. Os escores de Calgary e a ativação simpática não permitiram predizer a resposta ao TI.

Abstract Background: Left isomerism (LI) is a common finding in patients with biliary atresia (BA), and it can be identified by echocardiography. Several comorbidities may be present in patients with LI, including heart disease. Objective: To investigate the prevalence of LI and heart disease in children (< 18 years of age) with BA followed-up at Hospital das Clínicas, UFMG. Methods: This is a cross-sectional study involving patients diagnosed with BA between February 2016 and April 2020 who underwent transthoracic echocardiography and, in case of situs abnormalities, also electrocardiography. Results: Our study recruited 58 patients (mean age: 3.08 years; female/male ratio: 1.5:1). The general prevalence of situs abnormalities was 8.6% (5/58) and the most common one was LI (4/5 or 80%). One patient had situs inversus. Among patients with situs abnormalities, the general prevalence of heart disease was 80% (4/5), apart from anomalies of the inferior vena cava), with pulmonary valve stenosis (PVS) as the only change seen (75% of mild forms and 25% of moderate forms). Among patients with situs abnormalities, the prevalence of rhythm changes was 80% (4/5), and low atrial rhythm was the most common finding (3/4 or 75%). Conclusion: The prevalence of situs abnormalities in our sample was similar to that described in the literature. We observed an exclusive prevalence of PVS and a high prevalence of rhythm changes among patients with LI. Although the diagnosis of isomerism does not initially add much cardiovascular risk to the sample, possible late deterioration should be considered.

Resumo Fundamento: A insuficiência cardíaca é principal manifestação da cardiomiopatia dilatada na infância, e a avaliação sistematizada dos sinais e sintomas permite acompanhar o resultado do tratamento. Objetivo: Avaliar o uso de três escalas de classificação funcional da insuficiência cardíaca na evolução de crianças e adolescentes com cardiomiopatia dilatada. Métodos: Estudo longitudinal e observacional incluindo pacientes de zero a 18 anos com cardiomiopatia dilatada e insuficiência cardíaca inicial grave. Todos foram acompanhados por meio das escalas New York Heart Association (NYHA), The New York University Pediatric Heart Failure Index (The NYU PHFI) e Ross versão 2012. As análises estatísticas foram feitas com uso do Statistical Package for Social Science, versão 14.0, com teste de Mann-Whitney, teste qui quadrado ou de Fisher, aplicação da Curva Característica de Operação, teste de Wilcoxon e coeficiente de Kappa, para comparação das escalas, e curva Kaplan-Meier, para avaliação da sobrevida. O nível de significância adotado foi de 5%. Resultados: Foram incluídos 57 pacientes, com idade de 1 a 200 meses (média de 48,7 ± 55,9) e seguimento de 6 a 209 meses (média de 63,6 ± 48,4). Houve substancial concordância das escalas Ross 2012 e The NYU PHFI com a NYHA (Kappa = 0,71 e 0,82, respectivamente). A análise pareada pelo teste de Wilcoxon, comparando as escalas antes e após o tratamento, foi significativa (p < 0,0001). A sobrevida maior foi encontrada nos pacientes com classes I/II pela NYHA ou pontuação menor que 11 pontos nas outras. Conclusão: O uso das escalas de avaliação funcional da insuficiência cardíaca se mostrou útil no seguimento e na avaliação da resposta terapêutica. Pacientes que permaneceram em classes funcionais III ou IV de NYHA, ou com pontuação igual ou maior que 11 na Ross 2012 ou na The NYU PHFI tiveram pior prognóstico.

Abstract Background: Heart failure is the main manifestation of dilated cardiomyopathy in childhood, and the systematic evaluation of signs and symptoms allows monitoring the treatment outcome. Objective: To evaluate the use of three functional classification scales of heart failure in children and adolescents with dilated cardiomyopathy. Methods: Longitudinal and observational study including patients from zero to 18 years with dilated cardiomyopathy and severe initial heart failure. All of them were followed up using the New York Heart Association (NYHA), The New York University Pediatric Heart Failure Index (The NYU PHFI) and Ross version 2012 scales. Statistical analyzes were done using Statistical Package for Social Science, version 14.0, with Mann-Whitney test, Chi-Square test or Fisher's test, application of the Operating Characteristic Curve, Wilcoxon test and Kappa coefficient for comparison of scales and Kaplan-Meier curve for survival evaluation. The level of significance adopted was 5%. Results: A total of 57 patients, aged from 1 to 200 months (mean of 48.7 ± 55.9) and follow-up of 6 to 209 months (mean of 63.6 ± 48.4) were included. There was substantial agreement between the Ross 2012 scales, The NYU PHFI and NYHA (Kappa = 0.71 and 0.82, respectively). Paired analysis by the Wilcoxon test, comparing the scales before and after treatment, was significant (p < 0.0001). The greatest survival was found in patients with class I/II by NYHA or scores lower than 11 points in the others. Conclusion: The use of functional assessment scales of heart failure proved to be useful in the follow-up and evaluation of the therapeutic response and there was no difference between them. Patients who remained in functional classification III or IV NYHA or scores ≥ 11 in Ross 2012 or The NYU PHFI had worse prognosis.