Electrocardiographic and Autonomic Nervous System Changes after Changes in the Posture of Children and Adolescents with Duchenne Muscular Dystrophy. / Alterações Eletrocardiográficas e do Sistema Nervoso Autônomo com a Mudança de Postura em Crianças e Adolescentes com Distrofia Muscular de Duchenne.

BACKGROUND: Duchenne Muscular Dystrophy (DMD) is a rare inherited neuromuscular disease. At first, cardiac involvement may be asymptomatic. Therefore, assessing patients using non-invasive methods can help detect any changes. OBJECTIVES: Analyze the electrocardiogram (ECG) test and heart rate variability (HRV) of the DMD group and compare the information with that of the age-matched control group. METHODS: A prospective study with 27 male patients with DMD (11.9 years old), who underwent clinical evaluation, ECG, echocardiogram, and Holter monitoring. ECG (200% increase) was assessed by two independent observers. HRV was measured over time (24 h) and in the frequency domain, in the supine and sitting positions. The healthy group consisted of nine patients (11.0 years old). A value of p < 0.05 was considered statistically significant. RESULTS: The mean ejection fraction (EF) was 60% (34 to 71%). The Kappa coefficient for ECG measurements ranged from 0.64 to 1.00. An increase in the R/S ratio in V1 was observed in 25.9% of the subjects, pathological Q wave in 29.6%, and fragmented QRS in 22.2% in inferior/high lateral regions, with a negative correlation with EF (p = 0.006). There was low HRV, without the influence of any variable, including treatment. With the change in position, there was an increase in HR (p = 0.004), but there was no change in HRV. The LF/HF ratio was 2.7 in the DMD group and 0.7 in the control group (p = 0.002). CONCLUSIONS: In DMD subjects, prominent R waves in V1 and changes in the inferior/high lateral regions occurred in almost 30% of the cases. Lower vagal tone was observed without the influence of the variables age, ejection fraction, QT dispersion, and treatment. Despite the increase in HR, there was no adequate HRV response to the change in position.

FUNDAMENTO: Distrofia Muscular de Duchenne (DMD) é uma doença neuromuscular hereditária rara. O acometimento cardíaco inicial pode ser assintomático. Portanto, a avaliação por métodos não invasivos pode auxiliar sua abordagem. OBJETIVOS: Analisar o eletrocardiograma (ECG) e a variabilidade da frequência cardíaca (VFC) do grupo com DMD, e comparar com a do grupo controle pareado por idade. MÉTODOS: Estudo prospectivo com 27 pacientes masculinos com DMD (idade de 11,9 anos) que foram submetidos à avaliação clínica, ECG, ecocardiograma e Holter. ECG (aumento de 200%) foi avaliado por dois observadores independentes. VFC foi feita no domínio do tempo (24 h) e da frequência na posição supina e sentada. O grupo saudável foi de nove pacientes (11,0 anos). Um valor de p < 0,05 foi considerado estatisticamente significante. RESULTADOS: A média da fração de ejeção (FE) foi de 60% (34 a 71%). O coeficiente de Kappa para as medidas do ECG variou de 0,64 a 1,00. Foram verificados aumento da relação R/S em V1 em 25,9%, onda Q patológica em 29,6% e QRS fragmentado em 22,2% em regiões inferior/lateral alta, este com correlação negativa com FE (p = 0,006). Houve baixa VFC, sem influência de nenhuma variável, inclusive tratamento. Com a mudança da posição, houve aumento da FC (p = 0,004), porém não houve alteração da VFC. A relação LF/HF foi de 2,7 na DMD e de 0,7 no controle (p = 0,002). CONCLUSÕES: Nos participantes com DMD, as ondas R proeminentes em V1 e alterações nas regiões inferior/lateral alta ocorreram em quase 30% dos casos. Houve menor tônus vagal sem influência das variáveis idade, fração de ejeção, dispersão do QT e tratamento. Apesar do aumento da FC, não houve resposta adequada da VFC com a mudança de posição.

Clinical and Autonomic Profile, and Modified Calgary Score for Children and Adolescents with Presumed Vasovagal Syncope Submitted to the Tilt Test. / Perfil Clínico, Autonômico e Escore de Calgary Modificado de Crianças e Adolescentes com Presumida Síncope Vasovagal Submetidos ao Teste de Inclinação.

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.

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.

Perfil Clínico, Autonômico e Escore de Calgary Modificado de Crianças e Adolescentes com Presumida Síncope Vasovagal Submetidos ao Teste de Inclinação / Clinical and Autonomic Profile, and Modified Calgary Score for Children and Adolescents with Presumed Vasovagal Syncope Submitted to the Tilt Test

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 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.

Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia

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.

A Novel SPEG mutation causing congenital myopathy with fiber size disproportion and dilated cardiomyopathy with heart transplantation.

Congenital myopathies are a heterogeneous group of conditions diagnosed based on the clinical presentation, muscle histopathology and genetic defects. Recessive mutations in the SPEG gene have been described in recent years and are primarily associated with centronuclear myopathy with cardiomyopathy. In this report, we describe two Brazilian siblings, aged 13 and 6 years, with a novel homozygous mutation (c.8872 C>T:p.Arg2958Ter) in the SPEG gene leading to a congenital myopathy. In the older sibling, the muscle biopsy showed fiber size disproportion. The mean diameter of type 2 fibers (119 µm) was significantly higher than type 1 (57 µm) (P < 0,001) with a 72% prevalence of type 1 fibers. The patient also had progressive cardiomyopathy treated with heart transplantation. The present report expands the muscle histopathological findings related to mutations in the SPEG gene, including fiber size disproportion without central nuclei. Additionally, this report describes the first case of heart transplantation in a patient with SPEG mutations.

Pulmonary arterial pressure and nasal obstruction in mouth-breathing children: Similarities between adenotonsillar hypertrophy and allergic rhinitis.

BACKGROUND: Upper airway obstruction may cause pulmonary hypertension in childhood. In this study we aimed to identify a possible correlation of systolic pulmonary arterial pressure (SPAP), using Doppler echocardiography, with nasal patency (NP), as measured by rhinomanometry, in mouth-breathing (MB) children with allergic rhinitis (AR) and adenotonsillar hypertrophy (ATH). METHODS: In this cross-sectional study we evaluated 183 patients, from 2 to 12 years of age, at an MB referral clinic in Brazil, from December 2013 to 2017. We allocated patients to 4 etiology groups: group 1, 60 MBs with ATH; group 2, 47 MBs with AR; group 3, 43 MBs with both ATH and AR; and group 4, 33 nasal breathing control subjects. The ratio of total nasal inspiratory flow (assessed by active anterior rhinomanometry) and expected inspiratory flow adjusted for height determined the percent NP (%NP). RESULTS: The median %NP was higher in controls than in the MB groups (controls, 114% [79-147%]; ATH: 65% [5-116%]; AR: 57% [23-144%]; ATH and AR: 64% [3-120%]; p < 0.001). Median SPAP was higher in the MB groups than in controls (SPAP: ATH, 26.0 [20.0-35.0] mmHg; AR, 26.0 [22.0-32.0] mmHg; ATH and AR, 26.30 [20.0-34.0] mmHg; control, 22.0 [16.0-30.0] mmHg; p < 0.001). SPAP showed a negative association with %NP (Spearman's rho = -0.24; p < 0.001). CONCLUSION: Reduced nasal airflow in MB children showed a correlation with higher levels of systolic pulmonary arterial pressure. The AR and ATH groups were similar in nasal obstruction severity and systolic pulmonary arterial pressure level distribution.

Validation of a simplified score for predicting latent rheumatic heart disease progression using a prospective cohort of Brazilian schoolchildren.

OBJECTIVES: Echocardiographic (echo) screening is an important tool to estimate rheumatic heart disease (RHD) prevalence, but the natural history of screen-detected RHD remains unclear. The PROVAR+ (Programa de RastreamentO da VAlvopatia Reumática) study, which uses non-experts, telemedicine and portable echo, pioneered RHD screening in Brazil. We aimed to assess the mid-term evolution of Brazilian schoolchildren (5-18 years) with echocardiography-detected subclinical RHD and to assess the performance of a simplified score consisting of five components of the World Heart Federation criteria, as a predictor of unfavourable echo outcomes. SETTING: Public schools of underserved areas and private schools in Minas Gerais, southeast Brazil. PARTICIPANTS: A total of 197 patients (170 borderline and 27 definite RHD) with follow-up of 29±9 months were included. Median age was 14 (12-16) years, and 130 (66%) were woman. Only four patients in the definite group were regularly receiving penicillin. PRIMARY AND SECONDARY OUTCOME MEASURES: Unfavourable outcome was based on the 2-year follow-up echo, defined as worsening diagnostic category, remaining with mild definite RHD or development/worsening of valve regurgitation/stenosis. RESULTS: Among patients with borderline RHD, 29 (17.1%) progressed to definite, 49 (28.8%) remained stable, 86 (50.6%) regressed to normal and 6 (3.5%) were reclassified as other heart diseases. Among those with definite RHD, 13 (48.1%) remained in the category, while 5 (18.5%) regressed to borderline, 5 (18.5%) regressed to normal and 4 (14.8%) were reclassified as other heart diseases. The simplified echo score was a significant predictor of RHD unfavourable outcome (HR 1.197, 95% CI 1.098 to 1.305, p<0.001). CONCLUSION: The simple risk score provided an accurate prediction of RHD status at 2-year follow-up, showing a good performance in Brazilian schoolchildren, with a potential value for risk stratification and monitoring of echocardiography-detected RHD.

Acetaminophen in low doses for closure of the ductus arteriosus of the premature.

OBJECTIVE: The objective of the study is to report the experience with acetaminophen in low doses as an alternative to the treatment of the ductus arteriosus of the preterm newborn. MATERIALS AND METHODS: Retrospective study including preterm newborns with patent ductus arteriosus who received oral acetaminophen because treatment with indomethacin had failed or is contraindicated. A dosage consisted of a first dose of 25 mg/kg and maintenance doses of 30 mg/kg/day, for 3 to 7 days. A second cycle was administered in cases of reopening of the ductus arteriosus. The rates of ductal closure and surgery were calculated. Patients were categorized into responder and nonresponder groups for acetaminophen, and the average values of ductal diameter, weight, gestational age, and postnatal age were compared. RESULTS: Eighty-seven preterm newborns, with a postnatal age from 3 to 27 days, with average values of ductus arteriosus equal to 2.5 ± 0.8 mm/kg, gestational age 27.2 ± 1.9 weeks, and birth weight 888.9 ± 241 g, received acetaminophen for 3 to 7 days. A second cycle was administered in 15 preterm newborns. The ductus closure rate, after one or two cycles, was 74.7%, and the recommendations for surgical closure were progressively reduced from 50% in the 1st year to 6.2% in the past year. Lower ductal closure rate occurred in the group of newborns with the lowest average weight (P = 0.018), the highest average ductal diameter (P = 0.002), and the lowest average gestational age (P = 0.09). Postnatal age at the start of acetaminophen use was shown to be irrelevant regarding the treatment (P = 0.591). CONCLUSIONS: Acetaminophen in low doses showed to be an effective alternative for the closure of the ductus arteriosus for preterm newborns in whom treatment with indomethacin or ibuprofen failed or was contraindicated.

Neutrophil-to-lymphocyte ratio used as prognostic factor marker for dilated cardiomyopathy in childhood and adolescence.

OBJECTIVE: The objective of this study is to evaluate the neutrophil-to-lymphocyte ratio (NLR) and platelet-lymphocyte ratio, from the hemograms obtained from children and adolescents with dilated cardiomyopathy (DCM), and to correlate them with the levels of B-type natriuretic peptide (BNP) and with the clinical evolution of these patients in the long term. MATERIALS AND METHODS: Follow-up of 57 patients with DCM was made retrospectively, with hemogram and BNP level determination being performed after optimized therapy for heart failure. We compared the findings of the patients' examinations that progressed with stability in relation to the occurrence of transplant listing, cardiac transplantation, or evolution to death. RESULTS: The average age was 48 months, and the follow-up was 64 months. The average of the levels of neutrophils was greater in poor evolution group (7026 vs. 3903; P = 0.011) as well as the average of NLR (5.5 vs. 1.9; P = 0.034). The averages of hemoglobin, total leukocytes, lymphocytes, and platelets were similar in the groups. The area under the receiver operating characteristic curve for NLR in relation to the poor evolution was of 72.9%, being the best cutoff point of NLR ≥5.2 (sensitivity: 93.8% and specificity: 87.8%). Kaplan-Meier curves demonstrate that patients with NLR ≤5.2 (P = 0.001) and BNP <1000 pg/dl (P < 0.0001) presented greater survival. CONCLUSIONS: NLR (≥5.2) and lymphopenia (≤1000 lymphocyte/µL) were associated with a poor prognosis and a higher chance of evolution to death or cardiac transplant, similar to the findings for BNP.

Prognóstico da Cardiomiopatia dilatada com insuficiência cardíaca grave de acordo com escalas de classificação funcional na infância / Prognosis of dilated cardiomyopathy with severe heart failure according to functional classification scales in childhood

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

DOI: 10.5935/2359-4802.20170085 12 International Journal of Cardiovascular Sciences. 2018;31(1)12-21 ARTIGO ORIGINAL Correspondência: Fátima Derlene da Rocha Araújo Rua Indiana, 789, Ap 301. CEP: 30460350, Jardim América, Belo Horizonte, MG ­ Brasil. E-mail: fatima.derlene@hotmail.com, cleverpsi@hotmail.com Prognóstico da Cardiomiopatia Dilatada com Insuficiência Cardíaca Grave de Acordo com Escalas de Classificação Funcional na Infância Prognosis of Dilated Cardiomyopathy with Severe Heart Failure according to Functional Classification Scales in Childhood Fátima Derlene da Rocha Araújo, Rose Mary Ferreira Lisboa da Silva, Henrique de Assis Fonseca Tonelli, Adriana Furletti M Guimarães, Sandra Regina Tolentino Castilho, Zilda Maria Alves Meira Hospital das Clínicas da Universidade Federal de Minas Gerais, Belo Horizonte, MG ­ Brasil Artigo recebido em 14/02/2017; revisado em 17/04/2017; aceito em 05/06/2017 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. (Int J Cardiovasc Sci. 2018;31(1)12-21) Palavras-chave: Cardiomiopatia Dilatada, Prognóstico, Insuficiência Cardíaca, Escalas, Criança, Adolescentes. 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

Evolução Doppler Ecocardiográfica das Lesões Valvares Mitral e Aórtica em Crianças e Adolescentes com Cardite Reumática Subclínica e Leve / Doppler Echocardiographic Follow-Up of Mitral and Aortic Regurgitation in Children and Adolescents with Subclinical and Mild Rheumatic Carditis

Fundamento: As cardites reumáticas leve e subclínica se diferenciam basicamente pela ausculta de sopro regurgitativo mitral. A evolução destas formas não está bem estabelecida na literatura. Objetivo: Avaliar a evolução das cardites reumáticas leve e subclínica, considerando as valvites mitral e/ou aórtica (fase aguda) e a regressão, manutenção ou piora delas ao final do seguimento (fase crônica). Métodos: Estudo retrospectivo, longitudinal, incluindo pacientes com cardites reumáticas leve e subclínica. A evolução ecocardiográfica das valvites mitral e/ou aórtica foi comparada nos dois grupos, considerando a análise ao final do seguimento. Foram utilizados o teste qui quadrado e as curvas de sobrevida de Kaplan-Meier, com nível de significância p < 0,05. Resultados: Foram incluídos 125 pacientes, sendo 69 (55,2%) com cardite reumática subclínica e 56 (44,8%) com cardite reumática leve, com média de idade na fase aguda de 10,4 ± 2,6 anos e, ao final do estudo, de 19,9 ± 4,6 anos. O tempo de seguimento variou de 2 a 23 anos (média: 9,38 ± 4,3 anos). Na fase aguda, a regurgitação mitral leve/ moderada ou moderada foi mais frequente nos pacientes com cardite reumática leve (p = 0,001). A regurgitação aórtica leve ou leve/moderada também foi mais comum no grupo de cardite reumática leve (p = 0,045). Na fase crônica, observou-se que tanto a regurgitação mitral (p < 0,0001) quanto a regurgitação aórtica (p = 0,009) foram mais frequentes nos pacientes com cardite reumática leve, e a sobrevida livre de valvopatia residual foi maior no grupo de cardite reumática subclínica (p = 0,010). A regurgitação mitral residual foi maior no grupo de cardite reumática leve p < 0,0001), e a regurgitação aórtica residual foi semelhante nos dois grupos (p = 0,099). Conclusão: A resolução da regurgitação mitral foi maior nos pacientes com cardite reumática subclínica, e a involução da regurgitação aórtica foi menos frequente e semelhante nos dois grupos

Background: Mild rheumatic carditis (MRC) and subclinical rheumatic carditis (SRC) are basically differentiated through auscultation of mitral regurgitation murmur. The evolution of these forms is not well established in the literature. Objective: To evaluate the evolution of mild and subclinical rheumatic carditis, considering mitral and aortic regurgitation (acute phase) and regression, maintenance or worsening of these diseases at the end of follow-up (chronic phase). Methods: Retrospective, longitudinal study, including patients with mild and subclinical rheumatic carditis. The echocardiographic evolution of mitral and aortic regurgitation was compared in both groups, considering the analysis at the end of follow-up. The Chi-square test and Kaplan-Meier survival curves were used, with significance level established at p < 0.05. Results: A total of 125 patients were included, 69 (55.2%) with subclinical rheumatic carditis and 56 (44.8%) with mild rheumatic carditis, with a mean age in the acute phase of 10.4 ± 2.6 years and, at the end of study, 19.9 ± 4.6 years. The time of follow-up ranged from 2 to 23 years (mean: 9.38 ± 4.3 years). In the acute phase, mild/moderate or moderate mitral regurgitation was more frequent in patients with mild rheumatic carditis (p = 0.001). Mild or mild/moderate aortic regurgitation was also more common in the mild rheumatic carditis group (p = 0.045). In the chronic phase, we observed that both mitral (p < 0.0001) and aortic regurgitation (p = 0.009) were more frequent in patients with mild rheumatic carditis, and survival free of rheumatic heart disease was higher in the subclinical rheumatic carditis group (p = 0.010). Residual mitral regurgitation was higher in the mild rheumatic carditis group p < 0.0001), and residual aortic regurgitation was similar in both groups (p = 0.099). Conclusion: Mitral regurgitation resolution was higher in patients with subclinical rheumatic carditis, and the involution of aortic regurgitation was less frequent and similar in both groups

Pulmonary hypertension evaluation by Doppler echocardiogram in children and adolescents with mouth breathing syndrome / Avaliação de hipertensão pulmonar pela ecodopplercardiografia em crianças e adolescentes com síndrome do respirador oral

Abstract Introduction: Adenotonsillar hyperplasia (ATH) and allergic rhinitis (AR) are the most common causes of upper airway obstruction in children. Such diseases, by affecting the upper airways, can cause chronic alveolar hypoventilation, pulmonary vasoconstriction and pulmonary hypertension, which in some cases, are irreversible. Objective: This cross-sectional study aimed to evaluate the prevalence of pulmonary hypertension in two groups of mouth-breathing (MB) 2-12 years old children with ATH and isolated allergic rhinitis, through Doppler echocardiography. Methods: 54 patients with ATH and indications for adenoidectomy and/or tonsillectomy and 24 patients with persistent allergic rhinitis were selected and submitted to Doppler echocardiography. The Systolic Pulmonary Artery Pressure (SPAP) was determined by tricuspid regurgitation and the Mean Pulmonary Artery Pressure (MPAP) was calculated from the SPAP. Similar measurements were carried out in 25 nasal breathing (NB) individuals. Results: The mean MPAP and SPAP were higher in the MB than in the NB group (17.62 ± 2.06 [ATH] and 17.45 ± 1.25 [AR] vs. 15.20 ± 2.36 [NB] mmHg, p < 0.005, and 25.61 ± 3.38 [ATH] and 25.33 ± 2.06 [AR] vs. 21.64 ± 3.87 [NB] mmHg, p < 0.005, respectively) and the mean acceleration time of pulmonary flow trace (Act) was higher in the NB than in the MB group (127.24 ± 12.81 [RN] vs. 114.06 ± 10.63 ms [ATH] and 117.96 ± 10.28 [AR] MS [AR]; p < 0.0001). Conclusion: None of the MB children (ATH and AR) met the PH criteria, although individuals with both ATH and isolated AR showed significant evidence of increased pulmonary artery pressure by Doppler echocardiography in relation to NB individuals. No differences were observed between the ATH and AR groups.

Resumo Introdução: A hiperplasia adenotonsilar (HAT) e a rinite alérgica (RA) consistem nas causas mais comuns de obstrução de vias aéreas superiores em crianças. Tais afecções, ao comprometer a via aérea superior, podem ocasionar hipoventilação alveolar crônica, vasoconstrição pulmonar e hipertensão pulmonar, em alguns casos irreversível. Objetivo: Este estudo transversal objetivou avaliar a prevalência de hipertensão arterial pulmonar em dois grupos de crianças respiradoras orais (RO): com HAT e rinite alérgica isolada, de 2 a 12 anos, por meio de exame ecodopplercardiográfico. Método: Foram selecionados e submetidos à ecodopplercardiografia 54 pacientes com HAT com indicação de adenoidectomia e/ou tonsilectomia e 24 pacientes com rinite alérgica persistente. A pressão sistólica da artéria pulmonar (PSAP) foi determinada pela regurgitação tricúspide e a pressão média da artéria pulmonar (PMAP) foi calculada a partir da PSAP. Determinações similares foram feitas em 25 respiradores nasais (RN). Resultados: As médias da PMAP e da PSAP foram maiores nos grupos de RO do que nos RN (17,62 ± 2,06 [HAT] e 17,45 ± 1,25 [RA] vs. 15,20 ± 2,36 [RN] mmHg; p < 0,005; e 25,61 ± 3,38 [HAT] e 25,33 ± 2,06 [RA] vs. 21,64 ± 3,87 [RN] mmHg; p < 0,005; respectivamente) e a média do tempo de aceleração do traçado do fluxo pulmonar (TAc) foi maior nos RN que nos grupos de RO (127,24 ± 12,81 [RN] vs. 114,06 ± 10,63 ms [HAT] e 117,96 ± 10,28 [RA] MS [RA]; p < 0,0001). Conclusão: Nenhuma criança respiradora oral (HAT e RA) preencheu os critérios de HP, embora tanto os portadores de HAT quanto de RA isolada apresentaram evidências significativas de aumento da pressão arterial pulmonar pela ecodopplercardiografia em relação aos respiradores nasais. Não se observou diferença entre os grupos HAT e RA.

Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy: A Randomized Clinical Trial.

Importance: In Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), interventions reducing the progression of myocardial disease could affect survival. Objective: To assess the effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocardial fibrosis (MF) identified on cardiovascular magnetic resonance (CMR). Design, Setting, and Participants: A randomized clinical trial conducted in 2 centers included 76 male patients with DMD or BMD undergoing 2 CMR studies with a 2-year interval for ventricular function and MF assessment. In a non-intent-to-treat trial, 42 patients with MF and normal left ventricular ejection fraction (LVEF) were randomized (1:1) to receive or not receive ACE inhibitor therapy. The study was conducted from June 26, 2009, to June 30, 2012. Data analysis was performed from June 30, 2013, to October 3, 2016. Interventions: Randomization (1:1) to receive or not receive ACE inhibitor therapy. Main Outcomes and Measures: Primary outcome was MF progression from baseline to the 2-year CMR study. Results: Of the 76 male patients included in the study, 70 had DMD (92%) and 6 had BMD (8%); mean (SD) age at baseline was 13.1 (4.4) years. Myocardial fibrosis was present in 55 patients (72%) and LV systolic dysfunction was identified in 13 patients (24%). Myocardial fibrosis at baseline was an independent indicator of lower LVEF at follow-up (coefficient [SE], -0.16 [0.07]; P = .03). Among patients with MF and preserved LVEF (42 [55%]), those randomized (21 patients in each arm) to receive ACE inhibitors demonstrated slower MF progression compared with the untreated group (mean [SD] increase of 3.1% [7.4%] vs 10.0% [6.2%] as a percentage of LV mass; P = .001). In multivariate analysis, ACE inhibitor therapy was an independent indicator of decreased MF progression (coefficient [SE], -4.51 [2.11]; P = .04). Patients with MF noted on CMR had a higher probability of cardiovascular events (event rate, 10 of 55 [18.2%] vs 0 of 21 [0%]; log-rank P = .04). Conclusions and Relevance: In this 2-year, follow-up, randomized clinical trial of patients with Duchenne or Becker muscular dystrophy whose LVEF was preserved and MF was present as determined on CMR, ACE inhibitor therapy was associated with significantly slower progression of MF. The presence of MF was associated with worse patient prognosis. Trial Registration: clinicaltrials.org Identifier: NCT02432885.

Pulmonary hypertension evaluation by Doppler echocardiogram in children and adolescents with mouth breathing syndrome.

INTRODUCTION: Adenotonsillar hyperplasia (ATH) and allergic rhinitis (AR) are the most common causes of upper airway obstruction in children. Such diseases, by affecting the upper airways, can cause chronic alveolar hypoventilation, pulmonary vasoconstriction and pulmonary hypertension, which in some cases, are irreversible. OBJECTIVE: This cross-sectional study aimed to evaluate the prevalence of pulmonary hypertension in two groups of mouth-breathing (MB) 2-12 years old children with ATH and isolated allergic rhinitis, through Doppler echocardiography. METHODS: 54 patients with ATH and indications for adenoidectomy and/or tonsillectomy and 24 patients with persistent allergic rhinitis were selected and submitted to Doppler echocardiography. The Systolic Pulmonary Artery Pressure (SPAP) was determined by tricuspid regurgitation and the Mean Pulmonary Artery Pressure (MPAP) was calculated from the SPAP. Similar measurements were carried out in 25 nasal breathing (NB) individuals. RESULTS: The mean MPAP and SPAP were higher in the MB than in the NB group (17.62±2.06 [ATH] and 17.45±1.25 [AR] vs. 15.20±2.36 [NB] mmHg, p<0.005, and 25.61±3.38 [ATH] and 25.33±2.06 [AR] vs. 21.64±3.87 [NB] mmHg, p<0.005, respectively) and the mean acceleration time of pulmonary flow trace (Act) was higher in the NB than in the MB group (127.24±12.81 [RN] vs. 114.06±10.63ms [ATH] and 117.96±10.28 [AR] MS [AR]; p<0.0001). CONCLUSION: None of the MB children (ATH and AR) met the PH criteria, although individuals with both ATH and isolated AR showed significant evidence of increased pulmonary artery pressure by Doppler echocardiography in relation to NB individuals. No differences were observed between the ATH and AR groups.

Papel da ecocardiografia na avaliação das alterações cardiovasculares em recém-nascidos prematuros de muito baixo peso, com ênfase na presença do canal arterial / Echocardiography role in assessing cardiovascular changes in very low birth weight babies, with emphasis on the presence of the ductus arteriosus

O trabalho consistiu em uma revisão bibliográfica sobre as alterações cardiovasculares em neonatos prematuros. Tais alterações têm elevada prevalência nas unidades de terapiaintensiva neonatal, bem como há necessidade frequente de tratamento específico e implicações prognósticas. A revisão foi realizada de forma não sistematizada, a partir das seguintesfontes de pesquisas: PubMed, BVS e Medline. Foram definidas as causas de tais alterações hemodinâmicas,os métodos frequentemente utilizados para sua detecção e propostas alternativas mais objetivas e eficientes nesta avaliação, enfatizando-se diferentes parâmetros ecocardiográficos bem como as limitações de cada método.

Language Impairment in Adolescents With Sydenham Chorea.

BACKGROUND: Neuropsychiatric comorbidities are frequent in Sydenham chorea. However, cognitive impairment in Sydenham chorea has not been sufficiently described. The objective of this study was to evaluate expressive and receptive language deficits in adolescents with Sydenham chorea. METHODS: Twenty patients with Sydenham chorea were compared with 20 patients with rheumatic fever without chorea and 20 healthy controls. Participants were matched for age and gender. Participants were assessed with verbal fluency tasks (phonemic and semantic) and with verbal comprehension tasks (Token Test). Patients with Sydenham chorea were also assessed with the Universidade Federal de Minas Gerais Sydenham Chorea Rating Scale. RESULTS: Performance in verbal fluency and in verbal comprehension tasks differed significantly (P < 0.01) among the three groups. Patients with Sydenham chorea performed significantly worse than healthy control group in phonemic and semantic verbal fluency tasks as well as in the Token Test. The group with rheumatic fever also performed worse than healthy controls in phonemic verbal fluency. Severity of motor signs in Sydenham chorea inversely correlated with performance in phonemic verbal fluency (words beginning with letter S, and total sum of words beginning with letters F, A, and S). CONCLUSIONS: Adolescents with Sydenham chorea show difficulties in verbal fluency and in verbal comprehension. Patients with rheumatic fever also have some degree of language impairment. Future studies must investigate language impairment in difference stages of Sydenham chorea (acute, persistent, and remission) and putative biological markers.

Prevalence of rheumatic heart disease in a public school of Belo Horizonte.

BACKGROUND: Previous studies indicate that compared with physical examination, Doppler echocardiography identifies a larger number of cases of rheumatic heart disease in apparently healthy individuals. OBJECTIVES: To determine the prevalence of rheumatic heart disease among students in a public school of Belo Horizonte by clinical evaluation and Doppler echocardiography. METHODS: This was a cross-sectional study conducted with 267 randomly selected school students aged between 6 and 16 years. students underwent anamnesis and physical examination with the purpose of establishing criteria for the diagnosis of rheumatic fever. They were all subjected to Doppler echocardiography using a portable machine. Those who exhibited nonphysiological mitral regurgitation (MR) and/or aortic regurgitation (AR) were referred to the Doppler echocardiography laboratory of the Hospital das Clínicas of the Universidade Federal of Minas Gerais (HC-UFMG) to undergo a second Doppler echocardiography examination. According to the findings, the cases of rheumatic heart disease were classified as definitive, probable, or possible. RESULTS: Of the 267 students, 1 (0.37%) had a clinical history compatible with the diagnosis of acute rheumatic fever (ARF) and portable Doppler echocardiography indicated nonphysiological MR and/or AR in 25 (9.4%). Of these, 16 (6%) underwent Doppler echocardiography at HC-UFMG. The results showed definitive rheumatic heart disease in 1 student, probable rheumatic heart disease in 3 students, and possible rheumatic heart disease in 1 student. CONCLUSION: In the population under study, the prevalence of cases compatible with rheumatic involvement was 5 times higher on Doppler echocardiography (18.7/1000; 95% CI 6.9/1000-41.0/1000) than on clinical evaluation (3.7/1000-95% CI).

Prevalence of Rheumatic Heart Disease in a Public School of Belo Horizonte / Prevalência de Valvopatia Reumática Subclínica em Alunos de Escola Pública de Belo Horizonte

Background: Previous studies indicate that compared with physical examination, Doppler echocardiography identifies a larger number of cases of rheumatic heart disease in apparently healthy individuals. Objectives: To determine the prevalence of rheumatic heart disease among students in a public school of Belo Horizonte by clinical evaluation and Doppler echocardiography. Methods: This was a cross-sectional study conducted with 267 randomly selected school students aged between 6 and 16 years. students underwent anamnesis and physical examination with the purpose of establishing criteria for the diagnosis of rheumatic fever. They were all subjected to Doppler echocardiography using a portable machine. Those who exhibited nonphysiological mitral regurgitation (MR) and/or aortic regurgitation (AR) were referred to the Doppler echocardiography laboratory of the Hospital das Clínicas of the Universidade Federal of Minas Gerais (HC-UFMG) to undergo a second Doppler echocardiography examination. According to the findings, the cases of rheumatic heart disease were classified as definitive, probable, or possible. Results: Of the 267 students, 1 (0.37%) had a clinical history compatible with the diagnosis of acute rheumatic fever (ARF) and portable Doppler echocardiography indicated nonphysiological MR and/or AR in 25 (9.4%). Of these, 16 (6%) underwent Doppler echocardiography at HC-UFMG. The results showed definitive rheumatic heart disease in 1 student, probable rheumatic heart disease in 3 students, and possible rheumatic heart disease in 1 student. Conclusion: In the population under study, the prevalence of cases compatible with rheumatic involvement was 5 times higher on Doppler echocardiography (18.7/1000; 95% CI 6.9/1000-41.0/1000) than on clinical evaluation (3.7/1000-95% CI). .

Fundamento: Estudos indicam que o Doppler ecocardiograma possibilita a identificação de um maior número de casos de valvopatia reumática, quando comparado ao exame clínico, em indivíduos aparentemente saudáveis. Objetivos: Determinar a prevalência de valvopatia sugestiva de envolvimento reumático segundo as avaliações clínicas e Doppler ecardiográficas em alunos de escola pública de Belo Horizonte. Métodos: Estudo transversal realizado com 267 escolares entre 6 e 16 anos, selecionados de forma aleatória. Os alunos foram submetidos à anamnese e exame físico com o objetivo de estabelecer critérios prévios para o diagnóstico de febre reumática. Todos realizaram o estudo Doppler ecocardiográfico com o emprego de um aparelho portátil. Aqueles que apresentaram regurgitação valvar mitral (RM) e ou aórtica (RAo) sugestiva de não fisiológica foram encaminhados ao laboratório de Doppler ecocardiografia do Hospital das Clínicas da Universidade Federal de Minas Gerais (HC-UFMG) para a realização de novo estudo. Conforme os achados, os casos de valvopatia reumática foram classificados em definitiva, provável e possível. Resultados: Dos 267 escolares, um (0,37%) apresentou história compatível com o diagnóstico de febre reumática aguda (FRA), 25 (9,4%) apresentaram RM e/ou RAo consideradas não fisiológicas ao Doppler ecocardiograma portátil. Destes, 16 (6%) realizaram Doppler ecocardiograma no HC-UFMG, sendo evidenciadas: valvopatia reumática definitiva em um escolar; valvopatia reumática provável em três; valvopatia reumática possível em um escolar. Conclusão: Na população estudada a prevalência de casos compatíveis com envolvimento reumático foi cinco vezes maior ...