Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

Abstract Double-lumen aortic arch is a rare congenital anomaly related to persistence of the fifth aortic arch. It may be found alone or in association with other anatomical changes of the heart. We report a case of double-lumen aortic arch associated with coarctation of the aorta and patent ductus arteriosus in a child with a congenital malformation known as the VACTERL association (vertebral defects, imperforate anus, cardiopathy, tracheoesophageal fistula, renal abnormalities and limb anomalies).

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.

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

Indicators of surgical treatment of patent ductus arteriosus in preterm neonates in the first week of life / Indicadores para o tratamento cirúrgico na persistência do ducto arterial em neonatos prematuros na primeira semana de vida

OBJECTIVE: To identify clinical and echocardiographic indicators of the necessity for early surgical closure of patent ductus arteriosus in preterm neonates. METHODS: The prospective study was conducted at the Neonatal Unit of Hospital Municipal Odilon Behrens between 2006 and 2010. The study population comprised 115 preterm neonates diagnosed with patent ductus arteriosus in the first week after birth, of whom 55 (group S) were submitted to clinical and or surgical closure and 60 (group NS) received non-surgical treatment. The parameters analyzed were birth weight, diameter of the ductus arteriosus (DAD), left atrial-to-aortic root diameter ratio (LA:Ao), the quotient of DAD² and birth weight (mm²/kg), and ductal shunting. RESULTS: The study population comprised 58 males and 57 females. The average birth weight of group S (924 ± 224.3 g) was significantly (P=0.049) lower than that of group NS (1012.3 ± 242.8 g). The probability of the preterm neonates being submitted to surgical closure was 62.1% (P=0.006) when the DAD2/birth weight index was > 5 mm²/kg, 72.2% (P=0.001) when the LA:Ao ratio was > 1.5, and 61.2% when ductal shunting was high (P=0.025). CONCLUSION: The parameters DAD²/birth weight index > 5 mm²/kg, LA:Ao ratio > 1.5 and high ductal shunting were statistically significant indicators (P<0.05) of the need for surgical closure of patent ductus arteriosus in low birth weight preterm neonates. Moreover, when an LA:Ao ratio > 1.5 was associated with the occurrence of shock, the probability of surgical closure increased to 78.4%.

OBJETIVO: Identificar parâmetros clínicos e ecocardiográficos para a indicação do tratamento cirúrgico precoce da persistência do ducto arterial. MÉTODOS: Esse estudo prospectivo foi conduzido na Unidade Neonatal do Hospital Municipal Odilon Behrens entre 2006 e 2010. A população estudada compreendeu 115 neonatos prematuros diagnosticados com persistência do ducto arterial na primeira semana após o nascimento, dos quais 55 (grupo S) foram submetidos ao tratamento clínico e ou cirúrgico e 60 (grupo NS) ao tratamento clínico. Os parâmetros analisados foram peso ao nascer, diâmetro do ducto arterial (DAD), relação diâmetro do átrio esquerdo pelo diãmetro da aorta (AE/Ao), índice DAD2/peso ao nascer e fluxo no ducto. RESULTADOS: O estudo abrangeu 58 pacientes do sexo masculino e 57 do feminino. O peso médio ao nascer do grupo S (924 ± 224,3 g) foi significativamente (P=0,049) menor do que do grupo NS (1012,3 ± 242,8 g). A probabilidade dos neonatos prematuros serem submetidos à cirurgia foi 62.1% (P=0,006) quando o índice DAD2/peso ao nascer era > 5 mm2/kg, 72,2% (P=0,001) quando a razão LA:Ao era > 1,5 e 61,2% (P=0,025) quando o fluxo no ducto era alto. CONCLUSÃO: Os parâmetros DAD²/peso ao nascer > 5 mm²/kg, razão LA:Ao > 1,5 e alto fluxo no ducto foram preditores estatisticamente significativos (P<0,05) da necessidade de fechamento cirúrgico do persistência do ducto arterial em neonatos prematuros com baixo peso ao nascer. Adicionalmente, quando a razão LA:Ao > 1,5 estava associada ao choque, a probabilidade de tratamento cirúrgico aumentou para 78,4%.

Indicators of surgical treatment of patent ductus arteriosus in preterm neonates in the first week of life.

OBJECTIVE: To identify clinical and echocardiographic indicators of the necessity for early surgical closure of patent ductus arteriosus in preterm neonates. METHODS: The prospective study was conducted at the Neonatal Unit of Hospital Municipal Odilon Behrens between 2006 and 2010. The study population comprised 115 preterm neonates diagnosed with patent ductus arteriosus in the first week after birth, of whom 55 (group S) were submitted to clinical and or surgical closure and 60 (group NS) received non-surgical treatment. The parameters analyzed were birth weight, diameter of the ductus arteriosus (DAD), left atrial-to-aortic root diameter ratio (LA:Ao), the quotient of DAD² and birth weight (mm²/kg), and ductal shunting. RESULTS: The study population comprised 58 males and 57 females. The average birth weight of group S (924 ± 224.3 g) was significantly (P=0.049) lower than that of group NS (1012.3 ± 242.8 g). The probability of the preterm neonates being submitted to surgical closure was 62.1% (P=0.006) when the DAD2/birth weight index was > 5 mm²/kg, 72.2% (P=0.001) when the LA:Ao ratio was > 1.5, and 61.2% when ductal shunting was high (P=0.025). CONCLUSION: The parameters DAD²/birth weight index > 5 mm²/kg, LA:Ao ratio > 1.5 and high ductal shunting were statistically significant indicators (P<0.05) of the need for surgical closure of patent ductus arteriosus in low birth weight preterm neonates. Moreover, when an LA:Ao ratio > 1.5 was associated with the occurrence of shock, the probability of surgical closure increased to 78.4%.

Prognostic value of clinical and Doppler echocardiographic findings in children and adolescents with significant rheumatic valvular disease.

INTRODUCTION: The diagnosis of acute rheumatic fever (RF) is based on clinical findings. However, during the chronic phase of the disease, the clinical approach is not sufficient for the follow-up of the patients and the Doppler echocardiography is a tool for the diagnosis of cardiac involvement. Prognostic variables that influence long-term outcomes are not well known. METHODS: 462 patients with RF according to Jones criteria were studied, and followed-up from the initial attack to 13.6 ± 4.6 years. All patients underwent clinical assessment and Doppler echocardiography for the detection of heart valve involvement in the acute and chronic phases. Multivariate logistic regression analysis was used to identify the factors influencing long-term heart valve disease. RESULTS: Carditis occurred in 55.8% and subclinical valvulitis in 35.3% patients. In the chronic phase, 33% of the patients had significant valvular heart disease. No normal Doppler echocardiography exam was observed on patients who had severe valvulitis, although heart auscultation had become normal in 13% of these. In the multivariate analysis, only the severity of carditis and the mitral and/or aortic valvulitis were associated with significant valvular heart disease. Chorea or arthritis were protective factors for significant valvular heart disease, odds ratio 0.41 (95% C.I. 0.22 - 0.77) and 0.43 (95% C.I. 0.23 - 0.82), respectively. CONCLUSIONS: Our study suggests that the use of Doppler echocardiography during RF helps to identify prognostic factors regarding the development of significant valvular heart disease. Initial severe carditis is an important factor in the long-term prognosis of chronic RHD, whereas arthritis and chore during the initial episode of RF appears to be protective. Strict secondary prophylaxis should be mandatory in high risk patients.

[Takayasu's disease with severe heart and arterial involvement in a preschool-age child]. / Doença de Takayasu com grave envolvimento cardíaco e arterial em pré-escolar.

The authors describe a case of Takayasu's arteritis in a child of only 3 years of age, emphasizing the rarity of this disease in this age group. The child was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. After a detailed clinical examination, extensive diagnostic tests, and observation of the clinical evolution, the diagnosis was Takayasu's disease with severe cardiac and arterial involvement. The report warns pediatricians and cardiologists to awareness of the possibility of this disease in very small infants, in countries where it is underdiagnosed.

Doença de Takayasu com grave envolvimento cardíaco e arterial em pré-escolar / Takayasu's disease with severe heart and arterial involvement in a preschool-age child / Enfermedad de takayasu con grave compromiso cardíaco y arterial en pre-escolar

Neste artigo os autores descrevem um caso de arterite de Takayasu em uma criança de apenas 3 anos de idade, ressaltando-se a raridade dessa doença nessa faixa etária. A criança foi atendida em serviço de urgência em estado pós-comicial de convulsão tônico-clônica generalizada. Após exame clínico detalhado, vasta propedêutica e evolução do quadro clínico, fez-se o diagnóstico de doença de Takayasu com grave envolvimento cardíaco e arterial. O relato desse caso alerta pediatras e cardiologistas para o reconhecimento dessa entidade em crianças de baixa idade, nos países em que ela é pouco diagnosticada.

The authors describe a case of Takayasu's arteritis in a child of only 3 years of age, emphasizing the rarity of this disease in this age group. The child was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. After a detailed clinical examination, extensive diagnostic tests, and observation of the clinical evolution, the diagnosis was Takayasu's disease with severe cardiac and arterial involvement. The report warns pediatricians and cardiologists to awareness of the possibility of this disease in very small infants, in countries where it is underdiagnosed.

En este artículo los autores describen un caso de arteritis de Takayasu en una criatura de apenas 3 años de edad, destacándose la rareza de esa enfermedad en esa franja etárea. La criatura fue atendida en servicio de urgencia en estado post comicial de convulsión tónico-clónica generalizada. Después de examen clínico detallado, vasta propedéutica y evolución del cuadro clínico, se hizo el diagnóstico de enfermedad de Takayasu con grave compromiso cardíaco y arterial. El relato de este caso alerta pediatras y cardiólogos sobre el reconocimiento de esa entidad en criaturas de baja edad, en los países en que ésta es poco diagnosticada.

Valvite subclínica em pacientes com coréia de sydenham ou artrite como manifestações maiores da febre reumática / Subclinical valvitis in patients with sydenham chorea or arthritis as major manifestations of rheumatic fever

Objetivo: Avaliar a frequência de alterações doppler-ecocardiográficas em pacientes em fase aguda de febre reumática sem evidência clínica de envolvimento cardiovascular (valvite subclínica), tendo a coréia de Sydenham ou a artrite como manifestações maiores. Método: Realizou-se estudo retrospectivo, entre agosto de 2001 e agosto de 2002, por meio da análise dos prontuários de 44 pacientes em primeiro surto de febre reumática, que apresentaram coréia e/ou artrite como manifestação clínica maior, com exame cardiovascular normal. Para análise estatística foram excluídos desse total os seis pacientes que apresentaram artrite e coréia associadas. Constituíram-se dois grupos de estudo - grupo l: pacientes que apresentaram como manifestação maior apenas artrite (ou poliartrite); grupo ll: pacientes que apresentaram apenas coréia de Sydenham. Resultados: Dos 44 pacientes, 25 (56,8%) apresentaram artrite, 13(29,6%) coréia, e seis (13,6%) artrite e coréia. O estudo doppler-ecocardicoráfico demonstrou alterações valvares (espessamento valvar mitral e/ou aórtico, associado à regurgitação das referidas valvas) em 21 (47,7%) dos 44 pacientes (valvite sub-clínica). Alterações valvares compatíveis com valvite foram evidenciadas em seis (24%) dos 25 pacientes do grupo I, em 11)84,6%) dos 13 pacientes do grupo II, e em quatro (66,7%) dos seis pacientes que apresentaram artrite e coréia simultaneamente. Conclusão: O Dopplerecocardiograma proporcionou melhor avaliação dos pacientes em fase aguda da febre reumática quanto ao envolvimento cardíaco. Maior frequência de valvite foi encontrada entre os pacientes com coréia.

Aim: To evaluate the frequency of dopplerechocardiographic changes in patients with acute rheumatic fever without clinical evidence of cardiac involvement (subclinical valvitis). All patients presented, as major manifestation of acute rheumatic fever, Sydenham's chorea or arthritis. Methods: From August 2001 to August 2002, a retrospective analysis was made in 44 patients who had chorea or arthritis as major clinical manifestation in their first episode of rheumatic fever. All patients had normal cardiovascular examination. For statistical analysis six patients who presented both arthritis and chorea were excluded. Therefore, there were two groups: (1) patients who presented only arthritis as major manifestation; (2) patients who presented only Sydenham's chorea. Results: Among 44 patients, 25(56.8%) presented arthritis, 13 (29.6%) presented chorea and six (13.6%) presented both. Dopplerechocardiographic study demonsirated subclinical valvitis in 21 (47.7%) of 44 patients: six (24.0%) of patients in group I, 11(84.6%) of 13 patients in groupll, and four (66.7%) of six patients who presented arthritis and chorea. Conclusion: Dopplerechocardiogram provided best evaluation of cardiac involvement in patients with acure rheumatic fever. Valvitis was seem more frequently in patients with chorea.