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UNLABELLED: Transposition of the great arteries (TGA) is a congenital heart defect successfully corrected through arterial switch operation (ASO). Although this technique had significant impact in improving survival, little is known about the functional capacity of the operated patients long-term after surgery. OBJECTIVE: The aim of this study was to compare the functional capacity of children with TGA long-term after ASO with that of healthy children. DESIGN: Retrospective study. PATIENTS: All patients that performed cardiopulmonary exercise test (CPET) were included in the study. As a control group, healthy children in evaluation for physical activity that performed CPET during the same period were also enrolled. RESULTS: Thirty-one TGA patients (19 male) were compared with 29 age-matched controls (21 male). Maximum oxygen consumption was higher in the control group (45.47 ± 8.05 vs. 40.52 ± 7.19, P = .017), although within normal limits in both groups (above 90% of predicted value). The heart rate behavior during exercise was different in both groups, with a mean chronotropic index significantly lower in the TGA group (63% ± 14 vs. 81% ± 12, P < .001). CONCLUSIONS: Our results showed that exercise capacity long-term after ASO in TGA is well preserved although lower than in healthy children what might be explained by the presence of chronotropic incompetence in the TGA group.

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Dyslipidemia is one of the main risk factors associated with cardiovascular diseases. Few data on the impacts of congenital heart diseases are available with regard to the prevalence of dyslipidemia in children. Our study evaluated the lipid profile in children with congenital heart disease at a referral center. From January 2011 to July 2012, 52 pediatric patients had their lipid, metabolic and clinical profiles traced. The mean age was 10.4 ± 2.8 years and male/female rate of 1.38:1. Our population had 53.8% patients with high levels of total cholesterol and 13.4% (CI 95 %, from 6.6 to 25.2%) of them also presenting LDL levels ≥ 130 mg/dL, which characterizes dyslipidemia. The group of dyslipidemic patients presented only two obese individuals. Our data show that the presence of congenital heart disease does not lead to higher risk associated with the prevalence of dyslipidemia. Therefore, the screening of this specific population should follow the regular pediatric guidelines, which are also independent of the nutritional status of the children tested.

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INTRODUÇÃO: A experiência com o fechamento percutâneo da comunicação interatrial (CIA) em crianças pequenas é limitada. Avaliamos a factibilidade, a segurança e a eficácia desse procedimento em crianças com peso < 20 kg. MÉTODOS: Estudo descritivo observacional de uma coorte de crianças < 20 kg submetidas a tratamento percutâneo. Pacientes com dilatação ventricular direita e sintomas evidentes foram incluídos. Implantamos próteses aprovadas pela ANVISA, sob monitorização ecocardiográfica transesofágica. Os pacientes foram avaliados 1 mês, 3 meses, 6 meses e 12 meses após. RESULTADOS: Entre outubro de 1997 e maio de 2012, 80 pacientes foram tratados. As medianas de idade e peso foram de 4 anos (1-12) e 13,5 kg (5-20), respectivamente, 20 pacientes apresentavam alguma síndrome genética (25%) e 4 pacientes (5%) apresentavam CIA adicional. Somente um paciente necessitou duas próteses. Dois pacientes tinham defeitos associados, os quais foram tratados no mesmo procedimento (estenose pulmonar valvar e fístula arteriovenosa). Um paciente desenvolveu bloqueio atrioventricular total durante o implante da prótese, resolvido espontaneamente 36 horas após a remoção da prótese, sem necessidade de implante de marca-passo. Esse paciente foi tratado percutaneamente 6 meses após com sucesso, sem complicações. Setenta e nove pacientes receberam alta hospitalar em até 24 horas após o procedimento. Fluxo residual discreto (1-2 mm) foi observado em 5% dos casos antes da alta. Após 6 meses de seguimento, não foi detectado fluxo residual. Não houve complicações tardias no seguimento. CONCLUSÕES: O fechamento percutâneo da CIA em crianças pequenas selecionadas e sintomáticas é uma alternativa tera­pêutica factível, segura e eficaz, devendo ser a primeira opção para seu tratamento.

BACKGROUND: The experience with percutaneous closure of atrial septal defect (ASD) in infants is limited. We sought to determine the feasibility, safety and efficacy of this procedure in children weighing < 20 kg. METHODS: Observational study of a cohort of children weighing < 20 kg undergoing percutaneous closure. Patients with right ventricular enlargement and evident symptoms were included. ANVISA approved devices were implanted under transesophageal echocardiography monitoring. Patients were evaluated 1, 3, 6 and 12 months after the procedure. RESULTS: Eighty patients were treated between October 1997 and May 2012. Median age and weight were 4 years (1-12) and 13.5 kg (5-20), respectively, 20 patients had a genetic syndrome (25%) and 4 patients (5%) had an additional ASD. Only one patient required 2 devices. Two patients had associated defects that were treated in the same procedure (pulmonary valve stenosis and arteriovenous fistula). One patient developed total atrioventricular block during device implantation, solved spontaneously 36 hours after device removal, with no need for pacemaker implantation. This patient was successfully treated percutaneously 6 months later without complications. Seventy-nine patients were discharged within 24 hours after the procedure. A mild residual shunt (1-2 mm) was observed in 5% of the cases before discharge. There was no residual shunt 6 months after the procedure. There were no complications in the late follow-up. CONCLUSIONS: Percutaneous ASD closure in selected symptomatic infants is a feasible, safe and effective alternative and should be the first option therapy.

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Report of three cases of chylothorax and one case of chylous ascites in children who had undergone cardiac surgery with no response to routine clinical treatment, based on fasting and long-term parenteral nutrition. Treatment with octreotide at an initial dose of 1.0 mcg/kg/h was chosen, with a gradual increase of 1.0 mcg/kg/hr/day until a maximum dose of 4.0 mcg/kg/h. All cases had a favorable response, with gradual reduction of drainage output until prognosis improvement with no significant side effects.

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Relato de três casos de quilotórax e um caso de ascite quilosa em crianças em pós-operatório de cirurgia cardíaca, que evoluíram sem resposta ao tratamento clínico habitual, baseado em jejum e nutrição parenteral prolongada. Tratamento com octreotide na dose inicial de 1,0 mcg/kg/h foi escolhido, com aumento progressivo de 1,0 mcg/kg/h/dia até a dose máxima de 4,0 mcg/kg/h. Todos os casos tiveram resposta favorável, com redução progressiva do débito do dreno, até resolução do quadro, sem efeito colateral significativo.

Report of three cases of chylothorax and one case of chylous ascites in children who had undergone cardiac surgery with no response to routine clinical treatment, based on fasting and long-term parenteral nutrition. Treatment with octreotide at an initial dose of 1.0 mcg/kg/h was chosen, with a gradual increase of 1.0 mcg/kg/hr/day until a maximum dose of 4.0 mcg/kg/h. All cases had a favorable response, with gradual reduction of drainage output until prognosis improvement with no significant side effects.

Relato de tres casos de quilotórax y un caso de ascitis quilosa en niños en postoperatorio de cirugía cardíaca, que evolucionaron sin respuesta al tratamiento clínico habitual, basado en ayuno y nutrición parenteral prolongada. Fue elegido tratamiento con octreotide en dosis inicial de 1,0 mcg/kg/h , con aumento progresivo de 1,0 mcg/kg/h/día hasta la dosis máxima de 4,0 mcg/kg/h. Todos los casos tuvieron respuesta favorable, con reducción progresiva del débito del drenaje, hasta resolución del cuadro, sin efecto colateral significativo.

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OBJECTIVE: To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis. METHODS: Seventy-five patients (median age: 8 years) underwent the procedure through the retrograde femoral or carotid route. RESULTS: The procedure was completed in 74 patients (98.6%). The peak-to-peak systolic gradient dropped from 79.6 27.7 to 22.3 17.8 mmHg (P<0.001), the left ventricular systolic pressure dropped from 164 39.1 to 110 24.8 mmHg (P<0.001), and the left ventricular end diastolic pressure dropped from 13.3 5.5 to 8.5 8.3 mmHg (P< 0.01). Four patients (5.3%) died due to the procedure. Aortic regurgitation (AoR) appeared or worsened in 27/71 (38%) patients, and no immediate surgical intervention was required. A mean follow-up of 50 38 months was obtained in 37 patients. Restenosis and significant AoR were observed in 16.6% of the patients. The estimates for being restenosis-free and for having significant AoR in 90 months were 60% and 50%, respectively. CONCLUSION: Aortic valvuloplasty was considered the initial palliative method of choice in managing congenital aortic stenosis, with satisfactory short- and midterm results.

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OBJECTIVE: To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis. METHODS: Seventy-five patients (median age: 8 years) underwent the procedure through the retrograde femoral or carotid route. RESULTS: The procedure was completed in 74 patients (98.6 percent). The peak-to-peak systolic gradient dropped from 79.6±27.7 to 22.3±17.8 mmHg (P<0.001), the left ventricular systolic pressure dropped from 164±39.1 to 110±24.8 mmHg (P<0.001), and the left ventricular end diastolic pressure dropped from 13.3±5.5 to 8.5±8.3 mmHg (P< 0.01). Four patients (5.3 percent) died due to the procedure. Aortic regurgitation (AoR) appeared or worsened in 27/71 (38 percent) patients, and no immediate surgical intervention was required. A mean follow-up of 50±38 months was obtained in 37 patients. Restenosis and significant AoR were observed in 16.6 percent of the patients. The estimates for being restenosis-free and for having significant AoR in 90 months were 60 percent and 50 percent, respectively. CONCLUSION: Aortic valvuloplasty was considered the initial palliative method of choice in managing congenital aortic stenosis, with satisfactory short- and midterm results

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O eletrocardiograma associado ao exame físico e ao estudo radiológico constituem a base para o diagnóstico clínico em cardiologia pediátrica. O primeiro eletrocardiograma em humano foi realizado em 1887. Entretanto, somente em 1901 Willem Einthoven descreveu um galvanômetro para gravar as alterações da atividade elétrica na superfície corpórea. O traçado eletrocardiográfico deve ser consi- derado em relação a idade, deformidade torácica, más posições cardíacas, etc. Por outro lado, ao se interpretar um eletrocardiograma deve-se obedecer a uma sistemática. Existem tabelas que co-relacionam a idade e a freqüência cardíaca com as medidas obtidas no eletrocardiograma, sendo a mais elucidativa a idealizada por Davignon. Em relação ao eletrocardiograma anormal, considera-se que o eixo e a morfologia da onda P e do complexo QRS são parâmetros fundamentais na orientação das câmaras cardíacas. A análise da onda P permite estabelecer o diagnóstico da sobrecarga atrial direita ou esquerda. O diagnóstico de hipertrofia ventricular direita ou esquerda obedece a alguns critérios que valori- zam o complexo QRS. O que se considera normal para um determinado período de vida pode ser anormal em outra idade. Finalmente, o eletrocardiograma é um exame de fácil realização, baixo custo e sensível em muitas cardiopatias congênitas. É de grande ajuda quando bem analisado e em associação com a história clínica e o exame físico permite o diagnóstico adequado...

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