Luiz Alberto Christiani

Marfan's syndrome: early and severe form in siblings

Marfans syndrome is an inherited disorder of the connective tissue. Cardiologic manifestations, especially aortic dilation, are important causes of morbidity and mortality in the clinical course of the disease in adults and teenagers. In children, the presence of aortic aneurysm and its dissection or rupture is rare, occurring in patients with genetic mutation of the fibrillin gene but not in those who have the familial form of the disease. We describe here 2 patients, from the same family (siblings), diagnosed with gigantic aortic aneurysm early in infancy, one of them successfully undergoing surgery.

Noninvasive cardiac assessment in children of women with systemic lupus erythematosus

OBJECTIVE Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricular block. These children were compared with 30 children of women who had no lupus or anti-Ro/SSA antibodies, and no risk factors for congenital heart disease either. Their age groups matched. The following examinations were performed: cardiological physical examination, electrocardiography, echocardiography, and signal-averaged electrocardiography. RESULTS The statistical analysis showed no significant difference in ventricular function or in the cardiac conduction system between the groups. CONCLUSION In regard to the conduction system and ventricular function in the absence of total atrioventricular block, no statistically significant difference was observed between the children of women with systemic lupus erythematosus and children of healthy women.

Avaliação de Potenciais Fragmentados em Presença de Bloqueio de Ramo Direito sem Alterações Estruturais Miocárdicas Ventriculares. Estudo pelo Eletrocardiograma de Alta Resolução no Domínio da Freqüência

PURPOSE: To evaluate if the presence of right bundle branch block (RBBB), without structural myocardial abnormalities (SMA) can generate fragmented potentials (FP) and spectral turbulence on signal-averaged electrocardiogram (SAECG). METHODS: Twelve children with atrial septal defect (ASD) and incomplete right bundle branch block (IRBBB without SMA (group I) were compared to 17 children with post-operative tetralogy of Fallot (TF) with CRBBB, all with SMA, 5 with ventricular premature beats and 2 with sustained ventricular tachycardia (group II). All had SAECG on time (TD) and frequency domain (FD) with 5 variables analysed. RESULTS: All patients of group I had normal variables, in contrast with group II which presented abnormal variables suggesting FP and ST. CONCLUSION: In ASD without SMA, the isolated IRBBB did not generate FP and ST.Objetivo - Em ausencia de alteracoes estruturais miocardicas (AEM), avaliar se o bloqueio de ramo direito (BRD) gera potenciais fragmentados (PF) e turbulencia espectral (TE) no eletrocardiograma de alta resolucao (ECGAR). Metodos - Doze criancas com comunicacao interatrial (CIA) e bloqueio incompleto do ramo direito (BIRD) sem AEM (Grupo I), foram comparadas com 17 criancas com tetralogia de Fallot (TF) operada, BCRD e AEM, 5 com extra sistoles ventriculares e 2 com taquicardia ventricular sustentada (Grupo II). Todas fizeram ECGAR nos dominios do tempo (DT) e da frequencia (DF), com cinco variaveis analisadas. Resultados - Os pacientes do grupo I tiveram as variaveis normais apesar do BIRD. No grupo II, 4 das cinco variaveis foram anormais, sugerindo a presenca de PF e TE atribuiveis a AEM inerentes a malformacao e ao ato cirurgico. Conclusao - Na CIA o BIRD nao complicado de AEM nao gera PF e TE, nao constituindo fator de risco para taquicardia ventricular sustentada. Palavras-chave: eletrocardiograma de alta resolucao, turbulencia espectral, bloqueio do ramo direito.