The isolated perimembranous ventricular septal defect is one of the most common congenital cardiac malformations. Although surgery has been performed and has a low associated risk, it still involves morbidity due to factors such as residual leaks, atrioventricular block, postpericardiotomy syndrome and arrhythmias. It has been speculated that percutaneous closure of these defects could minimize such complications. Recently, a device designed specifically for perimembranous ventricular septal defect closure, the Amplatzer membranous ventricular septal defect occluder, has been developed. Initial clinical experience with this device has been very encouraging with results showing a high rate of complete closure and a low incidence of complications at mid-term follow-up. In this article, the authors review their own and others' experience with this device.

Device release was achieved by manipulation on the tricuspid apparatus.

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Neonatal lupus is an autoimmune disease due to passive acquisition of maternal antibodies during pregnancy 1,2 . These antibodies remain in the fetal and newborn circulation causing skin, hematological, and cardiologic abnormalities, and other conditions that imitate adult systemic lupus erythematosus 1,3,4 . These abnormalities are transient if in tissues capable of continuous regeneration, such as the skin and the hematopoietic system, and irreversible in the cases of impairment of the cardiac conduction system 1 . In healthy pregnant women, the frequency of anti-Ro/ SSA antibodies through immunodiffusion or contraimmunoelectrophoresis varies around 0.4 and 1%, respectively, and of anti-La/SSB antibodies around 0.7% 5 . In patients with systemic lupus erythematosus, the frequency of antiRo/SSA antibodies is approximately 40% and that of antiLa/SSB antibodies is 15 to 20% 5 . In patients with Sjögren's syndrome, the frequency of anti-Ro/SSA antibodies is 40 to 45% and that of anti-La/SSB antibodies is 15 to 20% 5 . The general prevalence of neonatal lupus in newborns of mothers with systemic lupus erythematosus ranges from 1 to 3% 5 , and the general estimate of a child with congenital total atrioventricular block is 1:60, which increases to 1:20 9 if the mother has circulating anti-Ro/SSA antibodies 5-8 . In 1993, Goble et al 8 reported that newborns from mothers with systemic lupus erythematosus who developed the disease before or during pregnancy had an increase in heart rate (corrected for age) and in the PR interval in the electrocardiogram corrected for heart rate. Other disorders of cardiac electrical conduction in children of women with anti-Ro/SSA antibodies have been reported, and the classification of these disorders as manifestations of neonatal lupus is still controversial 9,10 . This study aimed at comparing, from the cardiovascular point of view, children of women with systemic lupus erythematosus and children of healthy women through physical examination, electrocardiography, 2-dimensional Doppler echocardiography, color flow Doppler, and signalaveraged electrocardiography. Objective -

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) Objetivo -Em ausência de alterações estruturais miocárdicas (AEM), avaliar se o bloqueio de ramo direito (BRD) gera potenciais fragmentados (PF) e turbulência espectral (TE) no eletrocardiograma de alta resolução (ECGAR). Métodos -Doze crianças com comunicação interatrial (CIA) e bloqueio incompleto do ramo direito (BIRD) sem AEM (Grupo I), foram comparadas com 17 crianças com tetralogia de Fallot (TF) operada, BCRD e AEM, 5 com extra sístoles ventriculares e 2 com taquicardia ventricular sustentada (Grupo II). Todas fizeram ECGAR nos domínios do tempo (DT) e da freqüência (DF), com cinco variáveis analisadas. Resultados -Os pacientes do grupo I tiveram as variáveis normais apesar do BIRD. No grupo II, 4 das cinco variáveis foram anormais, sugerindo a presença de PF e TE atribuíveis a AEM inerentes à malformação e ao ato cirúrgico.Conclusão -Na CIA o BIRD não complicado de AEM não gera PF e TE, não constituindo fator de risco para taquicardia ventricular sustentada. Palavras

Background Percutaneous occlusion of ductus arteriosus is well established as the method of choice to treat this structural heart defect. A new generation of Amplatzer™ Duct Occluder II Aditional Sizes prostheses, with lower profile and greater flexibility, in addition to smaller retention discs, was developed for percutaneous treatment of patent ductus arteriosus. This study intended to demonstrate the experience of one center with the use of this device, evaluating technical aspects, immediate occlusion rates, and complications. Methods A retrospective study of a cohort of patients with patent ductus arteriosus treated with Amplatzer™ Duct Occluder II Aditional Sizes. Between October 2018 and March 2021, 27 patients with patent ductus arteriosus, types A and E according to the Krichenko classification, were treated with Amplatzer™ Duct Occluder II Aditional Sizes. The prosthesis was implanted by the usual anterograde approach in 25 patients, and retrograde in two cases. The prosthesis chosen were 2mm larger than the core of the defect, and the length of the prosthesis was 2mm for shorter lesions and smaller infants, and between 4 and 6mm for longer lesions. Results Out of 27 patients, 52% were female, with a mean age and weight of 44.2 months (1 to 135 months) and 15.8kg (2,0 to 29kg), respectively. In the sample, 11 patients presented type A patent ductus arteriosus, and 16 type E, with a mean ratio of 1.9:1 between the prosthesis waist and the central diameter. In all patients, the device was successfully implanted, and only one patient remained with residual shunt, presenting embolization of the prosthesis. This patient had the ductus arteriosus closed by another prosthesis. Conclusion Amplatzer™ Duct Occluder II Aditional Sizes demonstrated safety, versatility, and efficiency in cases of appropriate anatomy. The experience demonstrated here is from a single center, with the Amplatzer™ Duct Occluder II Aditional Sizes. Its increased flexibility and softness demand greater operator expertise.

Marfan's 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.