To judge whether an Amplatzer™ Septal Occluder can be used as standard therapy instead of surgery for closure of atrial septal defects we report our experiences in 200 patients. Of these patients, 127 had an atrial septal defect with haemodynamically significant left-to-right shunt, 68 patients a persistent oval foramen after presumed paradoxical embolism, and 5 had a fenestration after Fontan-repair. Mean age was 29.8 years (0.8 to 77.7 years). Body weight ranged from 6.9 to 120.0 kg (mean 51.5 kg). After diagnostic cardiac catheterization, and balloon-sizing of the defect, we implanted Amplatzer™ Septal Occluders with stents of 4 to 28 mm diameter. Follow-up studies were obtained after 48hours, and one, six, and twelve months. Transcatheter closure of the atrial septal defect proved successful in all without any relevant residual shunts. In particular, complete closure was achieved in all patients after presumed paradoxical embolism. The mean period of follow-up is 9–5 months, with a range from 0.4 to 23.5 months, giving a total of 1898 patient months. The occlusion rate after three month was 98.1°. A trivial haemodynamically insignificant residual shunt remained in 1.9° of the patients. Fluoroscopy times ranged from 0 to 43.5 minutes, with a median of 8.7 minutes. The excellent results in the short and medium term in children and adults have resulted in using this device routinely at the present time for closure of central atrial septal defects up to a diameter of 28 mm. Final judgement, however, is only possible after long-term follow-up.

We report two autopsy proven cases of unguarded mitral orifice associated with mirror-imaged atrial arrangement, discordant atrioventricular connections, double outlet right ventricle, pulmonary valvar stenosis or atresia, and atrialisation of the morphologically left ventricle. The morphologically left atrioventricular junction was devoid of valvar leaflets, and there was no tension apparatus within the ventricle. To the best of our knowledge, this is the first description of this rare cardiac malformation

Our study is based on the examination of 2,000 aortic valves obtained from fresh cadavers (1,499 males, 501 females) at the Institute of Forensic Medicine in Rio de Janeiro. We discovered 13 valves having two leaflets, giving a prevalence of 0.65%, much lower than generally reported in the literature. All 13 valves were from males, 10 from whites, three from mulattos, and none from blacks. Special attention was given to the raphe and the leaflets, the calcification of which can lead to stenosis. The only valves with normal texture and flexibility were two obtained from children less than one year old. All the other valves were thickened, and five of them had some degree of calcification. Six valves were judged to be functioning normally, while 7 valves were abnormal, 5 being stenotic and two showing evidence of insufficiency. The insufficiency in one was due to endocarditis, but in the other was due to redundancy of the leaflets. The only other cardiac anomaly discovered in these 13 cases was one patient with aortic coarctation. Bifoliate aortic valve, therefore, is probably the most common cardiac anomaly, although its prevalence as discovered in Brazil is lower than that reported in the literature. It affects mainly white males. After the fourth decade of life, most valves present some thickening, with stenosis being the most common complication. As is well recognised, infective endocarditis and aortic insufficiency are the other frequent complications. It is in general, nonetheless, an isolated anomaly.

A BIFOLIATE CONDITION OF THE PULMONARY: valve may occur as an isolated phenomenon in otherwise normal hearts or in association with other anomalies in congenitally malformed hearts. The former condition is very rare, whereas the latter condition is quite common.

The natural history of a ductal aneurysm detected prenatally is presented. Neurologic concerns in the neonate and absence of cardio-respiratory compromise permitted serial echo demonstration of constriction, thickening and subsequent closure with formation of thrombus in the aneurysm.

Cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.

Reported here is an obstruction of the right ventricular outflow tract caused by a tuberculoma in a 15-year-old boy who presented with a ventricular septal defect. The obstruction was discovered at surgery and the tuberculous aetiology was only demonstrated histologically. To the authors' knowledge, this is the first report of a tuberculoma of the heart associated with congenital heart disease.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

A 6-day-old male with interruption of the aortic arch at the isthmus (type A) had the typical phenotype of DiGeorge syndrome. There was also a doubly committed juxta-arterial ventricular septal defect and an unobstructed left ventricular outflow tract. Hypoplasia of the thymus was confirmed during a modified Blalock-Park operation. He had persistent hypocalcemia, and was susceptible to infection. He was subsequently revealed by the use of fluorescence in situ hybridization analysis to have 22q11.2 deletion. Interruption of the aortic arch at the isthmus is presumed to reflect abnormal fetal hemodynamics, and is considered a distinct pathogenetic entity from interruption between the left common carotid and subclavian arteries, the latter being the variant more frequently associated with DiGeorge syndrome. In our case, the 22q11.2 deletion likely played a major role in the etiology of the interrupted aortic arch.

Reported is a child with dilated cardiomyopathy, in whom medical therapy resulted in a mild improvement of cardiac function. Metabolic studies suggested the presence of a catecholamine-secreting tumour; and an adrenal neuroblastoma was identified and surgically removed. Following surgery, there was progressive and complete normalization of cardiac function. Although very rare, neurogenic tumours may be involved in the development of a dilated cardiomyopathy in the infant and child.

The authors discovered congenital absence of the portal vein, with visceral venous return to the right atrium, in a 5-year-old girl with aortic valvar stenosis. Interestingly, of the 19 patients, it was discovered that 11 reported with portal venous agenesis also had cardiac defects. We have, therefore, investigated the hypothesis that the congenital absence of the portal vein and the associated cardiac malformations may result from a similar embryologic insult, and that cardiac development may be affected by the systemic diversion of portal venous flow.

The syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. Pericardiectomy is the treatment of choice.