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We comment the results with non pharmacological treatment in 23 patients (mean age 11 +/- 4 years) with multiple episodes of palpitations, most of them required intravenous drugs for interrupting it. Twenty-eight procedures were performed, 17 catheter ablation and 11 surgical interventions. The energy source employed for fulguration until 1990 was DC and since 1991 radiofrequency. The best results with catheter ablation were obtained with accessory pathways. Interruption was achieved in all patients after a mean of 1.2 sessions per patient, but it was definitive in only 72%. Most of the patients (81%) remained asymptomatic without antiarrhythmic drugs. Nine patients with accessory pathways underwent surgery interruption, it was achieved in all; only one patient had arrhythmia recurrence, due to a second pathway in a different localization. We could not ablate a ectopic atrial tachycardia, that underwent surgery. There was only one indication for surgery in a patient with ventricular tachycardia. Catheter ablation does not present technical problem in children and teenagers, different from adults. The presented results lead us to suppose that the indications for non pharmacological treatment in the young will become more common.

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Between february and december 1990, we attempted percutaneous closure of patent ductus arteriosus (PDA) in 26 patients and in one ostium secundum atrial septal defect (ASD). One of the PDA was right sided with right aortic arch and two were post surgical leaks. We used the "USCI-Rashkind PDA double disc occluder" and the newly designed "Lock Clamshell" occluder for the ASD. Twenty seven patients (20 females and 7 males), aged 1.3 to 16 years (M = 5.6) and weighing 9.5 to 56 kg (M = 21.3), were studied. Diagnosis was made clinically with the aid of Doppler colour flow echocardiography, with follow-up studies at 24 hours, 6 months and 1 year after the procedure. Diameter of the ductus varied from 1.6 to 8.2 mm (M = 2.6). Successful implant of the device was achieved in 25 cases (95%), of which 18 (72%) were totally occluded at 24 hours and 21 (84%) after 6 months, remaining small residual leaks in four (16%). In only one patient total occlusion was demonstrated 1 year after device implantation. We achieved 22 (88%) total occlusions. One case was not possible to occlude, due to its large size. Angiocardiography demonstrated adequate anatomy in 18, and difficult in 8 patients. Twelve and 17 mm devices were used in 16 and 9 patients, respectively. Successful second implant with subsequent total occlusion was achieved in one patient with a previous moderate residual leak. The ASD was central and single, with a diameter of 20 mm. Immediate total occlusion was achieved. We conclude that percutaneous PDA closure is a safe and effective procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

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We describe a patient with angiographic findings of a left superior persistent vena cava draining in the left atrium with absence of the coronary sinus' roof and coexisting with Holmes' heart. The diagnosis can be made by selective angiography in the left atrium, pulmonary vein or at the left superior vena cava. We want to emphasize the importance of knowing this sort of anomaly before surgical treatment.

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Anomalies of the atrio-ventricular valves are frequent in cases of univentricular heart with severe symptomatology during the first year of life and in necropsy material. This suggests that they are important determinants of the natural history of the malformation. Radical "corrective" surgery, in the presence of these anomalies, may be less successful than palliative programs based on sound physiological grounds. Under such circumstances, enlargement or creation of an atrial septal defect may be necessary. A careful assessment of the state of the atrio-ventricular orifices has to be included in the cardiac catheterization protocol of cases of univentricular hearts. From a morphogenetic viewpoint, a single ventricular cavity is not the appropriate substrate for two normal atrio-ventricular valves to develop.

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Out of 1610 children's hearts with congenital malformations there were 27 specimens showing double outlet right ventricle. Cases with dextrocardia, situs inversus, or l-venticular loop were excluded. Anatomical examination was performed with particular reference to the infundibular region, the great vessels, and the ventricular septum. The commonest associated malformations were ventricular septal defect and pulmonary stenosis. Aortic stenosis was the predominant finding in those cases dying in the neonatal period. An aortic conus was associated with pulmonary stenosis, ventricular septal defect, and d-transposition, a pulmonary conus with ventricular septal defect and a double conus with stenosis of either great vessel. The anterior vessel always had a muscular conus and the posterior vessel was commonly stenotic.

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Seven cases of transposition with an unusually posterior aorta and mitral-aortic fibrous continuity are described. The cases are similar to four cases published by Van Praagh et al. in 1971. The use of the term 'p' transposition is suggested to distinguish this unusual form of transposition from cases with a subaortic conus ('d' and 'l' transposition). The use of the term 'transposition' is considered justifiable on both semantic and morphogenetic grounds and a possible common embryological mechanism for all forms of transposition is discussed.

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