Desembocadura anómala de la vena umbilical en aurícula derecha / Umbilical venous anomaly flow in right atrium

La introducción de la ecografía de alta resolución con Doppler color ha sido el mayor avance en la evaluación del sistema venoso fetal. El diagnóstico prenatal de las anomalías de este sistema son raras y sólo han sido descritos casos aislados. Las implicaciones hemodinámicas de los diferentes patrones de anomalías venosas fetales no se conocen del todo, en parte debido a que el sistema venoso fetal no ha sido estudiado en detalle. La vena umbilical que drena directamente en estructuras cardíacas derechas con ausencia del ductus venoso produce hidropesía fetal y está asociado a mortalidad, anomalías cromósomicas y malformaciones congénitas. Revisamos la literatura y presentamos un segundo caso de ausencia de vena umbilical intrahepática con conexión directa a aurícula derecha, ausencia de ductus venoso y cardiomegalia con predominio de cavidades izquierdas. La evaluación cuidadosa del sistema venoso umbilical y del ductus venoso debe formar parte de la rutina ante un feto con fallo cardíaco, cardiomegalia inexplicada e hidropesía fetal

The introduction of high-resolution ultrasonography with color Doppler offered a breakthrough in the evaluation of the human fetal venous system. Prenatal diagnosis of anomalies of these system are rare, and only anecdotal cases have been described. The hemodynamic implications of different pattern of venous fetal anomalies are probably not well understood because the fetal venous vein systems have not been studied in detail. The umbilical vein wich drains directly into right-sided structures with absent ductus venosus causes hydrops fetalis and its associated with mortality, chromosomal anomalies and congenital malformations. We reviewed the literature and presented the second case of intrahepatic absence of umbilical vein with no evidence of venous ductus and direct connection of the umbilical vein to right atrium with left heart overload. Careful assesment of the umbilical venous system and the ductus venosus should be part of the routine evaluation of fetus with heart failure, unexplained cardiomegaly and fetal hydrops (AU)

¿Miocardiopatía arritmogénica o reemplazo graso del ventrículo derecho? A propósito de dos casos / Arrhythmogenic cardiomiopathy of patty replacement of the right ventricular myocardium? Report of two cases

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Absceso cerebral secundario a cardiopatía congénita cianógena / Brain abscess secondary to cyanogenic congenital heart disease

Las cardiopatías congénitas cianógenas (CCC) son el factor predisponerte más importante en la aparición del absceso cerebral en la infancia. El diagnóstico del absceso cerebral en fase inicial es difícil pues se confunde a menudo con otros procesos neurológicos tales como tumores cerebrales, infartos o hemorragias, como sucedió en nuestro caso. La TC Craneal con contraste es el examen de elección para el diagnóstico precoz en cualquier niño con CCC que presente sintomatología neurológica. La RM con espectroscopia puede ser de gran ayuda en casos dudosos. El diagnóstico y tratamiento precoces disminuyen claramente la mortalidad y morbilidad de esta patología, permite iniciar tratamiento lo más pronto posible, hecho que se correlaciona con un mejor pronóstico. Presentamos el caso de un niño con una CCC y clínica neurológica de instauración aguda y recurrente, secundaria a un absceso cerebral y cuyo diagnóstico ofreció serias dudas en las fases iniciales (AU)

[Treatment of cardiac arrhythmia with radiofrequency in pediatrics]. / Tratamiento de arritmias cardíacas con radiofrecuencia en pediatría.

OBJECTIVE: The use of radiofrequency ablation of cardiac arrhythmias in pediatrics requires demonstration that the technique is effective and devoid of significant complications. In this study we present our experience in the ablation of cardiac arrhythmias in children and adolescents. PATIENTS AND METHODS: Between January 1992 and January 1997 we performed a total of 1,543 radiofrequency ablation procedures. Of these, 130 were performed in 117 patients younger than 18 years of age (58 were younger than 14 years old). Indication for ablation was the presence of drug refractory recurrent paroxysmal supraventricular tachycardia in 112 patients and permanent ventricular preexcitation in 5 asymptomatic patients. Final diagnosis in the 112 symptomatic patients was: 4 atrial tachycardias, 21 atrio-ventricular nodal reentrant tachycardias, 53 Wolff-Parkinson-White (WPW) syndromes, 33 orthodromic tachycardias using a concealed accessory pathway and 1 idiopathic left ventricular tachycardia. RESULTS: The initial ablation procedure was effective in 109/117 patients (93%) with a total duration of 90 +/- 31 minutes, using 16 +/- 11 minutes of radioscopy and a median of 4 radiofrequency applications. During follow-up, recurrence occurred in 9 patients (8%). In 7 of them and in 6 of those with an unsuccessful initial procedure, a second effective procedure was performed in 11/13 patients. Finally, radiofrequency ablation was effective in 111/117 patients (95%). We observed a single complication in a 15 year old girl who presented a thrombosis of the right femoral artery requiring balloon recanalization. Comparison of these results with those in the adult population showed no difference in terms of effectiveness, recurrences or complications. CONCLUSIONS: Radiofrequency ablation of cardiac arrhythmias in pediatric and young patients can be safely and effectively done. Results are similar to those obtained in adults suggesting that indications for ablation can also be similar.

[2-dimensional and Doppler echocardiography during atrioseptostomy using the Rashkind method. Alternative umbilical approach in the newborn infant]. / Ecocardiografía 2-D y Doppler durante la atrioseptostomía con técnica de Rashkind. Vía umbilical alternativa en el recién nacido.

Balloon atrial septostomy was performed in 16 patients using 2-D echocardiography (20 D Echo). Patients, 8 hours to 27 days old (5.6 +/- 8.5 days) had cyanotic congenital heart disease: (13 transposition of the great arteries (TGA), 2 pulmonary atresia (AP) and one mitral atresia (AM). In seven patients procedure was performed by way of saphenous-femoral vein. In the nine remaining it was done through umbilical vein, being successful in seven patients (it was necessary to use the saphenous-femoral vein in two). Seven in whom it was possible to use umbilical vein were less than 24 hours old. No complications arose as a consequence of technique, although in one, dissection performed by saphenous vein damaged femoral artery. AS was considered effective in all cases. There was no difference between results obtained by umbilical or saphenous-femoral veins. Umbilical approach proved to be simplest, fastest, and more reliable than saphenous-femoral access in the AS of those patients under 24 hours of age. Special importance is given to the value of 2-D Echo in performing AS and the immediate evaluation of the results and functional evaluation by pulsed Doppler echocardiography.

[Persistent tubular Nephrogram in a newborn (author's transl)]. / Nefrograma tubular persistent en el recién nacido. A propoósito de un caso.

The case presented is that of a newborn with palpable renal masses and oliguria that lasted for the first five days of live. The pyelography that was perfomed showed a prolonged tubular nephrogram and a light increase in kidney size. The good general state, normal test results, a kidney biopsy which failed to show any histological changes and its favorable development, suggested a diagnosis of transitory tubular obstruction caused by protein casts. It is suggested the diagnosis of the Tamm Horsfall proteinuria in the light of the works published in which similar case histories and urograms were cited. These works proved the presence of Tamm Horsfall mucoprotein by the use of specific immunofluorescence tests.

[Scurvey, presentation of six cases (author's transl)]. / Escorbuto. A propósito de seis casos.

Authors present six cases of scurvy admitted in their hospital from September 1977 to July 1978. Symptoms were similar in the majority of them: irritability, skin haemorrhages, swollen gums, scorbutic rosary, swelling and tenderness lower limbs. Radiographic findings more common were: Fraenkels' line "ground glass" appearance, corner sign, Wymberger sign, and in some cases subperiosteal haemorrhages with calcification. Ascorbic acid levels were below normal values in all cases, and overload test showed typical pattern found on patients with scurvy. Special mention is made on the low incidence of this disease in the last few years and also the causes that have motived the existence of this six cases in such a short period of time are analyzed.