[Three-dimensional echocardiography: preliminary experience in congenital cardiac disease]. / Ecocardiografía tridimensional: experiencia preliminar en las cardiopatías congénitas.

INTRODUCTION: Anatomical comprehension of congenital cardiac diseases by 2D echocardiography is occasionally very difficult. 3D echocardiography provides a more spatial anatomical information avoiding the need of two-dimensional reconstruction. METHODS: Of the 271 cases studied 80 were foetal and 191 patients. In all cases, 2D and 3D echocardiography was performed (Sonos 7500 with matrix probe). Four modes of 3D imaging were used. RESULTS: 3D echocardiography gave an accurate description of the size, form and wedges of septals defects. In atrioventricular septal defects and mitral anomalies, 3D echocardiography was useful for the assessment of dynamic valve morphology and mechanisms of regurgitation. In foetal screening the segmentary heart study was carried out from a single acoustic window. CONCLUSIONS: 3D real time echocardiography is a feasible, easy and rapid technique. It provides anatomical and functional details needed for an accurate comprehension of congenital cardiac diseases. In foetal screening, it provides an easier segmentary analysis of the entire foetal heart.

[Medical pericarditis: study of 19 cases]. / Pericarditis médicas: estudio de 19 casos.

19 cases of medical pericarditis admitted to the PICU from 1978 to 1989 were reviewed, to asses the diagnostic and therapeutical approach. The overall incidence is low, 0.32% of the patients admitted to the PICU in that time period. We have found neither tuberculous nor purulent pericarditis, with a 42% of idiopatic forms. Echocardiography was the best method for syndromic diagnosis. The ethiological diagnosis of pericarditis was made, in most cases, by means of non invasive methods or in association with the underlying disease. The cualitative analysis of the pericardial fluid was not useful for the ethiological diagnosis, therefore, we suggest pericardiocentesis should be performed mainly as a therapeutical tool. There were no correlation between the amount of fluid obtained on the first pericardiocentesis and the later incidence of the pericardial effusion. Biopsy brought no ethiological specificity in the cases it was performed.

[Total anomalous pulmonary venous drainage below the diaphragm. Report of three cases and semiologic considerations (author's transl)]. / Drenaje venoso pulmonar anómalo total infradiafragmático. Consideraciones semiológicas a propósito de tres casos.

Clinical, hemodynamic and pathologic findings of three patients with total anomalous pulmonary venous drainage to the portal vein system are presented. Some of the findings are emphasized because of its rarity in previous reports. These are: 1) Cyanosis was mild. 2) There was some obstruction of the foramen ovale, besides the obstruction at the portal system level. In two patients gastrointestinal bleeding and analitical evidence of renal failure, were found, both findings not previously reported to our knowledge.

Síndrome de Rendu-Osler-Weber: causa infrecuente de hipoxemia en la infancia / Rendu-Osler-Weber syndrome: an rare cause of hypoxemia in children

La telangiectasia hemorrágica hereditaria (HHT) o síndrome de Rendu-Osler-Weber es una entidad de las consideradas como «enfermedades raras» (afecta a uno de cada 3.000–8.000 individuos) cuya herencia autosómica dominante va determinada por la alteración en la codificación de los genes endoglina (ENG) y activin like Kinase 1, (ALK1) que causan el tipo 1 y 2 respectivamente de la HHT y conducen a displasia en el endotelio de la pared vascular debido a haploinsuficiencia para endoglina. Esto da lugar a una serie de manifestaciones clínicas que consisten básicamente en epistaxis repetidas, telangiectasias mucocutáneas y malformaciones arteriovenosas (MAV) viscerales. A continuación se presenta el caso clínico de un niño de 11 años que desarrolló hipoxemia franca debido a múltiples fístulas arteriovenosas pulmonares (AU)

Hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber syndrome is a rare genetic autosomic dominant disorder with an estimated prevalence of one in 3000–5000 individuals. This multisystemic vascular dysplasia is determined by the mutation of two main genes which are endoglin (ENG) or HHT1 and ALK1 or HHT2. These mutations induce the vascular disorders which cause recurrent epistaxis and eventually multiple telangiectasias and arteriovenous visceral malformations (AVM).We report the case of an 11-year-old boy who developed severe hypoxaemia due to multiple pulmonary arteriovenous malformations (AU)

Ecocardiografía tridimensional: experiencia preliminar en las cardiopatías congénitas / Three-dimensional echocardiography: preliminary experience in congenital cardiac disease

Introducción: Las cardiopatías congénitas pueden presentar una anatomía difícil de interpretar. La ecocardiografía tridimensional aporta imágenes únicas sin necesidad de reconstrucción mental anatómica tan a menudo necesaria en la ecocardiografía bidimensional. Material y métodos: Se estudiaron 271 casos: 80 registros fetales y 191 transtorácicos. En todos se realizó ecocardiografía bidimensional (2D) y tridimensional (3D) (equipo Philips Sonos 7500, con sonda matricial) con cuatro modalidades de ecografía 3D: 3D tiempo real, biplano, color y volumen completo. Resultados: En los defectos septales, esta técnica definió de manera precisa el tamaño, la forma y los bordes. En los canales auriculoventriculares y anomalías mitrales determinó la anatomía y mecanismos de regurgitación y estenosis. En el cribado fetal permitió un estudio segmentario del corazón fetal. Conclusiones: La ecocardiografía 3D en tiempo real es una técnica factible, sencilla y rápida. Aporta detalles anatómicos y funcionales esenciales en las cardiopatías congénitas. El rastreo fetal facilita un análisis segmentario completo del corazón fetal

Introduction: Anatomical comprehension of congenital cardiac diseases by 2D echocardiography is occasionally very difficult. 3D echocardiography provides a more spatial anatomical information avoiding the need of two-dimensional reconstruction. Methods: Of the 271 cases studied 80 were foetal and 191 patients. In all cases, 2D and 3D echocardiography was performed (Sonos 7500 with matrix probe). Four modes of 3D imaging were used. Results: 3D echocardiography gave an accurate description of the size, form and wedges of septals defects. In atrioventricular septal defects and mitral anomalies, 3D echocardiography was useful for the assessment of dynamic valve morphology and mechanisms of regurgitation. In foetal screening the segmentary heart study was carried out from a single acoustic window. Conclusions: 3D real time echocardiography is a feasible, easy and rapid technique. It provides anatomical and functional details needed for an accurate comprehension of congenital cardiac diseases. In foetal screening, it provides an easier segmentary analysis of the entire foetal heart