How to determine atrial situs? Considerations initiated by 3 cases of absent spleen with a discordant anatomy between bronchi and atria.

Abstract

Three cases have been documented in which abdominal heterotaxy with absent spleen was accompanied by a discordant anatomy between the tracheobronchial tree and the atria. In 1 instance the tracheobronchial anatomy was as in situs inversus but the venous connections and the atrial appendages suggested situs solitus. The second case exhibited a situs solitus anatomy of the tracheobronchial tree, but the morphological features of the atria were mixed up in the sense that the venous connections and atrial septum suggested situs inversus, whereas the atrial appendages were of a right isomeric type. The third case was interesting in showing a symmetrical tracheobronchial tree not accompanied by isomeric atrial appendages. The latter and the atrial septum exhibited situs solitus characteristics, though the venous connections suggested 'situs ambiguus'. Such observations have led us to reconsider the criteria that determine the classification of atrial situs. It is our contention that in cases characterised by major abnormalities in atrial anatomy, particularly with respect to the venous connections, the prime feature for identification of atrial situs would be the morphology of the atrial appendages, which represent the 'true' atrial Anlagen. Positive identification of atrial appendages would also enable a more precise definition of the term 'atrial situs ambiguus'. This term can then be defined as the situs characterised by either bilateral right-or bilateral left-sided morphological characteristics of the atrial appendages, irrespective of the venous connections or atrial septal anatomy.

Abnormal laterality and congenital cardiac anomalies. Relations of visceral and cardiac morphologies in the iv/iv mouse.

“…Such discordance has also been observed in humans. 27 Individual cardiac lesions found in the mouse, as detailed in Table 6, also show a similar spectrum of known malformations in humans. Atrioventricular septal defect, abnormal ventriculoarterial connections, pulmonary atresia, and anomalous pulmonary venous connections are particularly common findings in hearts with isomerism of right appendages in humans.28 The present study of murine right isomerism recapitulates most of this spectrum, albeit that anomalous pulmonary return was not detected.…”

Left bronchial isomerism associated with bronchomalacia, presenting with intractable wheeze.

“…These anatomical features have been described only once before. 6 We were in doubt about how to manage this child. There are no published reports of bronchial stenting in children, other than in the setting ofheart-lung transplantation.…”

Marcadores Sonograficos De Cardiopatias Congenitas. Interrupcion De La Vena Cava Inferior: A Proposito De Nuestra Experiencia Y Resultados

“…A pesar que no se adviertan defectos cardíacos durante la vida intrauterina, el estudio postnatal debe siempre incluir una ecocardiografía y una evaluación ecográfica abdominal, buscando dirigidamente malformaciones no pesquisadas a nivel cardíaco y las alteraciones extracardíacas asociadas a la poliesplenia (20,21). La asociación de interrupción de la VCI con canal auriculoventricular, bloqueo aurículoventriculr completo y poliesplenia se asocia a elevada mortalidad perinatal.…”