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Discordances between the different types of atrial arrangement and the positions of the thoraco-abdominal organs

Published online by Cambridge University Press:  15 August 2006

Ivan Machado-Atias
Affiliation:
Unidad de Estudio de la Patología Cardiovascular del Niño, Instituto de Anatomía Patológica, Universid ad Central de Venezuela, Caracas, Venezuela
Guillermo Anselmi
Affiliation:
Unidad de Estudio de la Patología Cardiovascular del Niño, Instituto de Anatomía Patológica, Universid ad Central de Venezuela, Caracas, Venezuela
Ivan Machado-Hernandez
Affiliation:
Unidad de Estudio de la Patología Cardiovascular del Niño, Instituto de Anatomía Patológica, Universid ad Central de Venezuela, Caracas, Venezuela
Cecilia Febres
Affiliation:
Unidad de Estudio de la Patología Cardiovascular del Niño, Instituto de Anatomía Patológica, Universid ad Central de Venezuela, Caracas, Venezuela

Abstract

Numerous investigations have addressed the most appropriate anatomical structures to be used for the identification of segmental arrangement (“situs”). Variations from normal have been termed “heterotaxy syndromes”. The assumption is frequently made that, in such instances, all the organs in a given individual case follow the same rules for segmental morphology. This leads to confusion. The starting point for cardiac analysis is the structure of the atriums. On this basis, we can define viscero-atrial discordance when, in usual arrangement, mirror imagery, and heterotaxy, the atriums and their appendages are no longer concordant with the remaining thoracic and abdominal organs. With this in mind, we examined a large number of autopsied specimens to identify all possible variations of thoracic structures and abdominal organs relative to atrial arrangement. We analyzed 1100 malformed hearts. Of these, 1046 had usual arrangement with normally situated heart (95%); 10 cases had usual arrangement with right-sided heart (0.90%); 25 showed mirror imagery (2.27%), and 19 were found with heterotaxy (1.72%). There was a total of 54 hearts of usual arrangement with right-sided heart, mirror imagery and heterotaxy, (4.90%) and, amongst them, 17 showed viscero-atrial discordances (31.48%). In those having usual arrangement with right-sided heart, there were 2 cases (20%); 8 in those with mirror imagery (32%); and 7 in heterotaxy (36.8%). In the 1046 cases of usual arrangement with normally situated heart, there were 12 examples of viscero-atrial discordances (1.14%). In all, we found a total of 29 cases of viscero-atrial discordances. Taken together, the discordances are best dealt with by describing separately, in every case of normal or abnormal atrial arrangement, the thoracic structures, the atrial appendages, and the abdominal organs. In the final analysis, the diagnosis of the cardiac arrangement rests on the location and morphology of the atriums and their appendages.

Type
Original Article
Copyright
2001 Cambridge University Press

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