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Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis

The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as “heterotaxy.” We have previously shown th...

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Autores principales: Mori, Shumpei, Anderson, Robert H, Nishii, Tatsuya, Matsumoto, Kensuke, Loomba, Rohit S
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5431030/
https://www.ncbi.nlm.nih.gov/pubmed/28566826
http://dx.doi.org/10.4103/apc.APC_171_16
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author Mori, Shumpei
Anderson, Robert H
Nishii, Tatsuya
Matsumoto, Kensuke
Loomba, Rohit S
author_facet Mori, Shumpei
Anderson, Robert H
Nishii, Tatsuya
Matsumoto, Kensuke
Loomba, Rohit S
author_sort Mori, Shumpei
collection PubMed
description The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as “heterotaxy.” We have previously shown that, when assessed on the basis of the extent of the pectinate muscles relative to the atrioventricular junctions, there is always isomerism of the atrial appendages in this setting. Doubt has been remained, however, as to whether these cardiac features can accurately be recognized during life. We have now encountered two patients showing features of the left and right bodily isomerism. Examinations of these patients made using computed tomography show that all features of isomerism, no matter how complex, can now be visualized during life. The images currently presented show, furthermore, that the features of the so-called “heterotaxy” can be seen during life, not only within the heart but also in all the thoracic and abdominal organs, albeit that the isomeric features are confined to the thoracic organs. Based on the images presented, we argue that if each system of organs is analyzed and described in independent fashion; then it is possible for clinicians to exclude any suggestion of ambiguity and to provide accurate descriptions of the overall arrangement. We further discuss the appropriate terminology to describe the entity we prefer to call isomerism, along with the indications and usefulness of computed tomography in revealing the anatomic features of the congenitally malformed heart.
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spelling pubmed-54310302017-05-31 Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis Mori, Shumpei Anderson, Robert H Nishii, Tatsuya Matsumoto, Kensuke Loomba, Rohit S Ann Pediatr Cardiol Pictorial Essay The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as “heterotaxy.” We have previously shown that, when assessed on the basis of the extent of the pectinate muscles relative to the atrioventricular junctions, there is always isomerism of the atrial appendages in this setting. Doubt has been remained, however, as to whether these cardiac features can accurately be recognized during life. We have now encountered two patients showing features of the left and right bodily isomerism. Examinations of these patients made using computed tomography show that all features of isomerism, no matter how complex, can now be visualized during life. The images currently presented show, furthermore, that the features of the so-called “heterotaxy” can be seen during life, not only within the heart but also in all the thoracic and abdominal organs, albeit that the isomeric features are confined to the thoracic organs. Based on the images presented, we argue that if each system of organs is analyzed and described in independent fashion; then it is possible for clinicians to exclude any suggestion of ambiguity and to provide accurate descriptions of the overall arrangement. We further discuss the appropriate terminology to describe the entity we prefer to call isomerism, along with the indications and usefulness of computed tomography in revealing the anatomic features of the congenitally malformed heart. Medknow Publications & Media Pvt Ltd 2017 /pmc/articles/PMC5431030/ /pubmed/28566826 http://dx.doi.org/10.4103/apc.APC_171_16 Text en Copyright: © 2017 Annals of Pediatric Cardiology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Pictorial Essay
Mori, Shumpei
Anderson, Robert H
Nishii, Tatsuya
Matsumoto, Kensuke
Loomba, Rohit S
Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis
title Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis
title_full Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis
title_fullStr Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis
title_full_unstemmed Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis
title_short Isomerism in the setting of the so-called “heterotaxy”: The usefulness of computed tomographic analysis
title_sort isomerism in the setting of the so-called “heterotaxy”: the usefulness of computed tomographic analysis
topic Pictorial Essay
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5431030/
https://www.ncbi.nlm.nih.gov/pubmed/28566826
http://dx.doi.org/10.4103/apc.APC_171_16
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