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Determining bronchial morphology for the purposes of segregating so-called heterotaxy

Published online by Cambridge University Press:  10 July 2015

Rohit S. Loomba ,
Andrew N. Pelech ,
Parinda H. Shah  and
Robert H. Anderson
Rohit S. Loomba*
Affiliation:
Children’s Hospital of Wisconsin, Division of Cardiology, Milwaukee, Wisconsin, United States of America
Andrew N. Pelech
Affiliation:
Children’s Hospital of Wisconsin, Division of Cardiology, Milwaukee, Wisconsin, United States of America
Parinda H. Shah
Affiliation:
Advocate Illinois Masonic Medical Center, Division of Radiology, Chicago, Illinois, United States of America
Robert H. Anderson
Affiliation:
Institute of Genetic Medicine, Newcastle University, Newcastle Upon Tyne, United Kingdom
*
Correspondence to: R. S. Loomba, Children’s Hospital of Wisconsin, Division of Cardiology, 9000 Wisconsin Avenue, Milwaukee, WI 53226, United States of America. [email protected]
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Abstract

Introduction

Heterotaxy is a unique clinical entity in which lateralisation of the thoraco-abdominal organs is abnormal, typically with isomerism of the bronchial tree and atrial appendages. This study was carried out to determine whether routine clinical imaging such as chest radiographs, angiographic images, and CT/MRI can determine bronchial isomerism, and how sidedness of bronchial isomerism correlates with overall features anticipated in hearts with isomeric atrial appendages.

Methods and results

We identified 73 patients with heterotaxy, in whom imaging clearly demonstrated the bronchial tree, seen at our institution since 1998. We calculated bronchial angles and lengths using all the available imaging modalities to determine the presence and sidedness of bronchial isomerism. This was then compared with the anticipated presence of isomeric atrial appendages based on the overall clinical findings, as the appendages themselves had not specifically been imaged.

The ratio of bronchial lengths revealed bronchial isomerism in all patients, with bronchial angles permitting distinction of right as opposed to left isomerism. We noted discordances between the identified bronchial isomerism and the presumed arrangement of the atrial appendages in nearly 20% of the patients in our cohort.

Conclusion

Routine clinical imaging with chest radiographs, angiographic imaging, and CT/MRI can determine the presence of bronchial isomerism in patients with so-called heterotaxy. Right as opposed to left isomerism can be distinguished based on bronchial angles. The finding of bronchial isomerism correlates well, but not totally, with the presumed isomerism of the atrial appendages as predicted from the identified intra-cardiac morphology.

Keywords

Bronchial morphologyisomerismheterotaxyX-rayCT
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 4 , April 2016 , pp. 725 - 737
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Copyright
© Cambridge University Press 2015 

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