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Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings

Published online by Cambridge University Press:  03 April 2017

Cornelia Tremblay ,
Rohit S. Loomba ,
Peter C. Frommelt ,
Donald Perrin ,
Diane E. Spicer ,
Carl Backer  and
Robert H. Anderson
Cornelia Tremblay
Affiliation:
Hospital for Sick Children, Toronto, Ontario, Canada
Rohit S. Loomba*
Affiliation:
Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Peter C. Frommelt
Affiliation:
Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Donald Perrin
Affiliation:
Hospital for Sick Children, Toronto, Ontario, Canada
Diane E. Spicer
Affiliation:
Department of Pediatric Cardiology, University of Florida, Gainesville, Florida, United States of America Johns Hopkins All Children’s Heart Institute, St. Petersburg, Florida, United States of America
Carl Backer
Affiliation:
Ann & Robert H. Lurie Children’s Hospital, Feinberg School of Medicine, 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, Department of Cardiology, Children’s Hospital of Wisconsin, 9000 Wisconsin Avenue, Milwaukee, WI 53226, United States of America. Tel: 414-266-2082; Fax: 414-266-2000; E-mail: loomba.rohit@gmail.com
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Abstract

Background

Bodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography.

Methods

We reviewed postmortem specimens of hearts from the archives at four institutions categorised as obtained from patients with “heterotaxy”. The cardiac structures were analysed using sequential segmental analysis. Non-cardiac structures were also examined if available. Statistical analyses were performed to compare differences in the settings of right as opposed to left isomerism.

Results

Specimens were available from 188 patients. Of these, 57 had left isomerism, and 131 had right isomerism. Atrial appendages were isomeric in all patients. A coronary sinus was found only in left isomerism, whereas a terminal crest, or a Eustachian valve, was found only in right isomerism. Interruption of the inferior caval vein was associated with left isomerism, whereas totally anomalous pulmonary venous connection was associated with right isomerism.

Conclusion

Isomerism is uniformly segregated on the basis of the morphology of the atrial appendages, itself defined by the extent of the pectinate muscles. Other features such as the presence of a coronary sinus and systemic venous return can further help with such segregation of isomerism.

Keywords

Heterotaxyisomerismechocardiographycoronary sinusatrial appendages
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 8 , October 2017 , pp. 1470 - 1480
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Copyright
© Cambridge University Press 2017 

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Footnotes

*

Cornelia Tremblay and Rohit S. Loomba are both first authors on this review with a shared first co-authorship.

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