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Understanding the spectrum of sinus venosus interatrial communications

Published online by Cambridge University Press:  10 May 2016

Justin T. Tretter ,
Sathish Chikkabyrappa ,
Diane E. Spicer ,
Carl L. Backer ,
Ralph S. Mosca ,
Robert H. Anderson  and
Puneet Bhatla
Justin T. Tretter
Affiliation:
Division of Pediatric Cardiology, New York University Langone Medical Center, New York, New York, United States of America
Sathish Chikkabyrappa
Affiliation:
Division of Pediatric Cardiology, New York University Langone Medical Center, New York, New York, United States of America
Diane E. Spicer
Affiliation:
Division of Pediatric Cardiology, University of Florida, Gainesville, Florida, United States of America Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St. Petersburg, Florida, United States of America
Carl L. Backer
Affiliation:
Division of Cardiovascular Surgery, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America
Ralph S. Mosca
Affiliation:
Division of Congenital Cardiac Surgery, New York University Langone Medical Center, New York, New York, United States of America
Robert H. Anderson
Affiliation:
Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom
Puneet Bhatla*
Affiliation:
Division of Pediatric Cardiology, New York University Langone Medical Center, New York, New York, United States of America Department of Radiology, New York University Langone Medical Center, New York, New York, United States of America
*
Correspondence to: Puneet Bhatla, MD, Division of Pediatric Cardiology, New York Langone Medical Center, Rivergate, 403 East 34th Street 4th floor, New York, New York 10016. Fax: +1-212-263-8301; E-mail: [email protected]
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Abstract

Background

It is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects.

Methods

We reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens.

Results

We evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases.

Conclusion

Appreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.

Keywords

Sinus venosus defectanomalous pulmonary veincardiac developmentatrial septal defect
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 3 , March 2017 , pp. 418 - 426
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Copyright
© Cambridge University Press 2016 

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