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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: Puneet.Bhatla@nyumc.org
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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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References

1. Oliver, JM, Gallego, P, Gonzalez, A, Dominguez, FJ, Aroca, A, Mesa, JM. Sinus venosus syndrome: atrial septal defect or anomalous venous connection? A multiplane transoesophageal approach. Heart 2002; 88: 634–638.CrossRefGoogle ScholarPubMed
2. Al Zaghal, AM, Li, J, Anderson, RH, Lincoln, C, Shore, D, Rigby, ML. Anatomical criteria for the diagnosis of sinus venous defects. Heart 1997; 78: 298–304.Google Scholar
3. Van Praagh, S, Carrera, ME, Sanders, SP, Mayer, JE, Van Praagh, R. Sinus venosus defects: unroofing of the right pulmonary veins – anatomic and echocardiographic findings and surgical treatment. Am Heart J 1994; 128: 365–379.CrossRefGoogle ScholarPubMed
4. Rose, C. Zue entwickelungsgeschichte des Suagetierherzens. Morphol Jahrb 1889; 15: 436–456.Google Scholar
5. Butts, RJ, Crean, AM, Hlavacek, AM, et al. Veno-venous bridges: the forerunners of the sinus venosus defect. Cardiol Young 2011; 21: 623–630.CrossRefGoogle ScholarPubMed
6. Mohun, TJ, Weninger, WJ. Imaging heart development using high-resolution episcopic microscopy. Curr Opin Genet Dev 2011; 21: 573–578.CrossRefGoogle ScholarPubMed
7. Anderson, RH, Mohun, TJ, Brown, NA. Clarifying the morphology of the ostium primum defect. J Anat 2015; 226: 244–257.CrossRefGoogle ScholarPubMed
8. Anderson, RH, Webb, S, Brown, NA. Clinical anatomy of the atrial septum with reference to its developmental components. Clin Anat 1999; 12: 362–367.Google Scholar
9. Crystal, MA, Al Najashi, K, Williams, WG, Redington, AN, Anderson, RH. Inferior sinus venosus defect: echocardiographic diagnosis and surgical approach. J Thorac Cardiovasc Surg 2009; 137: 1349–1355.CrossRefGoogle ScholarPubMed
10. Plymale, J, Kolonski, K, Frommelt, P, Bartz, P, Tweddell, J, Earling, MG. Inferior sinus venosus defects: anatomic features and echocardiographic correlates. Pediatr Cardiol 2013; 34: 322–326.CrossRefGoogle ScholarPubMed
11. Anderson, RH, Brown, NA, Moorman, AF. Development and structures of the venous pole of the heart. Dev Dyn 2006; 235: 2–9.Google Scholar
12. Webb, S, Brown, NA, Anderson, RH, Richardson, MK. Relationship in the chick of the developing pulmonary vein to the embryonic systemic venous sinus. Anat Rec 2000; 259: 67–75.3.0.CO;2-5>CrossRefGoogle Scholar
13. Webb, S, Brown, NA, Wessels, A, Anderson, RH. Development of the murine pulmonary vein and its relationship to the embryonic venous sinus. Anat Rec 1998; 250: 325–334.3.0.CO;2-Z>CrossRefGoogle Scholar
14. Webb, S, Kanani, M, Anderson, RH, Richardson, MK, Brown, NA. Development of the human pulmonary vein and its incorporation in the morphologically left atrium. Cardiol Young 2001; 11: 632–642.Google Scholar
15. Sizarov, A, Anderson, RH, Christoffels, VM, Moorman, AF. Three-dimensional and molecular analysis of the venous pole of the developing human heart. Circulation 2010; 122: 798–807.Google Scholar
16. Blom, NA, Gittenberger-de Groot, AC, Jongeneel, TH, DeRuiter, MC, Poelmann, RE, Ottenkamp, J. Normal development of the pulmonary veins in human embryos and formulation of a morphogenetic concept for sinus venosus defects. Am J Cardiol 2001; 87: 305–309.Google Scholar
17. DeRuiter, MC, Gittenberger-De Groot, AC, Wenink, AC, Poelmann, RE, Mentink, MM. In normal development pulmonary veins are connected to the sinus venosus segment in the left atrium. Anat Rec 1995; 243: 84–92.Google Scholar
18. Mahtab, EA, Vicente-Steijn, R, Hahurij, ND, et al. Podoplanin deficient mice show a RhoA-related hypoplasia of the sinus venosus myocardium including the sinoatrial node. Dev Dyn 2009; 238: 183–193.CrossRefGoogle ScholarPubMed
19. Anderson, RH, Brown, NA, Mohun, TJ. Insights regarding the normal and abnormal formation of the atrial and ventricular septal structures. Clin Anat 2016; 29: 290–304.Google Scholar