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A reappraisal of the sinus venosus defect

Lookup NU author(s): Professor Bob Anderson


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© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.OBJECTIVES: The phenotypic features and morphogenesis of the 'sinus venosus defect' remain controversial. The phenotypic features are anomalous systemic connections of 1 or more pulmonary veins that retain their left atrial connection, usually associated with a biatrial connection of the superior caval vein. Cases with these features, however, have not always been described as sinus venosus defects. METHODS: We reviewed the findings documented in the literature from 11 patients with a biatrial connection of the superior caval vein, most reported following an autopsy examination. We compared these findings with the anatomical details of 50 patients undergoing surgical correction in our centre, paying particular attention to the override of the superior caval vein. RESULTS: In only two-thirds of those undergoing surgery did the superior caval vein override the rims of the oval fossa, with the degree of override >50% in only 2 individuals. It is only these latter 2 cases that are directly comparable to the reported cases of biatrial connection of the superior caval vein. CONCLUSIONS: Our comparisons provide new insights into the developmental background and phenotypic features of the superior sinus venosus defect. The defects exist because of the anomalous systemic connection of the pulmonary veins that retain their left atrial connections but not always in association with a biatrial connection of the superior caval vein. In extreme cases, nonetheless, they can underscore the connection of the caval vein to the morphologically left atrium, frequently described previously as a 'biatrial connection'. The sinus venosus defect is better considered a venovenous malformation than a septal defect.

Publication metadata

Author(s): Chowdhury UK, Anderson RH, Pandey NN, Sharma S, Sankhyan LK, George N, Goja S, Arvind B

Publication type: Review

Publication status: Published

Journal: European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery

Year: 2022

Volume: 61

Issue: 6

Pages: 1211-1222

Print publication date: 01/06/2022

Online publication date: 28/01/2022

Acceptance date: 30/12/2021

ISSN (print): 1010-7940

ISSN (electronic): 1873-734X

Publisher: NLM (Medline)


DOI: 10.1093/ejcts/ezab556

PubMed id: 35090016