Objectives We sought to clarify the variations in the anatomy of the superior cavoatrial junction and anomalously connected pulmonary veins in patients with superior sinus venosus defects using computed tomographic (CT) angiography.
Methods CT angiograms of 96 consecutive patients known to have superior sinus venosus defects were analysed.
Results The median age of the patients was 34.5 years. In seven (7%) patients, the defect showed significant caudal extension, having a supero-inferior dimension greater than 25 mm. All patients had anomalous connection of the right superior pulmonary vein. The right middle and right inferior pulmonary vein were also connected anomalously in 88 (92%) and 17 (18%) patients, respectively. Anomalous connection of the right inferior pulmonary vein was more common in those with significant caudal extension of the defect (57% vs 15%, p=0.005). Among anomalously connected pulmonary veins, the right superior, middle, and inferior pulmonary veins were committed to the left atrium in 6, 17, and 11 patients, respectively. The superior caval vein over-rode the interatrial septum in 67 (70%) patients, with greater than 50% over-ride in 3 patients.
Conclusion Anomalous connection of the right-sided pulmonary veins is universal, but is not limited to the right upper lobe. Not all individuals have over-riding of superior caval vein. In a minority of patients, the defect has significant caudal extension, and anomalously connected pulmonary veins are committed to the left atrium. These findings have significant clinical and therapeutic implications.
- computed tomography angiography
- heart defects
- heart septal defects
Data availability statement
Data are available upon reasonable request.
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Contributors All of the authors contributed to one or more of the following: conception of design of the work (JR, SKG), data collection (JR, SKG, RR), data analysis and interpretation (JR, SKG, RR, GSG), drafting the manuscript (JR, SKG) and critical revision of the manuscript (all authors). All authors provided final approval of the manuscript. SKG and JR are responsible for the overall content as guarantors.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Disclaimer The views expressed in the submitted article by the authors are their own and not an official position of the institution.
Competing interests None declared.
Provenance and peer review Not commissioned; internally peer reviewed.
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