New anatomical classification to plan transcatheter correction of sinus venosus defect based on 3D models.

BACKGROUND

Superior sinus venosus defect (SVD) is a complex congenital heart disease (CHD) with a wide spectrum of anatomical variants. The innovative transcatheter correction of SVD (TCSVD) is feasible in selected cases. Patient selection requires a detailed anatomical evaluation. This study aims to provide an anatomical classification of SVD using 3D models.

METHODS

Computed tomography (CT) scans of hearts with superior SVD were 3D-modeled using semi-automatic segmentation. Key parameters such as superior vena cava (SVC) size, SVC overriding, caudal defect extension, and size/orientation of the anomalous pulmonary vein (APV) were assessed in this single-center cohort study.

RESULTS

We included 197 patients with superior SVD. SVC overriding was absent in 38 ​% of cases and exceeded 50 ​% in 7 ​% of cases. A single APV ostium was identified in 52 ​% of patients and additional ostia in 48 ​% of patients. Among children older than 12 years, 83 ​% had an SVC diameter greater than 14 ​mm (at the first quartile in adults). Based on the amount of SVC overriding and caudal extension of the defect, we classified the SVDs into two types: fenestration (30 ​%) and cavo-atrial (70 ​%). Associated lesions included left superior vena cava (15 ​%) and ostium secundum atrial septal defect (8 ​%).

CONCLUSION

A standardized 3D evaluation of SVDs provided a new anatomical classification that should prove useful for tailoring TCSVD strategies.