Utility of Multimodality Imaging in Superior Anomalous Pulmonary Venous Return With Inferior Sinus Venosus Defect

Inferior sinus venosus defects (SVD) are rare congenital malformations involving an overriding inferior vena cava resulting in an interatrial connection.

A female patient with multiple cryptogenic strokes was found to have a positive agitated saline study without evidence of atrial septal defect on transesophageal echocardiography. Cardiac computed tomography was used to diagnose partial anomalous pulmonary venous return (PAPVR) of the right superior and middle pulmonary veins associated with an inferior SVD. Three-dimensional printing was then used to aid in surgical planning.

Traditionally, an inferior SVD is associated with inferior PAPVR. This is a rare case of an inferior SVD with superior PAPVR that highlights the importance of multimodality imaging for accurate diagnosis and management.

Multimodality imaging is important in evaluating congenital heart disease, especially axial imaging such as cardiac computed tomography or cardiac magnetic resonance. Three-dimensional printed models can assist in presurgical planning to fully understand complex cardiac anatomy.