Multimodality Imaging of Double-Outlet Right Ventricle, Superior-Inferior Ventricles, and Malposed Great Arteries

The hallmark of superior-inferior ventricles is that the interventricular septum is horizontal to the diaphragm, with the ventricles lying one above each other. This is an unusual ventricular arrangement and can be associated with other defects, which makes it difficult to discern the intracardiac relationships.

A 2-kg premature baby received the diagnosis of double-outlet right ventricle, superior-inferior ventricles, a small subarterial and an additional large muscular ventricular septal defect (VSD), malposed great arteries, and coarctation of the aorta. A 3-dimensional (3D) printed cardiac model was completed to aid the decision-making process for single vs biventricular repair.

The 3D printed model demonstrated that the large muscular VSD was remotely located in relation to the malposed aorta. This would preclude placing an intracardiac baffle because of concern for inflow obstruction.

Multimodality imaging and 3D printing better delineated this complex combination of abnormal ventricular orientation, location of the VSD, and their relationship with the great arteries for surgical planning in a premature patient.