The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.