3-Dimensional Modeling Guided Transcatheter Repair of Dehisced Pulmonary Venous Baffle With Gore ASD Device

A 38-year-old woman with sinus venosus atrial septal defect and partial anomalous return of the right upper pulmonary vein underwent a Warden procedure but experienced a large residual defect after patch dehiscence. Image-derived 3D modeling informed novel device closure with a Gore Cardioform atrial septal occluder. (Level of Difficulty: Advanced.)

A 38-year-old woman presented with right heart dilation and a residual sinus venosus atrial septal defect (ASD) 1 year after undergoing a Warden procedure for sinus venosus ASD and partial anomalous return of the right upper pulmonary vein (RUPV) to the superior vena cava (SVC).Cardiac magnetic resonance (CMR) demon-strated dehiscence of the patch used to baffle the RUPV flow and a Qp:Qs of 2:1.

MEDICAL HISTORY
The patient had received a diagnosis of sinus venosus ASD and partial anomalous pulmonary venous return of the RUPV to the SVC at the level of the right pulmonary artery (RPA) during evaluation for palpitations.She underwent a Warden procedure with transection of the SVC above the anomalous RUPV, baffling of the pulmonary venous confluence through the sinus venosus ASD to the left atrium, and reanastomosis of the SVC to the right atrial appendage.

LEARNING OBJECTIVES
To better visualize complex defect created by patch dehiscence though multimodality imaging in conjunction with 3D modeling.To highlight the novel use of an ASD septal occluder for a dehisced baffle while avoiding surgical repair.Notably, unlike previously reported methods of redirecting pulmonary venous flow using covered stents, a septal occlusion device requires rims of tissue bordering at least some of the defect to provide a secure anchor for the device retention discs.
Circumferential visualization of these rims using CMR-derived modeling was particularly beneficial to the conception and design of this procedure.

FOLLOW-UP
Transthoracic echocardiography 1 day after the intervention demonstrated no residual leak around the device and normal right ventricular systolic shortening.The patient was discharged 1 day after the procedure and has not reported complications to date.

CONCLUSIONS
In summary, procedure-specific 3D modeling using after the Warden procedure, CMR demonstrated that the RUPV and right middle pulmonary vein (RMPV) appropriately returned to the SVC stump, but the autologous pericardial patch used to baffle the pulmonary venous drainage to the left atrium had completely dehisced, with a large residual sinus venous ASD (Figure 1, Video 1).The Qp:Qs by CMR was approximated to be 2:1, with associated moderate dilation of the right ventricle but preserved right ventricle ejection fraction.Given the complexity of the structural defect, further preprocedural modeling was performed.The CMR was segmented using the segment editor in the 3D slicer (www.slicer.org)(Figure 2, Video 2). 1 The segmented model demonstrated a large defect (2.4 Â 2.1 cm) in the floor of the SVC stump, allowing RUPV and RMPV flow to drain into the right atrium, as well as rims on the anterior and lateral aspects of the defect.Device closure was conceptualized based on this modeling and then simulated using the cardiac device simulator module in SlicerHeart (Figure 3, Video 3). 1,2MANAGEMENT After discussion of the risks and benefits of various treatment options, the patient elected to attempt catheter-based intervention as opposed to surgical recreation of the baffle.Procedural transesophageal echocardiography (TEE) and angiography confirmed findings of a large sinus venosus ASD at the floor of the SVC/pulmonary vein stump with left-to-right flow (Figures 4 and 5).Real-time 3D TEE was used almost exclusively for guidance of device placement because of familiarity with the 3D anatomy of the patient.Through a 14-F sheath in the right atrium, a 44-mm Gore Cardioform ASD occluder (Gore Medical) was advanced across the defect and deployed with the "left atrial" disc within the pulmonary venous baffle and the "right atrial" disc within the right atrium across the native inflow of the SVC, thereby reestablishing the floor of the baffle.3D TEE demonstrated stable device position with minimal residual flow around the device and no obstruction of the right pulmonary vein inflow to the left atrium (Figure 6, Video 4).

FIGURE 2
FIGURE 2 CMR-Based Modeling to Inform 3D Echocardiographic Views CMR was successfully used to visualize postsurgical anatomy and to plan a transcatheter closure of a sinus venosus defect using an atrial occlusion device.Patient-specific, image-derived modeling may be informative to the design of unique transcatheter interventions, particularly for atypical and unfamiliar surgically altered anatomy.

FIGURE 3 FIGURE 5
FIGURE 3 Comparison of Device Simulation With Procedural Fluoroscopy Imaging