Rapid Prototyping and Image Fusion Guidance for Transcatheter Closure of Superior Sinus Venosus Atrial Septal Defect

Abstract

Sinus venosus atrial septal defect (SVASD) accounts for 5–10% of all ASDs. Partial anomalous pulmonary venous connection (PAPVC) and pulmonary hypertension (PH) in patients with SVASD increases the risk for surgical or transcatheter intervention. Closure is preferred over medical therapy. Transcatheter closure is challenging due to complex anatomy, difficulty in evaluating suitability for intervention, and lack of dedicated devices. We present a systematic approach using multimodality imaging and 3D printing for transcatheter closure of superior SVASD with PAPVC using a custom-made CP Stent®. A 53-year-old with history of drug abuse presented with NYHA Class III symptoms, hypoxemia, atrial flutter, and PH (MPAP of 30 mmHg) requiring multidrug therapy. Echocardiogram raised suspicion of superior SVASD, confirmed by cardiac CT and CMR. Multidisciplinary team ruled out surgery due to high risk and recommended transcatheter closure, if feasible. CT and CMR demonstrated a 20 × 18 mm superior SVASD with PAPVC: right upper (RUPV) and middle pulmonary veins draining into the superior vena cava (SVC). To understand spatial relationships, a 3D model was printed with 1.5-mm transparent and flexible walls to deploy a non-sterile prototype of the stent in the SVC. Rotational angiogram was performed to test feasibility of in-vivo deployment in view of stent foreshortening (from 110 to 70 mm) and differential widening of the SVC: 23 mm at the junction of the innominate vein; 35 mm at the atrial end. Transcatheter closure of superior SVASD with PAPVC in patients with PH is feasible in suitable cases using a systematic approach with multi-modality imaging and 3D printing.