A 20-year-old male with LDS initially presented with a progressively enlarging ascending aortic aneurysm measuring up to 4.2 cm at the sinotubular junction. Multidisciplinary team decision was made to proceed with a surgical trans-sternal aortic root Bentall-type replacement with a 28 mm diameter graft. No immediate complications were observed. The 1-month follow-up cardiac CT demonstrated a new aortic outflow tract pseudoaneurysm with the sac measuring 20 × 17 mm in axial and 12 mm in craniocaudal dimensions, with a narrow neck measuring 3 mm. The pseudoaneurysm originated from the proximal anastomosis of the graft below the non-coronary cusp of the aortic valve and extended between the left and right atria (Fig. 1). Echocardiography demonstrated dynamic caliber change of the pseudoaneurysm, distending during systole, and shrinking during diastole. An interdisciplinary discussion between cardiac surgery, cardiology, interventional radiology took place. After consultation with the patient’s family, it was felt that due to the high risk of repeat surgery, the decision was made to manage the pseudoaneurysm conservatively with short interval imaging follow up. However, a subsequent CTA 2 months following surgery demonstrated interval increase in size of the pseudoaneurysm (Fig. 1), thus, endovascular embolization was recommended.
The procedure was performed under general anesthesia under normotensive conditions. Interventional cardiology and interventional radiology performed the procedure in a joint fashion. Through a 6-French sheath inserted in the right common femoral artery, a pigtail catheter was advanced into the left ventricle. Left ventriculogram demonstrated the aortic pseudoaneurysm with its neck arising just inferior to the aortic valve (Fig. 2a). Engaging the pseudoaneurysm neck required several attempts due to its proximity to the aortic valve. Stable position was eventually achieved using an IMA 6-French catheter (Performa®; Merit Medical, Salt Lake, UT, USA) with advancement of an exchange length 300 cm coronary wire (High-Torque Balance Middleweight; Abbott, Abbott Park, IL, USA) into the pseudoaneurysm, followed by advancement of a microcatheter (Velocity®; Penumbra, Alameda, CA, USA) into the sac neck. A total of 12 detachable microcoils (PC 400 Coil®; Penumbra, Alameda, CA, USA) were used for embolization of the pseudoaneurysm, beginning with a 22 mm diameter × 60 cm long Complex Standard “framing” coil, and subsequent filling of the pseudoaneurysm using a combination of standard and soft complex shaped coils ranging down to 9 mm in diameter. Post-embolization left ventriculogram demonstrated no residual filling of the aneurysm (Fig. 2c). Hemostasis at the femoral arterial puncture site was then achieved using a closure device (Angio-Seal®, Terumo, Shibuya City, Tokyo, Japan).
The patient was extubated and admitted to the cardiac intensive care unit for observation. No immediate post operative complications were observed. The patient was discharged after 2 days of clinical monitoring. Follow-up cardiac MRI 1 month as well as 1 year postoperatively demonstrated no residual or recurrent aneurysm (Fig. 3). Repeat echocardiography 4 years later did not show recurrence of the pseudoaneurysm.