Paravalvular Mitral Regurgitation Treated with an Amplatzer Septal Occluder Device: A Case Report and Review of the Literature

Case Report The patient is a 62-year-old male with a past medical history significant for hypertension, hyperlipidemia and coronary artery disease. In October 2004, he developed congestive heart failure and was diagnosed with severe mitral regurgitation secondary to bacterial endocarditis from probable Q-fever. He underwent mitral valve replacement with a bioprosthetic mitral valve (27 mm Carpentier-Edwards Perimount Plus Mitral Bioprosthesis, Edwards Lifesciences, Irvine, California) and single-vessel coronary artery bypass grafting with a reverse saphenous vein graft to the left circumflex artery. Blood cultures remained negative for endocarditis. However, Q-fever titers were elevated, and he was treated for presumed Q-fever endocarditis with intravenous ceftriaxone and oral daptomycin for 6 weeks. In June 2005, the patient presented with recurrent congestive heart failure from severe mitral regurgitation thought to be secondary to valve dehiscence. Saphenous vein bypass graft angiography showed an occluded graft to the left circumflex artery, and angioplasty and stent placement (3.0 x 24 mm Cypher™, Cordis Corp., Miami, Florida) were performed, with reestablishment of normal distal flow. He then underwent repeat mitral valve replacement with another bioprosthetic valve (27 mm Carpentier-Edwards Perimount Plus Mitral Bioprosthesis). Postoperatively, he required a dual-chamber pacemaker for symptomatic bradycardia. The patient next presented in July 2005 with recurrent congestive heart failure. Transesophageal echocardiography (TEE) showed severe mitral regurgitation with a 6 mm paravalvular defect adjacent to the mitral valve sewing ring on the lateral aspect of the ventricle near the left atrial appendage. He was refused surgery by two cardiac surgeons and was transferred to our institution for consideration of percutaneous treatment options. Consent for emergency treatment using an investigational device was obtained from the Institutional Review Board committee at our center, and written informed consent for the procedure was obtained from the patient. Prior to his arrival to the cardiac catheterization laboratory, he developed respiratory distress and was electively intubated. A 5 Fr sheath was placed in the right femoral artery and a 6 Fr sheath in the right femoral vein. He subsequently became hemodynamically unstable, with a decrease in systolic blood pressure to 70 mmHg. A 34 cc intra-aortic balloon pump (Datascope Corp., Montvale, New Jersey) was placed in the left femoral artery, and counterpulsation at 1:1 was initiated. The 6 Fr venous sheath was exchanged for an 8 Fr transseptal sheath (Medtronic Inc., Minneapolis, Minnesota). Under fluoroscopic and TEE guidance (Figure 1), the atrial septum was punctured using a Brockenbrough needle (St. Jude Medical, Inc., Minnetonka, Minnesota), and the transseptal sheath was advanced into the left atrium. Using TEE guidance, a 0.035 inch Magic Torque wire (Boston Scientific Corp., Natick, Massachusetts) was placed across the paravalvular defect from the left atrium into the left ventricle. A 4 Fr 120 cm length Glide Catheter (Boston Scientific) was advanced over the Magic Torque wire into the left ventricle. The Magic Torque wire was removed and a 0.035 inch J-tipped Amplatzer wire (AGA Medical, Golden Valley, Minnesota) was advanced through the Glide catheter into the left ventricle. The Glide catheter and transseptal sheath were removed, and a 25 mm sizing balloon (NMT Medical, Inc., Boston, Massachusetts) was placed across the defect. Balloon stretching showed a long canal in the lateral aspect of the paravalvular defect, with a maximum diameter of 9.5 mm (Figure 2). A 7 Fr Amplatzer delivery sheath (AGA Medical) was advanced across the defect into the left ventricle. A 12 mm Amplatzer septal occluder device was then advanced and positioned into the left ventricle. By TEE guidance, it appeared that the device was in adequate position within the defect and was released. Within several cardiac beats, the device embolized into the left ventricle (Figure 3), and after several minutes embolized to the abdominal aorta. The patient remained hemodynamically stable, and the device was retrieved using a 10 mm x 120 cm Snare catheter (ev3, Inc., Plymouth, Minnesota). A 14 mm Amplatzer septal occluder device was then advanced into the left ventricle through the 7 Fr Amplatzer delivery sheath. On this second attempt, a larger portion of the device was positioned on the left atrial side under TEE guidance. Good device stability was achieved, and the device was released (Figure 4). TEE imaging showed a significant reduction in mitral regurgitation to 1+, and the patient’s hemodynamics immediately improved, with an increase in systolic blood pressure to 120 mmHg. The intra-aortic balloon pump was removed several hours later in the coronary care unit. Over the ensuring 48 hours, intravenous diuretics were administered, and the holosystolic murmur of mitral regurgitation decreased substantially. The patient was extubated on the second hospital day. A follow-up transthoracic echocardiogram showed preserved left ventricular systolic function and normal prosthetic valve hemodynamics. The Amplatzer septal occluder device remained in good position in the lateral periprosthetic opening with a trivial residual leak (Figure 5). Discussion The initial report describing percutaneous closure of paravalvular leaks is credited to Hourihan et al in 1992.¹ In this study, the authors presented 8 patients, including 4 with paravalvular leaks, who were deemed to be poor surgical candidates. Three patients underwent successful placement of a Double-Umbrella Rashkind device for paravalvular aortic leaks, achieving either partial or complete closure of the leak and a subsequent improvement in clinical status. Since this initial report, a variety of devices have been used for the percutaneous treatment of paravalvular leaks^2–8 (Table 1). Use of the Gianturco-Grifka vascular occlusion device was described by Eisenhower et al in 2001, and is thought to be ideally suited for this indication.⁴ The ability of the Dacron bag to conform to the contours of the paravalvular defect is an attractive feature of the device. However, the lack of actual attachment of the device to the adjacent tissue raises a concern for possible device embolization. Coils have been used for the treatment of paravalvular leaks by several investigators including Moore et al, Pate et al, Moscucci et al and Boudjemline et al.^2,5,6,8 Although coils are widely available and have been used for occlusion of a variety of vascular structures, their use in larger paravalvular defects carries a significant risk of embolization into the systemic circulation. Among newer-generation devices, Kort et al used the Amplatzer ductal occluder device to treat a perivalvular leak in a small child with complete atrioventricular canal and periprosthetic mitral regurgitation.³ Despite successful implantation of the ductal occluder device in this case, the large retention skirts carry the risk of possible interference with function of the prosthetic valve leaflets. The Amplatzer septal occluder device was originally developed for the percutaneous treatment of atrial septal defects.⁹ However, the versatility of this device has led to several novel applications including the closure of Blalock-Taussig shunts,¹⁰ ventricular-to-atrial fistulae,¹¹ ventricular septal defects,^12,13 large patent ductus arteriosus¹⁴ and atrial septal defects following percutaneous mitral balloon valvuloplasty.¹⁵ To our knowledge, the use of the Amplatzer septal occluder device for the percutaneous closure of a mitral paravalvular leak has only been reported twice previously.^2,8 In 2002, Boudjemline et al used this device in combination with coils to treat a 9-year-old child with a complete atrioventricular canal that developed paravalvular mitral regurgitation and severe hemolysis associated with a mechanical mitral prosthesis.² Following the initial placement of a 6 mm Amplatzer septal occluder device and coils, there was continued paravalvular regurgitation, prompting placement of an 8 mm Amplatzer septal occluder device, with subsequent closure of the defect. In 2004, Pate et al used the Amplatzer occluder device in combination with coils to treat a mitral paravalvular leak in a 75-year-old male who had previously undergone unsuccessful mitral valve repair followed by mitral valve replacement.⁸ The ideal closure device and approach (retrograde from left ventricle-to-left atrium versus transseptal) for mitral paravalvular leaks remains unclear.¹⁶ Each of the commercially available devices has certain advantages and disadvantages. The recently-developed Amplatzer vascular plug is another potential option, and offers the choice of multiple sizes (4–16 mm in 2 mm increments), easy deployment and the ability to remove the device prior to release if the position is not optimal. The conformability of the Amplatzer vascular plug is another useful feature, but as yet, this device has not been used for this indication. In 2003, Piechaud provided an overview for the percutaneous closure of mitral paravalvular leaks.¹⁷ The retrograde approach for these defects may be optimal, as it provides advancement of the wire in same direction as the regurgitant jet. While a transseptal approach may also be used, it commits wire passage to be in the opposite direction of the blood flow across the defect and can therefore be challenging. The optimal approach using an Amplatzer device should also include consideration of where the larger portion of the device (i.e., the left atrial disc) would be best situated and less likely to interfere with prosthetic valve function. In summary, we describe a case of successful transcatheter closure of a moderate-sized paravalvular leak with the Amplatzer septal occluder device in a patient with excessive surgical risk. We were able to successfully close the paravalvular leak without interfering with normal prosthetic valve leaflet function. In patients with severe periprosthetic mitral regurgitation refractory to aggressive medical therapy who are not candidates for surgical intervention, use of the Amplatzer septal occluder device offers an attractive percutaneous treatment option.

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