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Case report Transcatheter closure of coronary artery fistula using the new Amplatzer vascular plug and a telescoping catheter technique Antonella De Santis, Alberta Cifarelli and Roberto Violini Coronary artery fistulas (CAFs) are rare congenital or acquired anomalies characterized by an abnormal communication between the coronary arteries and the cardiac chambers or great vessels. Most patients are asymptomatic during childhood but symptoms and complications have been reported with advancing age. Until recently, surgery was the routine mode of treatment for CAFs but, today transcatheter closure is recommended using a variety of devices, such as occlusion coils, vascular plugs, umbrella devices and covered stents. The case described here is of a 47-year-old woman with a large bilateral CAF draining into the pulmonary artery, successfully treated by implantation of two Amplatzer vascular plugs using a telescoping catheter technique. Introduction Coronary artery fistulas (CAFs) are rare congenital or acquired anomalies characterized by an abnormal communication between the coronary arteries and the cardiac chambers or great vessels. The majority of CAFs are congenital but may occasionally be detected following cardiac trauma or iatrogenic factors (cardiac surgery, coronary angioplasty, therapeutic chest irradiation, etc.) [1–3]. The estimated incidence in selected patients, submitted to coronary angiography, ranges from 0.3 to 0.8% [4]. Most fistulas arise from the right coronary artery (60%) and terminate in the right side of the heart (90%). Most patients are usually asymptomatic during childhood but, in older patients, late symptoms and complications, such as myocardial ischemia, pulmonary arterial hypertension, cardiac heart failure, arrhythmias, endocarditis and rupture with cardiac tamponade, have been reported [4–7]. Symptoms, complications and a significant shunt are the main indications for the treatment of CAFs [1]. Since transcatheter occlusion of fistulas was first reported in the 1980s [8,9], it has been considered an alternative to surgery, with excellent results and acceptable complication rates. Various closure devices are available but coil occlusion of the fistula is currently the most commonly used technique [10–12]. The Amplatzer vascular plug (AVP) (AGA Medical Corp., Golden Valley, Minnesota, USA) is a new occlusion device made of self-expanding cylindrical nitinol wire mesh secured at both ends with platinum marker bands. A stainless steel delivery cable, 1558-2027 ß 2010 Italian Federation of Cardiology J Cardiovasc Med 11:605–609 Q 2010 Italian Federation of Cardiology. Journal of Cardiovascular Medicine 2010, 11:605–609 Keywords: coronary fistula, transcatheter closure, vascular plug Interventional Cardiology, Department of Cardiovascular Medicine, S. Camillo Forlanini Hospital, Rome, Italy Correspondence to Dr Roberto Violini, Interventional Cardiology, Department of Cardiovascular Medicine, Azienda Ospedaliera San Camillo Forlanini, Circonvallazione Gianicolense n 87, CAP 00152 Rome, Italy Tel: +39 06 58704478; fax: +39 06 58704478; e-mail: [email protected] Received 26 April 2009 Revised 9 July 2009 Accepted 26 July 2009 135 cm in length, is attached to the microscrew located on one of the marker bands. The vascular plug is preloaded, attached to the delivery cable (Fig. 1). The AVP is available in various sizes, ranging from 4 to 16 mm in diameter (in 2 mm increments) and from 7 to 8 mm in length. The selected vascular plug should be 30–50% larger than the diameter of the target vessel. For delivery, a 5–8 French (F) standard coronary guide catheter can be used; the recommended internal diameter being from 0.056 to 0.088 inches. Until its delivery, the plug can be repositioned or removed if the position is not satisfactory. The present report refers to a case of bilateral fistula submitted to treatment with the new AVP and a complex delivery technique. Case report A 47-year-old woman was referred to our institute on account of a history of chest pain and dyspnea upon moderate exertion. At physical examination, a continuous murmur could be heard over the precordium at the second left intercostal space. Transthoracic color Doppler echocardiogram revealed a continuous flow in the main pulmonary artery, in the high parasternal short axis view. Contrast-enhanced electron-beam tomography disclosed the presence of a tortuous and enlarged vascular malformation emerging from the left anterior descending (LAD) artery to the pulmonary artery. A selective coronary angiography was, therefore, performed using 6F right Judkins and left Judkins catheters (Cordis Corporation, Miami, Florida, USA), which DOI:10.2459/JCM.0b013e3283313504 Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited. 606 Journal of Cardiovascular Medicine 2010, Vol 11 No 8 Fig. 1 Fig. 2 The AVP is made from 144 nitinol mesh. AVPs range in size from 4 to 16 mm in diameter in 2 mm increments and is 7–8 mm in length. The vascular plug is selected to be 30–50% larger than the target vessel diameter for secure delivery and occlusion. demonstrated a large bilateral coronary artery fistula draining into the pulmonary artery (Fig. 2a, b). The right fistula, arising alongside the ostium of the right coronary artery, had a large diameter and was located in front of the right ventricular ejection tract, towards the left section of the fistula, and was divided into two branches (superior and inferior) before draining into the pulmonary artery. The left fistula, uniformly dilated, arose from the middle portion of the LAD artery and, after a very tortuous crossing, drained into the main pulmonary artery. The proximal part of the LAD appeared dilated whereas the distal part showed normal dimensions. The right and left fistulas had a maximal diameter of 8 and 5 mm, respectively, with the narrowest diameters (proximal to the entrance into the pulmonary artery) of 5 and 4 mm, respectively. Coronary angiography did not show any evidence of atherosclerotic stenosis in the coronary arteries and no additional cardiac diseases were found. Left to right shunting, assessed by measuring the pulmonary to systemic flow ratio (QP : QS) was found to be 1.2. Given the referring symptoms and in order to prevent later complications, it was decided to attempt percutaneous closure of the CAF, as reported in the literature [1,13]. Closure of the CAF was performed in two steps. In the first, closure of the right part of the fistula was attempted following cannulation of the anomalous collector from the right femoral artery with a 7F Amplatz (AL) II guiding catheter (Launcher, Medtronic, Inc., Minneapolis, Minnesota, USA); the AVP (diameter 8 mm, length 6 mm) was advanced to the proximal side of the superior branch; Selective coronary angiography of a right (a) and left (b) coronary artery fistula draining into the main pulmonary artery. at the same time, dissection, with spontaneous occlusion of the inferior branch of the fistula, was observed. Angiography, performed 15 min later, confirmed the correct position of the device and occlusion of both branches of the fistula; the AVP was then released from the delivery cable (Fig. 3). During the delivery of the device, a transient elevation of the ST segment was observed in the inferior leads with angina and angiographic evidence of slow-flow in the right coronary artery, probably due to air embolization that resolved spontaneously. During the procedure the patient received intravenous (i.v.) heparin, achieving an activated clotting time of more than 250 s. After delivery of the device, aspirin (250 mg i.v.) was Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited. Transcatheter coronary artery fistula closure De Santis et al. 607 Fig. 3 Released AVP allows complete occlusion of the right CAF. administered. Total fluoroscopy time was 21 min. No postprocedural complications were observed and the patient was discharged 2 days later after a chest radiograph, standard ECG and Doppler echocardiographic study. Therapy with aspirin was prescribed to prevent eventual thromboembolic events. Closure of the left CAF was performed 2 months later, at the request of the patient. Because of the origin of the left CAF from the middle portion of the LAD artery and in order to avoid a deep intubation of the artery, necessary for a correct delivery of the AVP, we decided to use a microcoil Micrusphere Platinum (Micrus Endovascular, Sant Jose, California, USA) as a first choice. From the right femoral artery, the LAD was cannulated with a 8F AL II guiding catheter (Launcher, Medtronic, Inc.) and a guide wire (BMW, Abbott Vascular, Santa Clara, California, USA) was positioned within the anomalous collector. A microcatheter VASCO 2.1 F (AB Medica, Balt Extrusion INC, Montmorency, France) was advanced over the guide wire to the fistula and the guide wire was then removed; thereafter a microcoil Micrusphere Platinum 10 17.1 mm (Micrus Endovascular) was introduced to the narrowest segment of the fistula. Due to the large dimensions of the fistula and the high flow, stabilization of the coil was impossible. The guide wire was, therefore, reintroduced into the fistula, a second microcatheter VASCO 2.1 F (AB Medica, Balt Extrusion Inc.) was advanced parallel to the first one and a second microcoil Micrusphere Platinum 8 13.6 mm (Micrus Endovascular) was introduced in an attempt to occlude the malformation with the two coils simultaneously. The procedure was complicated by the migration into the pulmonary artery of one of the two coils (microcoil 10 17.1 mm), which was recovered using a 10.5 15 mm snare catheter advanced from the femoral vein to the pulmonary artery. An alternative method, a ‘telescoping catheter technique’, was then employed. The left coronary ostium was cannulated with an 8F left Judkins guiding catheter (Cordis Corporation), a guide wire (Wispher, Abbott Vascular) and the microcatheter VASCO were positioned distally into the fistula. A 5F right Judkins 3.5 guiding catheter was advanced in the 8F guiding catheter to the proximal part of the fistula (in the middle portion of the LAD) over the microcatheter, to maintain a more central position in the lumen and to avoid coronary dissections. The telescoping catheter technique was possible by prolonging the 5F guiding catheter, cutting the proximal hub and inserting a 5F introducer. The VASCO microcatheter and the guide wire were removed and an AVP (diameter 8 mm, length 6 mm) was then advanced in the 5F-guiding catheter into the fistula to the desired site of occlusion. Some minutes later, once the device was in the correct position, with almost complete occlusion of the fistula and absence of any obstruction in the LAD had been confirmed, the AVP was released (Fig. 4a). The final angiography confirmed an optimal result with TIMI grade III coronary flow (Fig. 4b). Fluoroscopy time was 40 min. This reflects the potential difficulty of these complex procedures. As is practice, during the procedure, heparin and aspirin were administered i.v. No postprocedural complications were observed. Five days later, a coronary angiography confirmed that no major side branches had been negatively affected by the plug which was in the correct position. A very small residual shunt was observed in the left artery fistula whereas a new, small, right artery fistula was demonstrated. The patient received aspirin for 6 months after the procedure. Discussion Fistulas are rare congenital or acquired, sometimes iatrogenic, anomalies of coronary arteries. The presence of symptoms, complications and a significant shunt are the main indications for coronary artery fistula closure. The first pathological account of CAF was published, in 1908, by Maude Abbott and the first successful surgical closure of a CAF was performed, in 1947, by Bjork and Crafoord, in a patient with a preoperative diagnosis of patent ductus arteriosus [14]. Since then, surgery has been the technique of choice in the treatment of CAF [15–17]; however, today, technical progress in interventional cardiology has allowed transcatheter closure in selected patients, with good success rates and an acceptable procedure-related morbidity. Various devices including Gianturco coils (Cook, Inc., Bloomington, Indiana, USA), Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited. 608 Journal of Cardiovascular Medicine 2010, Vol 11 No 8 Fig. 4 Closure of the left CAF by the ‘telescoping catheter technique’ (a). A 5F right Judkins 3.5 guiding catheter (arrow A) advanced in an 8F guiding catheter (arrow B) to the middle part of the LAD, and AVP (arrow C) advanced in a 5F guiding catheter into the fistula to the preferred site of occlusion. AVP positioned in the right CAF (arrow D). Once the correct position of the device and the absence of any obstruction in the LAD were confirmed, the AVP was released, resulting in occlusion of the CAF and maintenance of TIMI III coronary flow (b). Grifka Vascular Occlusion Device (Cook, Inc.), and Amplatzer Occlusion Devices (AGA Medical Corp.) have been described in the treatment of arteriovenous malformations. In a few studies, the use of AVP in the closure of CAF has been investigated, all of which confirm that it could be regarded as a well-tolerated and effective technique for this purpose [18–20]. The AVP is easy and fast to use and can be repositioned safely before final delivery if the placement is not satisfactory. In comparison, occlusion using coils sometimes requires complex manipula- tion of catheters, as well as the selection of various catheters and wires. The AVP is especially useful for embolization of short, high-flow vessels such as large arteriovenous malformations or large coronary fistulas where multiple coils may be needed to occlude the malformation and coil migration and catheter dislodgement might occur [12]. The AVP does not cause immediate thrombosis and in high-flow situations complete occlusion is usually obtained within 15 min [21]. Furthermore, the plug can be delivered easily through a small, 5F or 6F, guiding catheter. Our experience has further confirmed the efficacy and safety of AVP in the treatment of CAF. In our case, AVP was used in a simpler procedure (right CAF closure), where the fistula was proximal and the AVP was easily delivered, and in a very difficult procedure (left CAF closure) in a large, tortuous and distal fistula in which multiple coils would be needed to occlude the malformation, but it was impossible to stabilize these on account of the high flow. The AVP is more difficult to implant in distal fistulas as a stiff delivery catheter must be advanced distally in the vessel to the location where the AVP must be delivered. In our case, we found that it may be preferable to use two coronary guiding catheters like a ‘telescope’, first cannulating the coronary ostium with a large 8F catheter, then introducing and advancing inside it a smaller 5F guiding catheter as the delivery catheter. This technique, which, to our knowledge, has only once been reported previously in a similar case of coronary fistula occlusion [12], may be more traumatic but has the advantage of allowing welltolerated release of the device. However, to minimize the risks of coronary dissection, we advanced the smaller guiding catheter over a guide wire and a microcatheter in order to maintain a more central position in the lumen. Results of the procedure were excellent and no complications occurred. In conclusion, the AVP seems to be operator-friendly, simple to use in tortuous vessels, employing a coronary guiding catheter in the proximal fistulas and a telescoping technique in the more distal fistulas, thus obtaining complete occlusion of the CAF. Given the variability of anatomy seen in CAFs, the need to apply different techniques for device delivery, as well as the ability to manage various complications, suggest that no single transcatheter method is likely to be successful in all cases and flexibility in the interventional approach is important. Acknowledgements All figures were produced with the help of the radiology technicians. The authors are particularly grateful to Miss Ilaria Scancella. References 1 Balanescu S, Sangiorgi G, Castelvecchio S, Medda M, Inglese L. Coronary artery fistulas: clinical consequences and methods of closure. A literature review. Ital Heart J 2001; 2:669–676. Copyright © Italian Federation of Cardiology. 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