Jpn J Radiol DOI 10.1007/s11604-014-0335-8

CASE REPORT

Coronary cameral fistula treated by the Amplatzer vascular plug Anurag Yadav • T. B. S. Buxi • Savitha Srirama Jayamma Kishan Singh Rawat • Samarjit Singh Ghuman • Neeraj Agarwal



Received: 16 January 2014 / Accepted: 12 May 2014 Ó Japan Radiological Society 2014

Abstract Coronary artery anomalies occur in less than 1 % of the general population. With the advent of multidetector rows and 3D reconstruction, multidetector computed tomography has emerged as the modality of choice in the delineation of the complex coronary anatomy and diagnosis of coronary artery anomalies, helping in the institution of appropriate therapy. We report a case of coronary cameral fistula of the left anterior descending artery to the right ventricle, which was closed by an Amplatzer vascular plug. Coronary CT angiography was used to evaluate the patient prior to the procedure to locate the placement site for the vascular plug. Keywords Coronary artery anomaly  Termination anomaly  Coronary cameral fistula  Amplatzer vascular plug

A. Yadav (&)  T. B. S. Buxi  S. S. Jayamma  K. S. Rawat  S. S. Ghuman Department of CT and MRI, Sir Ganga Ram Hospital, Old Rajinder Nagar, New Delhi 110060, India e-mail: [email protected] T. B. S. Buxi e-mail: [email protected] S. S. Jayamma e-mail: [email protected] K. S. Rawat e-mail: [email protected] S. S. Ghuman e-mail: [email protected] N. Agarwal Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, Old Rajinder Nagar, New Delhi 110060, India e-mail: [email protected]

Introduction Coronary artery anomalies are congenital abnormalities that are incidentally encountered in about 0.3–1 % of healthy individuals. It is reported that with conventional catheter angiography, these anomalies are accurately identified in only 53 % [1]. They are classified as anomalies of origin, course and termination. A high takeoff, multiple ostia, a single coronary artery and origin of the coronary artery from the noncoronary sinus are classified as anomalies of origin. Myocardial bridging and duplication of the arteries are anomalies of the course. Coronary artery fistula, coronary arcade and extracardiac termination are classified as anomalies of the termination [2]. Coronary artery anomalies are also classified as hemodynamically significant or insignificant. Anomalous origin of the coronary artery from the pulmonary artery, anomalous origin of the coronary artery from the noncoronary sinus with an interarterial course, deep myocardial bridging and coronary artery fistula are the hemodynamically significant anomalies [1]. The fistulous termination anomalies are more rare than those of the origin and course. The majority of the fistulae are congenital in origin, but may be seen after cardiac surgery, such as valve replacement and post-myocardial biopsies [3]. Coronary artery fistula (CAF) is a termination anomaly in which one or more coronary arteries will terminate into a cardiac chamber, artery or vein. The overall incidence is about 0.05–0.25 % based on catheter angiography [4]. When the fistula is between a coronary artery and the cardiac chamber, it is specifically described as coronary cameral fistula. CAFs are associated with possible complications such as steal from the adjacent myocardium, thrombosis, embolism, endocarditis, endarteritis, arrhythmias, cardiac failure and atrial fibrillation [5]. In this

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article, we report a case of coronary cameral fistula of the left anterior descending artery terminating into the right ventricle with multiple openings. Multidetector computed tomography (MDCT) is considered to be the modality of choice for the diagnosis of coronary anomalies, although echocardiography, catheter angiography and magnetic resonance imaging have also been used to evaluate coronary artery anomalies. MDCT helps in the delineation of the complex coronary anatomy and pre-therapeutic assessment of the fistulae [4].

Case report A 5-year-old male child presented with recurrent chest infections since early childhood and failure to thrive. General physical examination was normal. On auscultation, the child had a continuous murmur all over the precordium, which was loudest at the left lower parasternal area. Chest radiograph revealed cardiomegaly with prominent pulmonary vascular markings, which suggested a significant shunt. The patient was subjected to transthoracic echocardiography (iE33 X matrix, Philips, Best, The Netherlands), which revealed an abnormal communication between the left anterior descending artery and right ventricular apex (Fig. 1a, b). Coronary CT angiography was performed on 128-row multidetector CT scanner (Ingenuity, Philips); 40 ml of 350 mg I/ml non-ionic contrast injected through a dual-head Medrad pressure injector at a flow rate of 3.5 ml/ s, using 120 kV, 476 mAs, 0.8-mm slice thickness, pitch 0.2, rotation time 0.4 ms with ECG synchronization and retrospective gating with a heart rate of 92 beats per minute, revealed a fistula (Fig. 2), which measured 7.1 mm in size (Figs. 3a, 4a) in its shortest dimension, which had a distal saccular dilatation (34 mm), of the left anterior descending artery communicating with the right ventricular apex through multiple openings (Fig. 4c) suggestive of a coronary cameral fistula. The therapeutic plan to close the fistula near the left main coronary arterial end was instituted as the fistula had multiple openings at it termination. The narrowest part of the fistula was approximately 16.6 mm from the left main trunk (Fig. 3a). The fistula was hemodynamically significant as demonstrated by the chamber dilatation of the right ventricle and dilatation of the pulmonary artery (Fig. 3b). The advantage of MDCT was in clearly depicting the distance of the left main coronary artery to the origin of the fistula, which gave us the confidence to carry out an interventional therapeutic procedure safely without damaging the coronary arteries and to plan the placement of the location of the vascular plug. The findings were confirmed on digital catheter

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angiography (Allura Xper FD20, Philips) by injections in left main coronary artery. Selective injection was performed in the left anterior descending artery, and the fistula was delineated. Thereafter, a guidewire was passed through the femoral arterial route and crossed through the fistula into the right ventricular cavity. This wire was snared into the femoral vein, thereby creating an arteriovenous loop. Then the plug delivery system was deployed through the femoral vein across the fistula. Thereafter, an 8-mm Amplatzer vascular plug (AVP) II was deployed in the narrowest part of the fistula through this delivery system (Fig. 5a, b). The post-device catheter angiogram showed no residual shunt across the fistula (Fig. 5b). The follow-up transthoracic echocardiography showed the plug in situ and no residual shunt across the fistula (Fig. 1c). Post-procedural CT angiography confirmed the vascular plug in situ and patent LMT (Fig. 6). A schematic representation of the anomaly before and after the Amplatzer occluder device insertion is shown in Fig. 7a, b.

Discussion Coronary cameral fistula is a term specifically used for an anomaly of the coronary arteries in which fistulous communication occurs with any one of the cardiac chambers. The more proximal the origin of the fistulous tract from the main coronary artery is, the more dilated it tends to be. Coronary cameral fistulae involve the right heart structures more commonly than the left heart structures. The right coronary artery and left anterior descending artery are the most common vessels involved. The left circumflex artery is rarely involved. More than 90 % of the fistulae drain into the right ventricle (45 %), right atrium (25 %), pulmonary artery (15 %), coronary sinus and superior vena cava. In less than 10 % of the cases, the fistula drains into the left atrium and left ventricle [1]. The symptoms usually depend on the size of the fistula and on the left-to-right shunting [6]. The pressure difference between the coronary artery and the chamber into which the fistula is draining determines the shunting [3]. Traditional imaging techniques to diagnose such vascular anomalies include transthoracic echocardiography (TTE) and catheter angiography. Catheter angiography is invasive, and overlapping between a tortuous fistula and adjacent cardiovascular structures may hinder complete evaluation of these anomalies. The fistulae can have multiple feeding vessels, and drainage site can have a focal single opening or multiple openings. The fistula with multiple openings at the termination is a diagnostic as well as therapeutic challenge,

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Fig. 1 Transthoracic echocardiography parasternal long axis views showing the fistulous tract between the coronary artery and right ventricle (black arrow a, b) and post-procedural echocardiography

showing the Amplatzer vascular plug in situ (white arrow c). AO aorta. Asterisk left coronary artery

Fig. 2 Coronary CT angiography. Axial sections showing the normal course of the left main coronary artery arising from the left coronary sinus (asterisk in a) and large fistula from the left anterior descending artery terminating in the apex of the right ventricle through multiple

openings. AO aorta, LA left atrium, LAD left anterior descending artery, F (small) origin of the fistula, F saccular dilatation of the fistula, RV right ventricle

Fig. 3 Coronary CT angiography. Axial images showing the size of the narrowest segment of the fistula (7.1 mm) (double lines in a). The distance of the fistula from the left main trunk (16.6 mm) (arrow in a) and the dilated main pulmonary artery (32.2 mm) (b). AO aorta, F fistula, MPA main pulmonary artery

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Fig. 4 Coronary CT angiography. Coronal and oblique multiplanar reformatted images showing the narrowest segment of the fistula from the left anterior descending artery (arrow in a) and its termination into

the right ventricle through multiple openings (asterisk in c). AO aorta, F fistula, RV right ventricle

Fig. 5 Digital catheter angiography showing the catheter in the aortic root to demonstrate the coronary system and a fistula from the left main coronary opening into the right ventricle (a), arteriovenous loop and vascular plug in situ and simultaneous injection in the aortic root, demonstrating no residual shunt across the fistula (b)

which may be complicated by the presence of an intervening sinusoidal network. With the advent of coronary CT angiography, a relatively non-invasive technique for the imaging of coronary arteries, because of its high contrast and spatial resolution, has made the complete delineation of these anomalies possible. Therapeutic planning of placement of the vascular plug from the left main coronary arterial end was instituted as the termination had multiple points of drainage in our case. The early intervention in this patient prevented an untoward cardiac emergency such as rupture of the fistulous tract and the eventual death of the patient. The goal of the treatment is to occlude the fistula to preserve the normal coronary blood flow. Identification of the number of fistulous communications, nature of the feeding vessels, the myocardium at risk and the sites of drainage help to optimize the procedural options available. Surgical and transcatheter closures are the treatment options described in the literature. Internal closure of the fistula within the receiving chamber is the surgery

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Fig. 6 Post-procedural oblique multiplanar reformatted image showing the vascular plug in situ and patent LMT (arrowhead)

Jpn J Radiol Fig. 7 Schematic representation of the coronary cameral fistula (pre- and postprocedure). RV right ventricle, AO aorta, LMT left main trunk, LAD left anterior descending artery, F fistula, AVP Amplatzer vascular plug, asterisk multiple openings

performed. Complications of surgery such as myocardial infarction, arrhythmia, transient ischemic changes and stroke preclude its consideration as a treatment of choice nowadays [7]. Reidy et al. [8] described transcatheter closure of coronary arterial fistulae way back in 1983 using a detachable balloon. Since then, several studies have suggested that transcatheter closure of fistulae is feasible, with excellent outcomes and lesser morbidity and mortality. Detachable balloons, stainless steel coils and platinum microcoils are the embolization materials used. Detachable balloons are rarely used now as they are complex to use and require large introducer catheters [3]. Nowadays a number of occluder devices are available to close the fistulae. Commonly, the Amplatzer occluder device (AOD) is used to close these fistulae. The AOD requires a small sheath and is useful in larger fistulae, which have easy and straight access from the right heart. The AOD has several advantages such as the use of a single device (as compared to multiple coils), a high rate of complete occlusion, improved control over placement and release of the device in comparison with many devices used to close coronary arterial fistulae. In this report, we describe our experience of transcatheter closure of a coronary cameral fistula that had multiple openings at its termination in the right ventricle with AVP. Transcatheter closure can be safely done only after the anatomical relationships between the fistula and surrounding structures have been delineated completely, which is possible with coronary CT angiography. Transcatheter closure of these fistulae is now considered to be an effective and safe alternative to surgery [6]. Advantages of the transcatheter approach include less morbidity, lower cost, shorter recovery time, and avoidance of thoracotomy and cardiopulmonary bypass. Coronary artery anomalies need earlier detection to avoid untoward cardiac emergencies, particularly in young individuals.

Conclusion A coronary cameral fistula of the left anterior descending artery to right ventricle, which had multiple openings at its termination, was closed by an Amplatzer vascular plug. Coronary CT angiography was used to evaluate the patient prior to the procedure to locate the placement site of the vascular plug. We conclude that with the advent of MDCT, the complex coronary anatomy and its anomalies can be evaluated with confidence; thus, the institution of the appropriate treatment is possible as in our case. Conflict of interest

None.

References 1. Kim SY, Seo JB, Do KH, Heo JN, Lee JS, Song JW, et al. Coronary artery anomalies: classification and ECG-gated multidetector row CT findings with angiographic correlation. Radiographics. 2006;26:317–33. 2. Angelini P. Coronary artery anomalies; an entity in search of an identity. Circulation. 2007;115:1296–305. 3. Qureshi SA. Coronary arterial fistulas. Orphanet J Rare Dis. 2006;21(1):51. 4. Sundaram B, Kreml R, Patel S. Imaging of coronary artery anomalies. Radiol Clin North Am. 2010;48:711–27. 5. Padfield GJ. A case of coronary cameral fistula. Eur J Echocardiogr. 2009;10:718–20. 6. Khachatryan T, Karnwal S, Hamirani YS, Budoff MJ. Coronary arteriovenous malformation, as imaged with cardiac computed tomography angiography: a case series. J Radiol Case Rep. 2010;4:1–8. 7. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. patient selection and results of transcatheter closure. J Am Coll Cardiol. 2002;39:1026–32. 8. Reidy JF, Sowton E, Ross DN. Transcatheter occlusion of coronary to bronchial anastomosis by detachable balloon combined with coronary angioplasty at same procedure. Br Heart J. 1983;49:284–7.

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Coronary cameral fistula treated by the Amplatzer vascular plug.

Coronary artery anomalies occur in less than 1 % of the general population. With the advent of multidetector rows and 3D reconstruction, multidetector...
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