The Egyptian Heart Journal (2013) xxx, xxx–xxx

Egyptian Society of Cardiology

The Egyptian Heart Journal

www.elsevier.com/locate/ehjwww.sciencedirect.com

SHORT COMMUNICATION

Unusual presentation of a huge right coronary artery fistula;

Technical issues with device closure

Ahmed Mowafy a,*, Howaida El-Said a,b

a Critical Care Department, Kasr Al Aini, Cairo University, Egyptb University of California, San Diego, United States

Received 6 June 2013; accepted 11 July 2013

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KEYWORDS

Coronary fistula;

ASD device closure;

Right coronary artery

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Abstract Coronary artery fistula (CAF) is considered an embryologic persistence of primitive

intra-trabecular spaces which allow the developing coronary artery to communicate with the other

cardiac chambers or vascular structures. It is observed in 0.05–0.25% of coronary angiographic

studies, most of which drain into a right heart chamber or into the pulmonary artery, while a con-

genital right coronary artery (RCA) into a left heart chamber is less frequent.5 In this study, we

describe an unusual case treated by closure device in the right coronary artery fistula to the left ven-

tricle, and associated literature is reviewed. A 40-year-old female presented with chronic cough,

otherwise asymptomatic. Echocardiogram revealed unusual flow into the LV with mild LV dilata-

tion. A 64 multi-slice CT scan confirmed the presence of a huge right coronary opening with a fis-

tula into the left ventricle. The decision was to close this fistula with device through the RCA into

the left ventricle. The management of this unusually large fistula is described with focus on technical

issues with device closure.ª 2013 Production and hosting by Elsevier B.V. on behalf of Egyptian Society of Cardiology.

1. Introduction

Coronary artery fistula (CAF) is considered an embryologicpersistence of primitive intra-trabecular spaces which allowthe developing coronary artery to communicate with the other

cardiac chambers or vascular structures.1 It is observed in0.05–0.25% of coronary angiographic studies,2 most of which

01001588482.

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tian Society of Cardiology.

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owafy A, El-Said H Unusualpt Heart J (2013), http://dx.do

drain into a right heart chamber or into the pulmonary ar-tery,3,4 while a congenital right coronary artery (RCA) into a

left heart chamber is less frequent.5 In this study, we describean unusual case treated by closure device in the right coronaryartery fistula to the left ventricle, and associated literature is

reviewed.

2. Case report

A 40-year-old female presented with chronic cough, otherwiseasymptomatic. Transthoracic echocardiogram revealed a mildto moderately dilated aortic root, mild aortic regurgitation,

mild to moderate left ventricular dilation with unusual flowin the left ventricle. Trans-esophageal echocardiogram sug-gested a coronary fistula with flow entering the left ventricle just

gyptian Society of Cardiology.

presentation of a huge right coronary artery fistula; Technicali.org/10.1016/j.ehj.2013.07.003

Figure 1 3D reconstruction showing the dilated right coronary artery arising from the right coronary cusp then running a surpigenous

course until it enters the left ventricle just inferior to the atrio-ventricular ring. Note the origin of the posterior descending artery (PDA).

Figure 3 Angiogram revealing the dilated right coronary artery

entering the LV and delineating the take off of the posterior

descending coronary artery and its distance from the mushroom

shaped part of the fistula.

2 A. Mowafy, H. El-Said

below the posterior leaflet of the mitral valve. A 64 multi-sliceCT scan was performed that confirmed the diagnosis and delin-eated the anatomy of the fistula. The right coronary artery itself

was massively dilated measuring up to 25 mm in diameter, ittook a very tortuous course then communicated with the leftventricle just below the atrio-ventricular ring (Fig. 1).

3. Cardiac catheterization

The procedure was performed under general anesthesia. Ac-

cess was obtained via the right and left femoral arteries andthe right femoral vein. TEE was performed prior to commenc-ing the catheterization to access the relationship between theentrance of the fistula into the LV and the mitral valve. The fis-

tula entered into the posterior medial aspect of the LV near theinter-ventricular septum, just superior to the posterior-medialpapillary muscle, but relatively distant from the posterior leaf-

let of the mitral valve. There was a mild aortic regurgitationand a trivial mitral regurgitation. The LV appeared mildly di-lated with good ventricular systolic function. The RCA/fistula

was injected in multiple projections to better delineate theanatomy of the fistula and to determine the take off of majortributaries and branches particularly the posterior descending

coronary artery. A 7F balloon angiographic catheter (Arrowinternational, Reading, PA) was used so as not to traumatizethe fistula during catheter manipulation and during the injec-tions. The fistula formed a large C shape as it took off the

RCA and measured 25 mm in it is widest dimension, it thentapered to a point around 10 mm, then opened into a larger

Figure 2 (a) Angiogram in the lateral projection reveals the hug

representation of the RCA and fistula; note the two areas of narrowin

Please cite this article in press as: Mowafy A, El-Said H Unusualissues with device closure, The Egypt Heart J (2013), http://dx.do

mushroom shaped area measuring 19 mm and then narrowedagain to about 14 mm as it entered the LV (Fig. 2). Fig. 3 re-veals the posterior descending coronary artery well away from

the final mushroom part of the fistula. After precise delineationof the anatomy it was elected to close the fistula as far distallyas possible which would be in this mushroom shaped terminal

part. As the largest part of this area measured 19 mm, weelected to use a 22 mm ASD device, knowing that the left sideddisk of the device is 36 mm. The fistula was made from the aor-tic end using a 6F JR 3.5 100 cm catheter (Cook Inc., Bloom-

ington, IN) and a Wooley Wire (Covidien, Plymouth, MN).The Wooley wire made it through the fistula into the LV,

ely dilated right coronary artery entering the LV. (b) Pictorial

g the mushroom shape of it is terminal end.

presentation of a huge right coronary artery fistula; Technicali.org/10.1016/j.ehj.2013.07.003

Figure 4 (a) Cine in the lateral projection reveals the exchange wire entering the fistula from the aortic end and protruding into the LV.

A snare is seen entering the LV via the aorta. (b) Cine in the lateral projection reveals the exchange wire snared and being pulled out of the

aorta.

Figure 5 (a) Cine in the lateral projection reveals the long sheath with both the cable from the device and the buddy wire in place after

the device is released. (b) Cine in the lateral projection showing the buddy wire as it is being pulled from the aortic end next to the device.

Figure 6 Angiogram in the lateral projection with and without contrast reveals the 22 mm Amplatzer device well seated in the fistula;

note how the device is elongated to take the shape of the terminal part of the fistula.

Unusual presentation of a huge right coronary artery fistula; Technical issues with device closure 3

but the JR was too short to reach the LV. The Wooley wirewas removed and a .035 Teflon exchange wire (COOKMedical

Inc.) was advanced into the LV. A Snare catheter (B BraunInterventional systems, Bethlehem, PA) was introduced intothe ventricle via the left femoral artery, however it did not have

the correct angle needed to snare the wire, so the snare wasplaced in a JR catheter that was used to snare the wire, thisfacilitated the torque of the snare. The wire was exteriorized

Please cite this article in press as: Mowafy A, El-Said H Unusualissues with device closure, The Egypt Heart J (2013), http://dx.do

out of the left femoral artery sheath and a wire loop was cre-ated (Fig. 4). A 10F 90 cm Flexor sheath (Cook Medical

Inc.) was introduced over the wire from the LV side. Thesheath barely reached the fistula and was �7 mm short frombeing where we wanted to be. It was elected to leave the wire

in place so as not to loose access while introducing the devicenext to it (buddy wire technique) (Fig. 5a). The left sided diskof the device opened in the mushroom shape of the fistula, but

presentation of a huge right coronary artery fistula; Technicali.org/10.1016/j.ehj.2013.07.003

4 A. Mowafy, H. El-Said

the right sided disk opened mostly in the LV. This was felt tobe inadequate, while performing the TEE the device slippedinto the LV (still attached). The device was pulled back into

the sheath and the sheath was advanced further into the fistulaby applying more traction on the side of the wire coming out ofthe right femoral artery. That allowed the sheath to end in a

better position well within the mushroom part. The same de-vice was advanced into the mushroom part of the fistula, thistime all the left and most of the right sided disk was placed in-

side the fistula with only a small protrusion into the LV. TEEwas then performed to confirm that the device was not interfer-ing with the mitral valve posterior leaflet or apparatus. An at-tempt was made at removing the buddy wire while the device

was still attached, but this was not possible due to crowdingof the sheath with the device cable next to the buddy wire. Itwas elected to release the device and remove the device cable

prior to removing the wire. The wire was pulled out towardthe aorta, thus pulling the device more into the fistula than intothe LV (Fig. 5b). Angiogram confirmed the device to be in the

fistula with small amount of flow though the device (Fig. 6).Patient was maintained on low dose aspirin. At 6 month followup patient symptoms resolved, the shunt through the device

was trivial and LV size was significantly decreased.

4. Discussion

Coronary artery fistulas have been recognized since 1965.7 Theetiology of CAFs may be congenital, traumatic or iatrogenic,that is, after coronary intervention or valve replacement. Com-munication between a coronary artery and a cardiac chamber

is less frequent. The most prevalent receiving chamber is theright ventricle (45%), followed by the right atrium (25%)and the pulmonary artery (20%).8 However CAF between

the coronary artery and a left chamber is a rare condition.The clinical diagnosis of CAF is difficult because clinical pre-sentation, laboratory and electrocardiographic manifestations

are nonspecific. Most patients with CAF, as in the current pa-tient, present with typical angina pectoris without CAD. BothCAF and cardiomegaly can produce myocardial ischemia and

angina, and their association could aggravate the ischemia.The main mechanism of myocardial ischemia seems to be re-lated to the coronary steal phenomenon.9,10

CAFsmostly remain asymptomatic. The treatment of CAF is

essentially medical; conservative management with continuedfollow-up of these patients appears to be appropriate.11 Surgicalcorrection is exceptional and may be considered in only severe

forms with refractory to medical treatment. In the presence ofsymptoms of congestive heart failure, significant left-to-rightshunt and arrhythmias, other major cardiac lesions, concomi-

tant CAD, elective closure of coronary fistula are generally ac-cepted. Clinical symptoms of coronary ischemia, such asexertional angina or dyspnea are the primary indication for theclosure of a fistula.9 The first successful transcatheter coil embo-

lization of a coronary artery fistula was performed in 1982.7 Ourexperience shows that the transcatheter occlusion of coronaryfistula is a safe and effective procedure that is at least as success-

ful as surgical treatment. Nevertheless, the technique may not besuccessful in every case. It is essential to select the optimal embo-lization method in relation to the size and location of the fistula,

and to prepare a range of devices to cope with unexpectedrequirements and avoid multiple occlusion procedures.

Please cite this article in press as: Mowafy A, El-Said H Unusualissues with device closure, The Egypt Heart J (2013), http://dx.do

4.1. Technical issues

Coronary fistula are commonly tortuous, however because ofthe late presentation, the size of the patient and the unusuallylarge size of the fistula, it seemed like nothing we used was long

enough. It would have been easier if we had longer cathetersand sheaths available. Coronary fistulae should be closed asfar distally as possible to spare as many branches of the coro-nary artery as possible.6 The use of the buddy wire has been

reported in coronary artery intervention,12 however its use inthe scenario of coronary fistula to our knowledge has not beenreported . Using the buddy wire was a fall back when the de-

vice was inadequately placed the first time around and madereintroduction of the sheath straightforward. The buddy wirehowever made it cumbersome after the device was placed; as

it could not be removed until the device was released, whichcould have dislodged the device. For that reason the wirewas pulled out toward the aorta, rather than toward the LV

to avoid embolization of the device. Thrombus formation inthe fistula after closure has been reported,13 it is our impres-sion that this is caused by roughening of the intima of the cor-onary artery by excessive manipulation, for that reason

whenever possible one should avoid leaving wires exposed inthe fistula and avoid introducing the long sheath into the fis-tula, hence the snare technique to get access. One should al-

ways understand the anatomy of the fistula completelybefore attempting to enter or close it. In our case we studiedthe fistula with a 64 multi-slice CT prior to the catheterization

procedure, this helped clarify the anatomy on the outside,however the relationship of the fistula to vital structures forexample the mitral valve apparatus in our case could have onlybeen delineated with trans-esophageal echocardiography. To

achieve the best results one should use all available modalitiesto reach a clear understanding of the anatomy before embark-ing on a long and tedious procedure like this.

5. Conclusion

Coronary fistula may have very unusual presentations; height-

ened awareness facilitates diagnosis of these unusual cases. De-vice closure of huge coronary fistula is feasible and safe onlywhen all precautions including clear understanding of anatomy

and carefully thought through approaches are used.

References

1. Barbosa MM, Katina T, Oliveira HG, Neuenschwander FE, Oli-

veira EC. Doppler echocardiographic features of coronary artery

fistula: report of 8 cases. J Am Soc Echocardiogr 1999;12:149–54.

2. Kamineni R, Butman SM, Rockow JP, Zamora R. An unusual

case of an accessory coronary artery to pulmonary artery fistula:

successful closure with transcatheter coil embolization. J Interv

Cardiol 2004;17:59–63.

3. Fernandes ED, Kadivar H, Hallman GL, Reul GJ, Ott DA,

Cooley DA. Congenital malformations of the coronary arteries:

the Texas Heart Institute experience. Ann Thorac Surg

1992;54:732–40.

4. Said SA, Lam J, Van der, Werf T. Solitary coronary artery fistulas:

a congenital anomaly in children and adults. A contemporary re-

view. Congenit Heart Dis 2006;1:63–76.

5. Liu L, Wei X, Yan J, Pan T. Successful correction of congenital

giant right coronary artery aneurysm with fistula to left ventricle.

Interact Cardio Vasc Thorac Surg 2011;12:639–41.

presentation of a huge right coronary artery fistula; Technicali.org/10.1016/j.ehj.2013.07.003

Unusual presentation of a huge right coronary artery fistula; Technical issues with device closure 5

6. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB,

Lock JE. Management of coronary artery fistulae: patient selec-

tion and results of transcather closure. J Am Coll Cardiol

2002;39:1026–32.

7. Alekyan BG, Podzolkov VP, Cardenas CE. Transcatheter coil em-

bolization of coronary artery fistula. Asian Cardiovasc Thorac Ann

2002;10:47–52.

8. Kidawa M, Peruga JZ, Forys J, Krzeminska-Pakula M, Kasprzak

JD. Acute coronary syndrome or steal phenomenon: a case of

right coronary to right ventricle fistula. Kardiol Pol

2009;67:287–90.

9. Saglam H, Koco!ullari CU, Kaya E, Emmiler M. Congenital coro-

nary artery fistula as a cause of angina pectoris. Turk Kardiyol

Dern Ars 2008;36:552–4.

Please cite this article in press as: Mowafy A, El-Said H Unusualissues with device closure, The Egypt Heart J (2013), http://dx.do

10. Stierle U, Giannitsis E, Sheikhzadeh A, Potratz J. Myocardial

ischemia in generalized coronary artery-left ventricular microfistu-

lae. Int J Cardiol 1998;63:47–52.

11. Schleich JM, Rey C, Gewillig M, Bozio A. Spontaneous closure of

congenital coronary artery fistulas. Heart 2001;85:E6.

12. Cheena A. Buddy wire technique for stent placement at non aorto

osteal coronary lesions. Int J Cardiol 2007;118(3):e75–80, 12 June.

13. Gowda Srinath T, Laston Larry A, Kutty Shelby, et al. Interme-

diate to long term outcome following congenital artery fistulae

closure with focus on thrombus formation. Am J Cardiol

2011;107(2).

presentation of a huge right coronary artery fistula; Technicali.org/10.1016/j.ehj.2013.07.003