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Images in cardiovascular medicine

Unrecognized anomalous left circumflex coronary artery arising from right sinus of Valsalva: a source of perioperative complication Mariano Pellicano, Gabor Toth, Giuseppe Di Gioia, Dan Rusinaru, William Wijns, Emanuele Barbato, Bernard De Bruyne, Ivan Degrieck and Carlos Van Mieghem In this article we provide a very interesting and challenging PCI of unrecognized anomalous left circumflex coronary artery (LCx) arising from right sinus of Valsalva (RSV) after aortic valve replacement (AVR). This case presentation focuses the attention on important criteria for recognition of abnormal LCx coronary artery, that is the most frequent congenital coronary variant. Failure to demonstrate the anomaly can lead to erroneous interpretation of coronary anatomy with fatal complication in case of aortic valve replacement, as a consequence of accidental ligation or compression of the anomalous vessel. This procedure, especially in the presence of a bioprosthesis aortic valve just implanted, constitutes a challenge for the interventional cardiologist

Introduction Anomalous origin of the left circumflex coronary artery (LCx), from either the right coronary artery (RCA) or the right sinus of Valsalva (RSV), is the most frequent congenital coronary variant, with a prevalence of 0.18–0.67% at coronary angiography, depending on populations studied.1,2 Generally, this type of coronary anomaly has a benign clinical course and is usually diagnosed at autopsy. However, few cases of sudden death, acute myocardial infarction (AMI), and angina pectoris in the absence of atherosclerotic lesions have been reported. In particular, failure to demonstrate the anomaly can lead to erroneous interpretation of coronary anatomy and, as a consequence, can result in potentially fatal complications in case of aortic valve replacement (AVR), owing to accidental ligation or compression of the anomalous vessel.3–5

Case report A 74-year-old woman with a history of hyperlipidemia, family history of coronary artery disease, and prior anterior AMI treated with primary percutaneous coronary intervention (PCI) and stenting of left anterior descending artery (LAD) was admitted to our hospital because of symptomatic aortic valve stenosis. The preoperative echocardiogram showed mild left ventricular hypertrophy, a preserved left ventricular ejection fraction with 1558-2027 Copyright ß 2015 Wolters Kluwer Health, Inc. All rights reserved.

and at the same time a question mark regarding the strategy, choice of guiding catheter, guide wire, and type of stent to use. J Cardiovasc Med 2015, 16:000–000 Keywords: anomalous origin of left circumflex coronary artery, aortic valve replacement, percutaneous coronary intervention Cardiovascular Center Aalst, OLV Clinic, Aalst, Belgium Correspondence to Mariano Pellicano, MD, Cardiovascular Center Aalst, OLV Clinic, Moorselbaan n 164, B 9300 Aalst, Belgium. Tel: +32 0 53724447; fax: +32 0 53724550; e-mail: [email protected] Received 20 August 2014 Revised 17 November 2014 Accepted 27 November 2014

apical hypokinesia, a heavily calcified tricuspid aortic valve with severe aortic stenosis (aortic valve area of 0.6 cm2; mean gradient of 68 mmHg), and mild aortic regurgitation. The preoperative cardiac catheterization confirmed the severe aortic stenosis. The coronary angiogram revealed a normal LAD with a patent drug-eluting stent (DES) in its proximal part, a normal dominant RCA, a long left main stem, and a vessel that was being interpreted as a small circumflex artery (LCx) (Fig. 1a). After discussion within the heart team, it was decided to proceed with surgical AVR. The patient underwent a straightforward surgical procedure with implantation of a bioprosthesis valve without intraoperative complications. Two hours after cardiac surgery, she developed multiple episodes of ventricular fibrillation. After electrical reconversion, the electrocardiogram showed diffuse ST-T segment changes. Urgent coronary angiography was performed, which showed similar anatomy as compared with the preoperative examination. However, a nonselective injection of the RCA revealed a proximally occluded anomalous LCx arising from the RSV (type A)1 (Fig. 1b). Using a Judkins right 4 6-French guiding catheter and a ‘double wire technique’,6 the occlusion was easily crossed. The vessel was successfully reopened using several predilatations and finally implantation of two overlapping sirolimus eluting stents (2.5  18 mm and 2.75  22 mm) in the proximal and middle third segment DOI:10.2459/JCM.0000000000000251

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Fig. 1

(b)

(a) LCX

LAD Anomalous LCx arising from RSV (type A)

(a) Preoperative coronary angiography with erroneous interpretation of the anatomy of the left coronary artery. (b) A nonselective injection of the right coronary artery (RCA) shows a separate origin of the anomalous left circumflex coronary artery (LCx) from the right sinus of Valsalva (RSV) (type A).

of the vessel (Fig. 2). The angiographic result was excellent, as well as the further postoperative stay in the hospital without recurrence of any heart rhythm abnormalities. At the outpatient clinic 1 year later, she is stable and asymptomatic.

Discussion The recognition and angiographic demonstration of an anomalous LCx with retroaortic course is of particular importance, especially in patients undergoing AVR. The ‘sign of nonperfused myocardium’ and the ‘aortic root sign’ are two classical angiographic tricks that allow recognition of the anomaly before its direct visualization. ‘Nonperfused myocardium’ is the term that is used to describe the absence of arterial distribution to the lateral and posterior part of the left ventricular myocardial wall

when selectively injecting the left main coronary artery. The size of this ‘nonperfused’ area is directly proportional to the quantitative distribution of the anomalous coronary artery and may be very obvious or rather subtle. When confronted with this phenomenon, it should raise the suspicion that the angiographic study is incomplete. ‘Aortic root sign’ is the other indirect angiographic sign that can only be depicted when performing a left ventriculography in right anterior oblique projection. In this situation, the initial part of the anomalous LCx is seen in profile behind the aortic root, as it courses to the left atrioventricular groove.1 Another helpful sign is a seemingly long left main stem, which represents the proximal segment of the LAD. In retrospect, the abnormal origin of the LCx could have been recognized on the first angiographic study of our patient

Fig. 2

(a)

(b)

Urgent percutaneous coronary intervention (PCI) of the proximal and middle third segment of the anomalous left circumflex coronary artery (LCx).

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Unrecognized anomalous left circumflex coronary artery: a source of perioperative complication Pellicano et al. 3

as indeed there was absence of arterial distribution to the lateral and posterior myocardium. The initial difficulty for the identification and selective cannulation of this vessel could be related to the acute take-off angle in case of a type A anomalous LCx. Some authors suggest the use of preformed catheters designed for opacification of aortocoronary vein grafts for selective cannulation.1 These patients are prone to myocardial ischemia because of injury to the anomalous artery that results from compression by the prosthetic valve ring and edema around the replaced valve.4 Careful suturing of the aortic annulus and selection of a smaller prosthetic valve can avoid the risk of injury and compression of the anomalous LCx. Specific surgical techniques are the insertion of a supraannular prosthesis or biological stentless prosthesis, implantation of a homograft, or the use of a simple interrupted suture technique.3 Nevertheless, a case of sudden cardiac death 5 years after AVR has also been described, despite recognition of the anomaly preoperatively and the intentional undersizing of the implanted prosthetic valve; in this case, the prosthetic valve ring had distorted the artery leading to infarction, as observed at autopsy.7

a multipurpose guiding catheter, which could easily dissect the origin of the vessel. The preferred catheters are a Judkins right and an Amplatz left or right guiding catheter.8 Some authors suggest that PCI might be the best revascularization strategy when confronted with an anomalous LCx because of the relatively small caliber and because of the particular anatomical course, which often precludes grafting of the vessel. The small caliber of the retroaortic portion could present an issue of restenosis following PCI. The risk of restenosis can be minimized by using relatively large diameter stents (3 mm and above) or through the use of drug-eluting stents, as we did in this case.8

References 1

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Although several cases of PCI of an anomalous LCx have been reported, also in the setting of an acute coronary syndrome (ACS), this is the first case of urgent PCI after AVR. This procedure remains a challenge for the interventional cardiologist in terms of adequate cannulation of the vessel and guide support. Selection of the appropriate guiding catheter in this situation depends on the exact position of the ostium of the vessel on the RSV. In our case, we found a good support with a Judkins right 4 guiding catheter, and using a ‘double wire technique,’ we were able to relatively easily advance the balloons and the stents through the vessel. In general, it is advised to avoid

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Page HL, Engel HJ, Campbell WB, Thomas CS. Anomalous origin of the left circumflex coronary artery: recognition, angiographic demonstration and clinical significance. Circulation 1974; 50:768–773. Angelini P. Coronary artery anomalies: an entity in search of an identity. Circulation 2007; 115:1296–1305. Yokoyama S, Takagi K, Mori R, Aoyagi S. Aortic valve replacement in patients with an anomalous left circumflex artery: technical considerations. J Card Surg 2012; 27:174–177. Roberts WC, Morrow AG. Compression of anomalous left circumflex coronary arteries by prosthetic valve fixation rings. J Thorac Cardiovasc Surg 1969; 57:834–838. Morin D, Fischer AP, Sohl BE, Sadeghi H. Iatrogenic myocardial infarction. A possible complication of mitral valve surgery related to anatomical variation of the circumflex coronary artery. Thorac Cardiovasc Surg 1982; 30:176– 179. Das GS, Wysham DG. Double wire technique for additional guiding catheter support in anomalous left circumflex coronary artery angioplasty. Cathet Cardiovasc Diagn 1991; 24:102–104. Veinot JP, Acharya VC, Bedard P. Compression of anomalous circumflex coronary artery by a prosthetic valve ring. Ann Thorac Surg 1998; 66:2093– 2094. West NE, McKenna CJ, Ormerod O, Forfar JC, Banning AP, Channon KM. Percutaneous coronary intervention with stent deployment in anomalouslyarising left circumflex coronary arteries. Catheter Cardiovasc Interv 2006; 68:882–890.

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Unrecognized anomalous left circumflex coronary artery arising from right sinus of Valsalva: a source of perioperative complication.

: In this article we provide a very interesting and challenging PCI of unrecognized anomalous left circumflex coronary artery (LCx) arising from right...
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