From the Department of Cardiology, Royal Hospital, Sultanate of Oman. The authors report no conflicts of interest regarding the content herein. Manuscrript submitted August 28, 2008, provisional acceptance given September 24, 2008, manuscript accepted October 2, 2008. Address for correspondence: Prashanth Panduranga, MBBS, MD, Department of Cardiology, Royal Hospital, PB 1331, Sultanate of Oman, Muscat, 111. E-mail: firstname.lastname@example.org
ABSTRACT: Coronary arteries of anomalous origin are uncommon and all three coronary arteries arising from a single sinus of Valsalva is very rare. Percutaneous coronary intervention of such arteries is technically difficult and challenging. We report the case of a patient with all three coronary arteries arising from the right sinus of Valsalva who presented with acute coronary syndrome. Coronary angiography showed significant stenosis of the left circumflex and left anterior descending coronary arteries followed by successful angioplasty and stenting of these arteries.
J INVASIVE CARDIOL 2009;21:E33–E36
Case Report. A 62-year-old diabetic and hypertensive male was admitted to our center with typical rest anginal pain, T-wave inversion in leads V5–6, I and AVL and positive troponin T. Coronary angiography using Judkins catheters revealed all three coronary arteries originating separately from the right sinus of Valsalva (RSOV) (Figures 1 and 2). The right coronary artery (RCA) arose from the RSOV and followed the usual pathway to the right atrioventricular sulcus.The left anterior descending artery (LAD) course was anterior to the pulmonary artery. The course of the left circumflex artery (LCX), better seen with a Multipurpose catheter (Figure 2), was posterior to the aortic root. The anomalous circumflex artery originated from the right coronary sinus traversing in a retroaortic course, and there were 90% and 95% tight stenotic lesions of the mid-LCX. An anomalous LAD arising separately from the right coronary sinus traversing anterior to the pulmonary artery had an 80% proximal stenosis and the RCA was dominant and disease-free. The patient’s left ventricular function was normal, with a left ventricular ejection fraction (LVEF) of 64%. During intervention of the LCX and LAD, the arteries could not be cannulated even after using different catheters including the Amplatz catheter. The LCX was selectively cannulated with a Multipurpose MPA1 6 Fr guide catheter (Cordis Corp., Miami Lakes, Florida). The LCX stenotic lesions were successfully crossed with a 0.014 inch Zinger support wire (Medtronic, Inc., Minneapolis, Minnesota) and dilated with a 2 × 20 mm Worldpass balloon (Cordis) (Figure 3). After predilatation of the narrowed segments, two 2.75 × 13 mm Multi-Link Zeta stents (Guidant Corp., Indianapolis, Indiana) were deployed at 16 atm with a small overlap. A final diagnostic angiogram showed an excellent angiographic result (Figure 4). The LAD was cannulated using an Internal Mammary Curve (IM) 6 Fr guiding catheter (Cordis) and the lesion was crossed with 0.014 inch Hi-Torque BMW wire (Guidant) and directly stented using a 3 x 13 mm Multilink Zeta stent with an excellent final result (Figure 5 ). The immediate post-procedure stay of the patient was uneventful and he was discharged the next day on dual antiplatelet therapy. The patient continued to remain asymptomatic with a good quality of life at 6-month follow up. Discussion. Coronary arteries of anomalous origin are uncommon and found in only 0.17–1.3 % of patients.1,2 Although clinically thought to be benign, these anomalies can cause myocardial ischemia, infarction and/or sudden death. The imaging modalities to diagnose these anomalies are standard coronary angiography along with computed tomography (CT),3 magnetic resonance imaging (MRI)4 and echocardiography.5 Recently, intravascular ultrasonography (IVUS) has been used to evaluate the mechanisms of ischemia in these patients and has shown an intramural course of these arteries.6 Anomalous origin of the LCX from the RSOV or proximal RCA is one of the most common forms of coronary artery anomalies. It has been reported in 0.17–0.45% of patients undergoing selective coronary angiography.2,7,8 This anomalous LCX artery passes behind the aortic root and has not been associated with death. A RCA arising from the left sinus of Valsalva (LSOV) as a separate vessel or as a branch of a single coronary artery has been reported in 0.03%–0.17% of patients who undergo angiography.2 The most common course of an anomalous RCA arising from the LSOV is interarterial. This variant can be associated with sudden cardiac death in up to 30% of patients. A left coronary artery arising from the RSOV as a separate vessel or as a branch of a single coronary artery occurs in 0.09–0.11% of patients who undergo angiography.9 An interarterial course may be seen in up to 75% of patients with this anomaly, and such patients are at high risk of sudden cardiac death due to the acute angle of the ostium, the stretch of the intramural segment and the compression between the commissure of the right and left coronary cusps. However, this anomalous left coronary artery may also take a retroaortic, prepulmonic or septal course. The LAD may arise from the right sinus in tetralogy of Fallot, double-outlet right ventricle and transposition complexes, but rarely in patients with otherwise normal hearts. Anomalous coronary arteries with separate RCA, LAD, and LCX, all arising from individual ostia in the right sinus of Valsalva has been described as an exceedingly rare occurrence,10 and for the most part only isolated case reports exist in the literature. Yamanaka and Hobbs2 have reported one case in their series, Engel et al11 found four cases and in Coronary Artery Surgery Study, Click et al12 reported only three cases. Around 27 such cases have been reported previously and the largest number of these anomalies was reported recently by Kashyap et al who had 7 patients with this anomaly diagnosed using coronary angiography and cardiac MRI4 with a reported incidence of 3.1% among 16,109 coronary angiograms. In their series, the RCA arose from the RSOV and followed the usual pathway to the right atrioventricular sulcus, the LAD course was anterior to the pulmonary artery or the right ventricular outflow tract in the majority of cases and along the interventricular septum in a few. The course of the LCX was posterior to the aortic root in all patients. This pattern corresponds to the anatomical course of all three coronary arteries in our patient. Regarding the association between atherosclerosis and coronary artery anomalies, an analysis from the coronary artery surgery study12 showed that anomalous circumflex coronary arteries had a significantly greater degree of stenosis than what is found in nonanomalous arteries in age- and gender-matched control patients. Increased risk of myocardial injury is thought to result from several different etiologies including a narrowed or slit-like ostium of the vessel, acute angle of takeoff or an interarterial course. Myocardial injury or ischemia has occasionally been reported with courses typically thought of as benign.13 The management of patients with an anomalous coronary artery includes medical treatment or observation, coronary angioplasty with stent deployment or surgical repair. Coronary anomalies cause technical problems during coronary angiography as well as during percutaneous transluminal coronary angioplasty (PTCA). There are only a few reports of PTCA of anomalous coronary arteries including rotational atherectomy and stenting (“rota-stenting”) and laser angioplasty.14–18 Most of the reports involve percutaneous intervention of an anomalous LCX or RCA and, very rarely, an LAD. When cannulating the coronary arteries, the vessel’s orifice configuration and its exit angulation from the aorta play an important role in choosing the proper guiding catheter. The location and morphology of the target lesion and the course of a target anomalous artery affect the delivery of stents. Previous reports of intervention in anomalous coronary arteries have documented considerable technical difficulties related to the location of the ostium of the anomalous artery as well as its anatomic course and morphology. The initial angiographic obstacle is caused by the origin of the target vessel. Repeated attempts at cannulation of the ostium of the anomalous vessel with various guiding catheters are commonly required. Over the years, improved guiding catheter configurations, supportive guidewires, rapid-exchange low-profile balloons, flexible, low-profile debulking devices and stents have helped to overcome these difficulties. In our patient, the initial angiography was performed using Judkins catheters. However, during PTCA of the LCX and LAD, cannulation was not possible with the Judkins and Amplatz catheters. The Ostium of the LCX was anterior and inferior and use of the Multipurpose guiding catheter in this patient provided easy cannulation with enough backup support to ensured proper angiographic opacification. The choice of the Multipurpose guiding catheter was based on its shape, large area of support and location of the artery just below the right ostium with an inferior takeoff. It provided the maximum stable support required for smooth passage of the balloon as well as stent. The tip of the catheter sits well in the anomalous vessel and the curve rests stably against the opposite aortic wall. Thus, it appears that the Multipurpose guiding catheter may be the best choice for PTCA of a coronary artery with a similar anomaly. The LAD ostium was posterior with superior takeoff, hence the internal mammary catheter cannulated it well and provided good support for angioplasty. Several techniques have been reported for PTCA in anomalous arteries.15,19,20 Topaz et al15 described various aspects of orifice configuration, anatomy of the artery, location of atherosclerotic lesions and guiding catheter selection as well. Proper guiding catheter selection decreases procedure time in PTCA involving anomalous coronary arteries and thus increases success rates. We were able to cannulate the anomalous LCX and LAD in our case with relative ease and had good backup support using the Multipurpose and IM guiding catheters, respectively. Thus, after carefully studying the course of the anomalous artery and the location of the lesion, and with selective use of the guiding catheter, angioplasty and stenting can be successfully performed in patients with anomalous LCX and LAD originating from the RSOV. To our knowledge, this is the first case report of a patient with all three coronary ostia arising from the RSOV who underwent PCI of the LCX and LAD.
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