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Case Report
Percutaneous Coronary Intervention in Anomalous Right Coronary Arteries Arising from the Left Sinus of Valsalva:
A Report of Tw
February 2006
Congenital anomalies of the origin and distribution of the coronary arteries are infrequent and are found in 0.3–1.2% of patients undergoing coronary angiography.1–3 The prevalence is greater if the origin of a coronary artery from an ectopic ostium that is still in the correct sinus of Valsalva is included.4
The incidence of anomalous origin of the right coronary artery (RCA) out of the right sinus of Valsalva ranges from under 0.01–0.09%.1–6 It usually arises from the left sinus of Valsalva or the ascending aorta above it, and in most reported cases it courses between the great vessels. Rarely, the anomalous origin is found to be from the posterior sinus or another coronary artery.
Selectively cannulating the anomalous RCA arising from the left sinus of Valsalva is usually challenging owing to the unusual site of its ostium within the sinus and its ellipsoidal shape, the acute opposite direction course of its proximal segment (Figure 1), and the lack of specially-designed catheters.7 In most cases, it is possible to visualize the vessel with catheters designed for cannulating the left main stem such as oversized or undersized left Judkins and Amplatz catheters, XB, Voda and left coronary bypass catheters. If the anomalous vessel has significant atherosclerotic disease requiring percutaneous coronary intervention (PCI) the cannulation procedure becomes even more demanding. Simple visualization of the artery is not enough. Guiding catheter stability and extra support for advancing the guidewire, balloon and stent catheters through an artery with sharp turning takeoff and opposite direction course of its main trunk are paramount.
Although the overall prevalence of an anomalous RCA arising from the left sinus of Valsalva is very low, it is unlikely that a busy interventionalist will not encounter such an unusual case during his or her career. Nguyen et al recently reviewed 19 cases and we identified 10 additional reported cases of PCI in such arteries.8–15 Many authors have made suggestions for a standardized approach despite their limited experience. We describe our experience with two cases that illustrate that each case requires a slightly different approach. We also discuss tips that may increase the likelihood of technical success. Of interest isthat in both cases, similarly to most cases reported to date, the lesions were situated in the proximal segment as it coursed between the aorta and the pulmonary artery.
Case 1. A 50-year-old male with a history of diabetes mellitus, hypertension and dyslipidemia presented with an inferior ST-elevation myocardial infarction (STEMI) with right ventricular involvement. He received thrombolytic therapy and a limited cardiac enzyme elevation was noted. He underwent elective coronary angiography in our laboratory 3 weeks later where an anomalous RCA arising from the left sinus of valsalva with a 90% stenosis at its proximal segment was found (Figure 2A). He also had a lesion of borderline significance in the proximal LAD. The anomalous RCA could not be cannulated with diagnostic Judkins left 3.5, 4 or 4.5 catheters, but was well visualized with an Amplatz Left 2 catheter. The use of a similar 6 Fr guiding catheter offered only limited support, and when a BMW guidewire (Guidant Corp., Indianapolis, Indiana) was advanced into the proximal RCA, it disengaged altogether. However, it was possible to advance a softer hydrophilic ChoICE® PT guidewire (Boston Scientific Corp., Natick, Massachusetts). The proximal lesion was dilated with a 3.0 x 20 mm balloon, but it was impossible to advance a 3.5 x 23 mm Zeta stent (Guidant) due to poor catheter backup. Finally, the stent was advanced and successfully implanted after changing the entire guide set-up and using a 6 Fr XB 4 catheter (Figure 2B). The fluoroscopic time was 32.8 minutes, the X-ray dose 261.2 Gy cm2, and 600 ml of contrast agent were used.
The patient underwent LAD angioplasty and stenting 4 months later, and no in-stent restenosis was noted in the RCA at that time.
Case 2. A 76-year-old female with a history of hypertension and dyslipidemia presented with an inferior STEMI. She received thrombolytic therapy, and a modest cardiac enzyme elevation was noted. During her hospitalization, she experienced postinfarction angina and was transferred to our laboratory for coronary angiography. An anomalous RCA arising from the left sinus of Valsalva with a 95% stenosis at its proximal segment was found. The left coronary artery had only minor atherosclerotic disease.
The anomalous RCA could not be cannulated with Judkins Left 3.5 and 4 diagnostic catheters, but was well visualized by using a 6 Fr XB 3.5 guiding catheter (Figure 3A). However, the back-up support offered by this catheter was poor and a ChoICE PT guidewire could not be advanced distally in the RCA. Finally, this was achieved easily by using a 6 Fr Amplatz Left 1 guiding catheter. Dilatation with a 2.5 x 15 mm balloon and implantation of a 2.75 x 12 mm Taxus® stent (Boston Scientific) were straightforward (Figure 3B). The fluoroscopic time was 40.5 minutes, the X-ray dose 119.3 Gy cm2, and 550 ml of contrast agent were used.
Discussion
We report our experience involving two patients who had significant atherosclerotic disease and who were successfully treated with PCI and stent implantation in an anomalous RCA arising from the left sinus of Valsalva. Our contribution brings the total reported number of such cases to 31.8–15 We also present an illustrative MRI image of our second patient’s coronary artery anomaly.
The acute angle of origin of the anomalous RCA from the juxtacommissural area of the left sinus of Valsalva toward an abrupt rightward and downward direction creates a slit-like luminal orifice and ostial stenosis due to phasic kinking.16–18 In addition, phasic obliteration in the proximal segment of the anomalous RCA as it courses between the aorta and the pulmonary artery has been demonstrated with IVUS studies, and in some cases can be the only substrate for myocardial ischemia in the absence of atherosclerotic disease.19 These peculiar anatomic circumstances make selective cannulation and, to a greater extent, percutaneous intervention in the anomalous artery extremely challenging, as evidenced by ours and others’ experiences. Attesting to the difficulties of selective cannulation of the anomalous RCA is that a purpose-designed catheter has been recently developed.15
In agreement with previous reports, we faced several technical challenges that required frequent catheter exchanges to find the best-fitting catheter and guidewire, as validated by the extended fluoroscopic time and the amount of contrast agent used.
In contrast to some previous reports, we did not find the usage of a particular guiding catheter suitable for both of our cases. Specifically, a Judkins Left guiding catheter, reported to be successful by others,9,14,19,20 was not capable of cannulating the anomalous artery. We were able to cannulate the anomalous arteries with Amplatz Left and XB guiding catheters, but even then, the support offered was not equal and we actually had to interchange these two catheters in both cases to perform the stent implantation. Our experience confirms that each case requires an individualized approach and demonstrates how unpredictable the best-fitting catheter can be, and also supports the view that no single strategy can be successful in all cases. The choice of guidewire can also affect the final outcome. We found that use of a soft, hydrophilic wire such as the ChoICE PT offered an advantage over stiffer, nonhydrophilic wires that require enhanced guiding catheter support.
An interesting observation is that the pattern of atherosclerosis in both our anomalous RCAs was very similar. The stenosis was located in the proximal segment of the artery as it traverses between the great vessels. We reviewed all the reported PCI cases in anomalous RCAs arising from the left sinus of Valsalva and found that the same pattern emerges as dominant (20 of 22 lesions, or 91%; 20 with published images and 2 with clearly stated diseased segment were located in the proximal segment).8–15,20 Although this could be the result of lesion selection or reporting bias, the skewed spread of the disease toward the proximal segment is remarkable. It is possible that the unusual angle of takeoff, the phasic compression and longitudinal vessel wall stress and the altered shear stress in that segment of the anomalous RCA, predispose patients to accelerated atherosclerosis. Although our data support this theory, conclusive confirmation of any atherogenous effect of this anomaly is problematic due to its rarity.
It has been suggested that the abnormal origin and course of anomalous coronary arteries could make them more prone to atherosclerosis due to altered flow patterns.21 Previous studies have suggested either a greater degree of stenosis in anomalous arteries, or no difference compared to normally-originating coronary arteries.1,21,22
The effect of the alteration of hemodynamic factors on atherosclerosis has been studied in myocardial bridging, another condition where phasic exterior compression is exerted in a coronary artery. Pathology and ultrasound studies have shown that the presence and evolution of atherosclerosis are greatly affected in this situation, with the segment beneath the bridge protected, in contrast to the neighboring and the proximal segment in particular that exhibit accelerating intimal thickening and atherosclerosis.23,24 Evidently, the proximal segment of an anomalous RCA arising from the left sinus of Valsalva is different in that it has no adventitia, but is suspended into aortic media and the inner elastic lamina and is additionally subjected to phasic longitudinal wall stress. However, concurrent to myocardial bridging, in our cases and most other reported PCI cases, the lesion was at the inlet or outlet of the tunnel between the RCA segment of the great vessels.
Optimal guiding catheter seating with the best possible coaxial alignment offering adequate backup support, the general principle for successful PCI, are extremely important when dealing with an anomalous RCA arising from the left sinus of Valsalva. We found that a guiding catheter with a curve that seats against the posterior wall of the aorta, such as the Amplatz and XB catheters, provide the reliable backup needed for the intervention. However, our experience confirms that despite the fact that we were faced with two patients with very similar anatomy, each patient required an individualized approach and that no single catheter is necessarily optimal. Because these cases are rare, the reported experiences of other practitioners may provide helfpul tips, but they do not necessarily increases success rates or decrease radiation exposure and contrast load. The lesions treated in our patients and in the majority of other reported cases were located in the proximal segment of the anomalous RCA, suggesting that this segment may be susceptible to accelerated atherosclerosis.
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