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Dual-Guidewire Percutaneous Intervention of Anomalous
Coronary Artery Facilitated by Steerable Guidewire
Case Report. A 56-year-old male without previous cardiovascular history was admitted with chest pain suggestive of acute coronary syndrome. He reported experiencing exertional angina over the previous several days. His past medical and social histories were significant for hypertension and hyperlipidemia, as well as a 40-pack per year history of tobacco abuse. Home medications included 25 mg/day of atenolol, 10 mg/day of lisinopril and 20 mg/day of simvastatin. His family history was significant for coronary disease, but no history of unexplained sudden death.
The patient remained chest pain-free on topical nitrates, subcutaneous enoxaparin and intravenous eptifibatide infusion. Cardiac markers were within normal limits, and the electrocardiogram revealed only nonspecific ST- and T-wave abnormalities. Subsequent cardiac catheterization the following day revealed no significant stenoses in the left coronary system; however, a paucity of vasculature was noted in the lateral wall (Figure 1). Although the right coronary artery (RCA) was likewise free of significant disease, an anomalous left circumflex artery (LCX) with a 95% mid-stenosis was seen arising acutely from the right coronary ostium (Figure 2). At this point, we discussed a computerized tomographic angiography (CTA) evaluation with the patient (who had received no sedation). If the LCX was found to travel between the great vessels, coronary artery bypass surgery would be recommended. The patient adamantly and repeatedly stated that he would refuse any heart surgery, regardless of the findings. Based upon this decision, we proceeded with percutaneous intervention.
After an oral bolus of 600 mg of clopidogrel, an intravenous bolus of 0.75 mg/kg of bivalirudin and a continuous infusion of 1.75 mg/kg/hour were administered, eptifibatide was discontinued. A 6 Fr multipurpose side-hole guide catheter was selected to accommodate the inferior origin of the anomalous LCX. An initial Whisper guidewire (Abbott Vascular, Abbott Park, Illinois) was advanced distally into the RCA to serve as an anchor. The guide catheter was then disengaged from the RCA ostium to improve the angle of wire entry into the LCX. Next, a sharp curve was formed on a second Whisper wire. Although the acutely-curved wire tip successfully entered the LCX ostium, any further gentle advancement led to buckling of the wire tip and repeated prolapse into the proximal RCA. This wire was then exchanged for the deflectable tip SteerIt wire (Cordis Corp., Miami Lakes, Florida). A gentle curve was formed on the latter wire, and it was advanced into the proximal RCA, just beyond the LCX ostium. The curvature was then increased to a sharp, almost perpendicular angle, and the wire was slowly withdrawn. The tip subsequently “fell” easily into the LCX ostium. Immediately, the tip was relaxed, and the wire was easily advanced into the distal vessel (Figure 3). Subsequent balloon predilatation with a 2.0 x 15 mm Maverick balloon (Boston Scientific Corp., Natick, Massachusetts) (Figure 4) and deployment of a 2.5 x 14 mm Microdriver stent (Medtronic Inc., Minneapolis, Minnesota) proceeded without difficulties. The final angiographic result was excellent (Figure 5).
The patient was discharged the next day with an additional 75 mg/day of clopidogrel, 325 mg/day of aspirin, nicotine patches and sublingual nitroglycerines, as needed. He has remained symptom-free, but unfortunately refused to use the nicotine patches or any other smoking cessation aides. As of the time of this report, he remains asymptomatic, but an avid smoker.
Discussion
The incidence of coronary anomalies is estimated at 0.3–2%.1,2 A major consideration in these patients is the potential for sudden cardiac death. Due to the relative rarity of these aberrations, however, the overall incidence among sudden death autopsies has been reported to be from 0.6%–1.2%.3 One postmortem series demonstrated that, when compared to those under age 30, older patients demonstrated a lower risk of sudden death, likely reflective of the survival advantage of a less malignant anatomy. Non-sudden cardiac death, however, was more common in the older group, consistent with the observed greater atherosclerotic burden. It has also been postulated that the acute angulations in many anomalous coronary arteries may further promote local atherosclerosis through enhanced shear stress.4
One classification scheme divides coronary anomalies into 5 subtypes: (1) anomalous origin of > 1 coronary artery from the aorta; (2) single coronary ostium from the aorta; (3) anomalous origin of > 1 coronary artery from the pulmonary artery; (4) congenital hypoplastic coronary arteries; and (5) coronary fistulae.5 Our discussion will focus on the first 3 varieties. In their pathological review of 242 coronary anomalies, Taylor et al found that the origin of the left main artery (LM) from the right coronary sinus was most predictive of sudden death. By contrast, the origin of the LCX as a branch of the RCA, as seen in our patient, was not clearly associated with sudden death. Although 2 patients with the latter anomaly died, 1 had severe atherosclerotic stenoses and the other died of heart failure secondary to dilated cardiomyopathy. Interestingly, in individuals with a contralaterally located coronary artery, those with a single ostium appeared to have a more benign course than those with separate ostia. Moreover, among patients with single coronary arteries, those originating from the right sinus of Valsalva were more likely to experience sudden death, likely reflective of aberrations of the LM or left anterior descending (LAD) ostia.6
Pathophysiologically, the risk of sudden death associated with an anomalous coronary artery traversing between the great vessels (interarterial) may be linked to several factors. Autopsy series have reported small or “slit-like” ostia, which sometimes contain flaps or ridges.5,6 These findings were first described by Cheitlin et al in 1974.7 Moreover, these coronary arteries often arise at acute angles, further adding to flow limitations. When the LM arises from the right cusp or RCA, it may initially traverse intramuscularly through the interventricular septum. This finding alone, however, does not appear to result in adverse outcomes. The origin of the LAD from the right sinus may be associated with tetralogy of Fallot. This anomaly is an important surgical consideration to avoid intraoperative transection of the vessel.8 Furthermore, exercise-induced dilatation of the great vessels can result in dynamic coronary compression, leading to profound ischemia.5,6 It has been reported that 5–35% of sudden cardiac deaths may be attributable to such an interarterially located anomalous coronary artery.9,10
In some cases, the slit-like ostium is related to the artery’s intramural location within the aortic wall.4 One report described transesophageal echocardiographic identification of such an intramural anomaly in a 14-year-old boy with an acute myocardial infarction (MI). The LM was seen traveling within the anterior aortic wall and subsequently exiting between the great vessels. Color Doppler interrogation demonstrated flow acceleration consistent with compression, and coronary angiography further confirmed the slit-like ostium.11 Not infrequently, however, identification of such an intramural path may only be possible intraoperatively,12 as the coronary artery actually exits the aortic wall at the appropriate sinus of Valsalva.13 Angelini and coauthors reported intravascular ultrasonographic findings of anomalous intramural coronary arteries and identified proximal segmental intussusception as the main pathophysiologic mechanism. The hypoplasia index, defined as the ratio of the proximal-to-distal circumference, as well as the asymmetry index, the ratio of the minimal-to-maximal diameters in the oval intramural segment, were proposed as additional measures of severity. They further demonstrated enhanced proximal narrowing with dobutamine infusion.14
In one of the largest angiographic series on anomalous coronary arteries, Yamanaka and Hobbs found an incidence of 1.3% among 126,595 reviewed studies. Of these anomalies, 87% were abnormalities with an ostial location, with the most common being separate LAD and LCX ostia. Prognostically benign, this anatomic variation has also been associated with bicuspid aortic valves. The origin of the LCX from the RCA, as in our patient, likewise was associated with a favorable outcome. However, in patients undergoing mitral or aortic valve replacement, care must be taken to avoid trauma to the retroaortic arterial segment.15
The Bland-White-Garland syndrome, characterized by an aberrant coronary origin from the pulmonary artery, is a rare finding in the adult population, as 90% of these patients die during infancy. The anomalous vessel, usually the LM, is collateralized in a retrograde fashion by antegrade flow in the normal coronary artery. The subsequent drainage into the pulmonary artery thus establishes a left-to-right shunt. The majority of patients become symptomatic with angina, heart failure or myocardial infarction. Surgical ligation and bypass grafting are the recommended treatments.16 One pediatric series described 4 patients (mean age, 8.5 months) with an aberrant LM originating from the pulmonary artery and coursing intramurally through the aortic wall prior to the left ventricular distribution. Identification of the intramural aortic course, however, was only possible intraoperatively.17
As anomalous coronary arteries are typically found in nontraditional locations, guide catheter engagement and guidewire manipulation in these situations are often challenging. In our case, while a sharp wire-tip curve was necessary to gain entry into the acutely angulated LCX ostium, further advancement resulted in buckling and prolapse into the RCA. The SteerIt wire can be a valuable tool in anatomies requiring dynamic wire-tip conformations. The device is an 0.014 inch diameter wire consisting of a thin filament affixed at its distal tip to that of a hypotube in which it is housed. A sliding handle provides the operator control of continuous and variable tip curvature deflection throughout the procedure.18
We have previously described its utility in recrossing deployed stents by forming a maximal curve or “knuckle” at the proximal stent edge to avoid substrut passage. After exiting the stent, the tip is then relaxed and reshaped to navigate the distal coronary anatomy.18 In the present case, the wire tip was advanced beyond the target ostium and then carefully withdrawn with a newly formed acute curve. In tightly angulated branches, we have found that this technique frequently allows the wire tip to “fall” into the ostium. The tip is then relaxed immediately to avoid prolapse with further advancement. The versatile tip conformation of this wire may be helpful in treating other acutely angluated coronary anomalies. Moreover, the use of a rail or anchor wire in the main vessel allowed for catheter disengagement to improve the angle of entry into the anomalous LCX.
The late Dr. Thomas Linnemeier previously described an innovative guide catheter modification for intervention of an anomalous LCX from the RCA. By cutting a small hole along the greater curvature near the tip of a Judkins right catheter, access to the inferiorly located LCX can be obtained.19 Percutaneous interventions in one or both coronary arteries originating from a single ostium from the right sinus of Valsalva has previously been described in the literature.20,21 In their case report, Kang and associates were unable to advance a stent into the aberrant left coronary artery during the initial procedure. A repeat intervention 2 weeks later was required for successful stent deployment.21
Given the potential technical challenges, a higher complication rate may be expected in these cases. Lee et al reported arapidly extending ascending aortic intramural hematoma resulting from percutaneous intervention in an anomalous RCA. Emergent surgical repair was required.22 Hershey and associates performed primary percutaneous intervention in a nonsurgical candidate with a single coronary ostium presenting with acute MI and cardiogenic shock. The patient had a collateralized right coronary occlusion and a culprit thrombus in the anomalous LM traversing between the great vessels. The procedure was life-saving, and the patient made a complete recovery.23 As catheter-based interventions for interarterial anomalous coronary arteries are reserved only for unusual circumstances, no large-scale, long-term follow-up data are available.
Previously, invasive angiography was required to delineate and rule out an interarterial course of the anomalous artery. This technique usually involved concomitant insertion of a pulmonary artery catheter and coronary angiography from the left lateral projection. Identification of the aberrant vessel’s course in relation to the pulmonary artery and ascending aortic catheter positions, however, can, at times, be challenging. Today, cardiac computerized tomographic angiography and cardiac magnetic resonance imaging provide clear, noninvasive, visualization of both the anomalous vessel course and surrounding anatomic structures. Additionally, combined pharmacologic stress testing further allows for functional evaluation of any detected coronary stenoses.24 In our case, however, the patient’s decision precluded any surgical considerations. Given that the aberrant vessel was a small, nondominant circumflex rather than the entire left coronary system, coupled with the patient’s sedentary lifestyle, we did not aggressively pursue the surgical option. Furthermore, as discussed above, this particular aberrant anatomy has not been associated with sudden death risk.4 Surgical bypass is the therapy of choice for an interarterial anomalous coronary artery.4,23 At least one report, however, has described successful stenting of such an aberrant vessel after failed bypass surgery.25
In summary, complete diagnostic evaluation of coronary anomalies frequently involves delineation of their courses in relation to the great vessels. Classification of benign versus potentially malignant forms of coronary anomalies are summarized in Table 1. When atherosclerotic disease is diagnosed in patients with the benign anatomic variations, percutaneous intervention may be a reasonable therapy. These cases, however, may require innovative guide catheter selection and careful guidewire manipulations. We believe the steerable guidewire, in conjunction with a second anchor wire, is a potentially useful tool in percutaneous intervention of anomalous and other angulated coronary anatomies.
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