Abstract
Background
Anomalous origin of the left main coronary artery from the right sinus of Valsalva (ALCA-RSV) is exceedingly rare yet complicates emergency angioplasty for ST-segment elevation myocardial infarction.
Cases Summary
We describe 2 ST-segment elevation myocardial infarction patients in whom ALCA-RSV was identified during primary percutaneous coronary intervention. Despite procedural challenges, timely revascularization was achieved using customized access, catheter strategies, and adjunctive devices. Postprocedural computed tomography coronary angiography excluded malignant courses. Both patients achieved positive outcomes.
Discussion
ALCA-RSV, while exceptionally uncommon, is associated with sudden cardiac death and procedural difficulties during percutaneous coronary intervention. Early identification, precise anatomical imaging, and adaptable techniques are key to successful management. This series contributes to limited existing literature, underscoring that favorable results are possible with tailored intervention strategies.
Take-Home Messages
ALCA-RSV complicates emergency angioplasty and necessitates adjusted strategies to achieve revascularization. Multimodality imaging aids risk assessment and delineates malignant from benign courses.
Key words: acute coronary syndrome, coronary artery anomalies, malignant versus benign coronary course
Graphical Abstract
Anomalous coronary arteries originating from the opposite sinus of Valsalva are rare, with an estimated prevalence of 0.6% to 1.5% in coronary computed tomography angiography (CTCA) and angiography populations.1, 2, 3 Anomalous left coronary artery from the right sinus of Valsalva (ALCA-RSV) is even less common, accounting for only 0.15% of cases.2,4 Despite their rarity, these anomalies are clinically significant as they can lead to ischemia, arrhythmias, or sudden cardiac death (SCD).5 In acute coronary syndromes, they complicate management, posing challenges during percutaneous coronary intervention (PCI).5, 6, 7 Few case reports exist of successful PCI for this anomaly in patients presenting with ST-segment elevation myocardial infarction (STEMI), and to our knowledge, only one case series has been published.8 Early recognition of this anomaly is crucial for guiding PCI and minimizing complications and mortality.1,6 This series presents 2 STEMI patients with ALCA-RSV, emphasizing the importance of early identification and tailoring management strategies during PCI to optimize outcomes.
Patient 1
A 59-year-old hypertensive male presented with 10 days of indigestion-like chest pain. On presentation, he experienced dyspnea, nausea, and diaphoresis. His cardiovascular examination was yet unremarkable. An electrocardiogram (ECG) done on arrival revealed ST-segment elevation in the anterior leads. Troponin levels were significantly elevated at 1,400 ng/L. The combination of his clinical presentation, ECG findings, and risk factors (age, gender, and hypertension) were consistent with an anterior STEMI.
An emergent primary coronary angioplasty was undertaken. Right radial artery access was established, and an extra backup 3.5 guide catheter was used for engagement. Initial attempts to engage the LCA were unsuccessful, and nonselective imaging failed to visualize the left coronary sinus. The right coronary artery (RCA) was only partially engaged, and the obtained pictures raised the suspicion of an anomalous left coronary origin. At that point, the procedure was complicated by severe arterial spasm of the upper limb and hemodynamic deterioration with cardiogenic shock requiring inotropic support. Troubleshooting these challenges, we switched to right femoral artery access. Amplatz Left (AL) 0.75 guide catheter was then selected and provided reasonable engagement with appropriate images. This confirmed the presence of an ALCA originating from the right sinus of Valsalva and bifurcating into the left circumflex and left anterior descending (LAD) arteries; the latter was totally occluded from the ostium with a short stump. A GuideLiner extension catheter (Teleflex) was used for additional support. This facilitated predilatation and thrombus aspiration, followed by the deployment of 2 drug-eluting stents in the LAD, achieving TIMI flow grade 3.
Postprocedure, the patient had an uneventful recovery. CTCA excluded malignant course with retro-aortic course. A cardiac magnetic resonance (CMR) imaging later showed moderately impaired left ventricular systolic function with an ejection fraction (EF) of 38%, with no reversible ischemia. He was discharged on dual antiplatelet therapy and secondary prevention medications. He completed cardiac rehabilitation exercises and returned to work.
A transthoracic echocardiogram was performed several months later and demonstrated improvement in the left ventricular function, with an EF of 45%. The patient remained asymptomatic during follow-up and was subsequently discharged to his general practitioner (Figure 1, Video 1).
Figure 1.
Patient 1: Coronary Angioplasty and CT Images
(A) Coronary angiography images before and after angioplasty to LAD. Arrow showing LAD stump and after stenting with a drug eluting stent. (B) CT coronary angiogram with the anomalous origin of the left main coronary artery with retro-aortic course and proximal LAD stent in situ. Arrow showing the retro-aortic course of left main and LAD proximal stent. LAD = left anterior descending.
Patient 2
A 59-year-old man known to have hypertension and an ex heavy smoker presented with central chest and epigastric tightness radiating to the back, which progressively worsened over the last 2 weeks. An initial 12-lead ECG demonstrated dominant R waves and ST-segment depression in the right precordial leads. A CT aorta ruled out dissection. A posterior ECG subsequently showed ST-segment elevation in posterior leads, and his troponin level was markedly raised at 800 ng/L. He was then transferred to a primary percutaneous intervention center. Right radial artery access was obtained. Nonengagement of the left coronary cusp using Judkins catheters led to an aortogram, which showed both coronary arteries taking origin from the right coronary sinus. Partial engagement of the RCA demonstrated proximal occlusion.
An AL 1 guide catheter with a GuideLiner extension catheter was used, and a workhorse wire was passed through the occluded RCA with significant difficulty and the use of a proximal anchor balloon for support. The lesion was dilated with several balloons, which resulted in TIMI flow grade 3. Further images revealed a loop and calcifications in the mid-segment, and surprisingly, the distal segment of the RCA was supplying the left circumflex territory. After predilatation with noncompliant balloons, stenting was facilitated with 2 overlapping drug-eluting stents followed by optimization with noncompliant balloon. The angiographic result was satisfactory with TIMI flow grade 3.
Following angioplasty, the patient was commenced on dual antiplatelet therapy and secondary prevention medications. An echocardiogram before discharge showed impaired left ventricular systolic function with an EF of 46% and akinetic basal, mid-inferolateral, and inferior segments. The patient recovered well and was discharged.
To rule out a malignant course, an ECG-gated CTCA was performed. This revealed a subpulmonic nonmalignant course. Interestingly, the CTCA reported an unaddressed severe distal RCA lesion. A subsequent angiography showed patent RCA stents, and the distal lesion was assessed with pressure wiring, which yielded negative results. The patient was asymptomatic and was discharged to his general practitioner (Figure 2, Video 2).
Figure 2.
Patient 2: Coronary Angioplasty and CT Images
(A) Coronary angiography images before and after angioplasty to RCA. Black arrow showing the left main artery arising from the right coronary sinus, blue arrow showing RCA supplying LCX territory. (B) CT coronary angiogram with the anomalous origin of the left main coronary artery with subpulmonic nonmalignant course. Black arrow showing anomalous origin of the left main, white arrows denoting its course through the basal anterior septum below the pulmonic valve. LCX = left circumflex; RCA = right coronary artery.
Discussion
Coronary artery anomalies (CAAs) encompass diverse structural deviations from normal coronary anatomy.1,9 To discriminate them from normal variants, an incidence of <1% in the general population has been historically suggested.6 However, the increasing utilization of diagnostic imaging for coronary artery disease resulted in more frequent detection, with studies reporting an incidence of 1.3% in population undergoing coronary angiography.6 Despite being rare, CAAs have garnered attention due to their association with an increased risk of SCD in multiple studies. They were the second-most-common cause of SCD in young competitive athletes according to autopsy reports.5 CAAs are broadly classified into anomalies of origin, course, or termination1,9 (Table 1). Further classification by the course is of more clinical significance, with retro-aortic, interarterial, subpulmonic, prepulmonic, and retrocardiac courses being recognized1,3,5 (Figure 3). The interarterial course, often termed “malignant,” has been linked to SCD, in addition to the malignant coronary origin from the pulmonary trunk.5 Despite this association, the exact clinical and prognostic implications of CAAs remain incompletely understood.7
Table 1.
Simplified Classification of Coronary Artery Anomalies
| Anomalous origin | Anomalous pulmonary origin
|
| Anomalous course | Myocardial bridging Coronary aneurysm |
| Anomalous termination | Coronary fistula |
Figure 3.
Anatomical Illustration of Anomalous Coronary Arteries
(A to F) Anatomical illustrations of the normal origin of the left coronary artery and various courses of the anomalous origin from the right coronary sinus.
Our cases represent examples of an anomalous aortic origin from the contralateral sinus of Valsalva (AAOCA). The true prevalence of this anomaly is difficult to ascertain. A prevalence of 0.1% to 0.3% among the general population has been suggested.6 In 2 large studies, the prevalence was found to be 1.5% to 1.7% in CTCA population.2,3 After excluding known anomalous coronaries, the prevalence was 0.8% in one of the CTCA studies, which aligns with reported prevalence in one of the largest angiography studies.3,6 The data regarding clinical significance and prognosis are heterogenous. Many autopsy studies have linked AAOCA to an increased risk of SCD, particularly in young athletes.5 In contradiction, CTCA studies have failed to find any significant difference in outcomes compared to the control group.3,10 An ALCA-RSV as in our cases is exceedingly rare with an estimated prevalence of 0.1% in CT and angiography population.2,6
Risk stratification in patients with CAAs is essential in determining the likelihood of adverse outcomes, particularly SCD. Besides the coronary course, high-risk features include cardiovascular symptoms, acute angulation of the coronary takeoff, “slit-like” proximal narrowing, narrowing length >5.4 mm, and intramural segments within the aortic wall.1,5 Imaging is pivotal for both diagnosis and risk assessment. CTCA and CMR provide detailed anatomical characterization of these anomalies including the presence of other high-risk anatomical features.3,9 CTCA, with its superior spatial resolution, became the preferred imaging modality for accurate risk stratification and guiding management decisions.2,3,7 In addition, stress testing can assist in guiding treatment strategies,1,7 which was used in our first patient via CMR and second patient with an elective pressure wire testing.
In our cases, CTCA was instrumental in precisely delineating the anomalous coronary anatomy and guide further management plans. Retro-aortic and subpulmonic courses were identified in the first and second cases, respectively, which excluded malignant interarterial pathways. Furthermore, CMR ruled out inducible ischemia in the first case, confirming that no further intervention was necessary. This comprehensive imaging approach informed our decision toward conservative management, obviating the need for surgical referral.
ALCA-RSV presenting with STEMI is exceedingly rare. In a 2-center study of 12,092 patients undergoing PCI, only 10 patients with an AAOCA presented with STEMI (prevalence of 0.05%).7 ALCA-RSV was recognized in just 2 patients.7 Despite the inherent procedural challenges, successful outcomes remain achievable with tailoring the interventional strategy.7,8 The technical challenges encountered during angiography and angioplasty were accurate diagnosis, early detection, appropriate catheter selection, wiring the occluded vessel, and backup support for balloon or stent delivery, risking dissecting the other backup coronary vessel.4,7 Therefore, careful assessment of the AAOCA configuration, angulation, course, and subsequent selection of an appropriate guide catheter is critical to the final success of the procedure.4,7
Guidance on PCI in patients with anomalous coronary artery is limited, and innovation remains essential in current literature. Several strategies have been proposed on how to tackle the unique challenges of PCI in those patients. Here are the main recommendations along with our experience in managing similar cases:
Access selection
The femoral approach was used in most patients with AAOCA with suggestions that it may provide better outcome due to easier catheter manipulation and exchange.4,7 However, because an underlying culprit anomalous coronary artery is often unexpected in the acute coronary syndrome setting, radial access may be viable. Despite femoral access facilitating catheter exchange and adjunctive tool use in our first case, we successfully performed PCI via the radial approach in the second case. This suggests that selecting the access route based on operator proficiency and patient-specific factors may be more practical than adhering to one approach.
Catheter selection and image acquisition
Selective cannulation of an aberrant coronary can be technically challenging, and aggressive attempts at engagement may risk catheter-induced dissection.4,7 There is no consensus on the optimal catheter selection for anomalous coronaries, leaving the choice largely to the operator's experience and the specific anatomy encountered. Although previous studies have reported success with Judkins right catheters for engaging ALCA-RSV, we achieved success using AL catheters in both cases, underscoring the importance of operator flexibility and innovation.4,7
Adjunctive tools for extra backup support
Several studies and case reports have suggested the use of anchoring balloons to provide additional support during PCI in anomalous coronary arteries.7,8 Extension catheters, such as the GuideLiner, have been shown to enhance stability and facilitate stent delivery in these complex anatomies. In both our cases, the extension catheter was instrumental in ensuring stable guide catheter to prevent aggressive guide catheter intubation and dissection, which contributed to the success of stent delivery and deployment. In addition, the anchor balloon technique was used in the second patient which provided good support for successful angioplasty.
Wiring and stent choice
Apart from gentle manipulation, the literature does not provide specific recommendations on wire or stent selection.7 In our experience, a workhorse wire was used, the stent selection was tailored based on the specific lesion and anatomical considerations, and no complications arose in either case.
Conclusions
ALCA-RSV presenting with STEMI is exceptionally rare yet poses significant challenges during PCI. Our series highlights the importance of tailored intervention strategies and operator adaptability to ensure successful outcomes in addition to the rule of CTCA or CMR to assess the anomalous course. Continued innovation is needed to optimize management strategies for these complex cases.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Take-Home Messages
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•
Percutaneous coronary intervention in patients with anomalous origin of the left main coronary artery from the right sinus of Valsalva can be very complex especially in emergency situations and calls for adjusted strategies to achieve successful revascularization.
-
•
Using different imaging modalities aids risk assessment of this anomaly and delineates malignant from benign courses.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
For supplemental videos, please see the online version of this paper.
Appendix
Patient 1 Angioplasty
(A) Posteroanterior caudal view with the anomalous left main coronary artery from RCA with an occluded ostial LAD with a very short stump. (B) Left anterior oblique caudal view showing wiring of the LAD. (C) Left anterior oblique caudal view showing successful angioplasty to proximal LAD..2.3
Patient 2 Angioplasty
(A) Left anterior oblique view demonstrating occluded proximal RCA. (B) A nonselective image, followed by aortogram confirming the absence of the left coronary artery from the left aortic sinus and origin from the right coronary sinus. (C) Posteroanterior caudal angiographic view showing selective guide engagement into anomalous left main from the right sinus of Valsalva, and distal collateral supply of the distal RCA. (D) Left anterior oblique cranial view of the RCA after successful wiring and predilatation. (E) Posteroanterior cranial view showing successful angioplasty to RCA.4.5.6.7.8
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Associated Data
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Supplementary Materials
Patient 1 Angioplasty
(A) Posteroanterior caudal view with the anomalous left main coronary artery from RCA with an occluded ostial LAD with a very short stump. (B) Left anterior oblique caudal view showing wiring of the LAD. (C) Left anterior oblique caudal view showing successful angioplasty to proximal LAD..2.3
Patient 2 Angioplasty
(A) Left anterior oblique view demonstrating occluded proximal RCA. (B) A nonselective image, followed by aortogram confirming the absence of the left coronary artery from the left aortic sinus and origin from the right coronary sinus. (C) Posteroanterior caudal angiographic view showing selective guide engagement into anomalous left main from the right sinus of Valsalva, and distal collateral supply of the distal RCA. (D) Left anterior oblique cranial view of the RCA after successful wiring and predilatation. (E) Posteroanterior cranial view showing successful angioplasty to RCA.4.5.6.7.8