Abbreviations
AAOCA, Anomalous aortic origin of a coronary artery
AL1, Amplatz Left 1
AOG, Aortography
CAA, Coronary artery anomaly
CAG, Coronary angiography
CCTA, Coronary computed tomography angiography
JL3.5, Judkins Left 3.5
JR4, Judkins Right 4
LAD, Left anterior descending artery
LCC, Left coronary cusp
MP, Multipurpose
NCC, Non-coronary cusp
RCA, Right coronary artery
RCC, Right coronary cusp
SCD, Sudden cardiac death
STJ, Sinotubular junction
INTRODUCTION
Congenital coronary artery anomalies (CAAs) are uncommon, with a reported prevalence of 0.3-5.64% in angiographic and autopsy studies. Clinical presentations are often nonspecific, ranging from chest pain and exertional dyspnea to syncope and ventricular arrhythmias.1
Certain anatomical variants, particularly those with interarterial or intramural courses, can predispose patients to dynamic coronary compression between the aorta and pulmonary artery, leading to myocardial ischemia.2 Additionally, high take-off coronary arteries — defined as coronary arteries originating above the sinotubular junction (STJ) of the aorta — are generally regarded as benign variants. However, no universally accepted cutoff for the vertical distance of origin has been established, and these anomalies may contribute to procedural challenges during cardiac catheterization.3
We report an elderly patient with an anomalous single coronary ostium arising above the STJ of the ascending aorta, outside the aortic sinuses of Valsalva. This case illustrates the diagnostic challenges, potential clinical risks, and technical difficulties encountered during coronary angiography (CAG) in older individuals with CAAs.
CASE
An 81-year-old Taiwanese man, a former smoker (60 pack-years, quit 5 years ago), was functionally independent. His medical history included hypertension, chronic obstructive pulmonary disease (GOLD stage 2B), treated with olodaterol and tiotropium. He recently experienced exertional angina, dyspnea on exertion, palpitations, and chest tightness.
Thallium-201 myocardial perfusion imaging showed a mild reversible perfusion defect of the left anterior descending artery (LAD) territory and moderate perfusion defect of the right coronary artery (RCA) territory. Medical therapy was initiated. A 24-hour Holter monitor revealed sinus rhythm with infrequent premature beats (< 1%).
Two months later, he developed progressive dyspnea over 3 days and presented to the emergency department. Initial vital signs: temperature 36.3 °C, blood pressure 232/80 mmHg, heart rate 81 bpm, respiratory rate 30/min, and oxygen saturation 94% on room air. Lung auscultation revealed wheezes and crackles. Chest radiography indicated pulmonary congestion. Electrocardiography demonstrated sinus rhythm with mild ST-segment depression in leads V4-V6, and N-terminal pro-B-type natriuretic peptide was elevated at 589 pg/mL with normal cardiac enzymes. Under a tentative diagnosis of acute pulmonary edema, the patient improved after intravenous furosemide and nitroglycerin. Transthoracic echocardiography revealed a dilated left atrium (44 mm), mildly dilated aortic root (41 mm), moderate aortic regurgitation, preserved left ventricular ejection fraction (64%), and estimated pulmonary artery pressure of 16 mmHg. Given the concordance between stress-induced ischemia and acute heart failure, invasive CAG was performed to exclude obstructive coronary artery disease as a precipitating cause.
A 5F Judkins Right 4 (JR4) catheter was introduced to engage the right coronary cusp (RCC), but despite multiple non-selective injections in the RCC and non-coronary cusp (NCC) under various projections, the right coronary ostium could not be identified (Figure 1A). A 6F Judkins Left 3.5 (JL3.5) guiding catheter was then positioned at the left coronary cusp (LCC); however, repeated probing and contrast injections failed to visualize the left main ostium (Figure 1B-D). A CHAMP catheter (Medtronic) with a wide, rounded, soft "J"-shaped distal curve was subsequently used to search for an anomalous coronary origin, but engagement was unsuccessful, raising suspicion of a congenital anomaly, prompting aortography (AOG).
Figure 1.

(A) In the left anterior oblique (LAO) 50° projection, a 5F Judkins Right 4 (JR4) diagnostic catheter was positioned at the right coronary cusp region. Multiple non-selective contrast injections were performed, but no right coronary ostium could be identified. (B, C) A 6F Judkins Left 3.5 (JL3.5) guiding catheter was then advanced and positioned at the left coronary cusp (LCC). An additional angiographic view in the spider projection (LAO caudal 57°/20°) was obtained, yet the left main coronary ostium remained unvisualized. In the anteroposterior (AP) projection, the catheter tip was directed toward the area of aortic root calcification, and multiple non-selective contrast injections were performed; however, no left main ostium could be identified. (D) Another angiographic view in the spider projection (LAO caudal 57°/20°) again failed to demonstrate the left main coronary ostium. (E, F) Given the failure to identify the coronary ostium, aortography was performed, demonstrating moderate-to-severe aortic regurgitation and opacification extending caudally from the ascending aorta along the calcified coronary plane.
AOG revealed moderate-to-severe aortic regurgitation and contrast opacification extending caudally along the calcified coronary plane (Figure 1E-F). A 5F Amplatz Left 1 (AL1) catheter was advanced, and subsequent ascending aortic injections revealed a single downward-facing coronary ostium originating above the STJ, supplying the LAD, left circumflex artery, and RCA (Figure 2A-E). Multi-view angiography confirmed severe coronary calcification but no significant stenosis. Retrospective review of prior non-contrast chest computed tomography suggested an interarterial course of the RCA from the left side tracing the course of the calcified coronary artery (Figure 2G-H). To facilitate anatomical visualization, a schematic illustration depicting the single high take-off coronary ostium and the suspected interarterial RCA course is provided (Figure 2F).
Figure 2.
(A, B) An attempt was made to identify the coronary ostium from the ascending aorta. A single, downward-facing ostium was visualized arising from the ascending aorta, giving rise to the left anterior descending artery (LAD), left circumflex artery (LCX), and right coronary artery (RCA). The right anterior oblique (RAO) 10° caudal 12° (A) and RAO 20° cranial 30° (B) projections clearly demonstrate this anomalous single coronary ostium originating outside the aortic cusps. (C) Alternative projection: RAO 20° caudal 20° demonstrating the LAD and LCX. (D) Alternative projection: LAO 50° cranial 10° demonstrating the RCA. (E) Alternative projection: LAO 50° caudal 0° demonstrating the distal RCA. (F) Schematic anatomical illustration demonstrating three coronary arteries arising from a single high take-off ostium above the sinotubular junction, with the calcified RCA suspected to course between the ascending aorta and pulmonary artery (PA). (G, H) Marked atherosclerotic mural calcifications were observed in both the right and left coronary arteries. Based on the calcification trajectory, the RCA was suspected to course between the aorta and the PA. STJ, sinotubular junction.
Given the patient’s advanced age and limited mobility, the family elected conservative management and declined further coronary computed tomography angiography (CCTA) evaluation. Upon discharge, the patient was prescribed aspirin, antihypertensive agents, a beta-blocker, and nitrates to reduce cardiovascular risk and relieve angina. During regular outpatient follow-up, the patient remained clinically stable with good symptom control. This anatomical configuration, featuring a single coronary ostium with a high take-off from the ascending aorta, has infrequently been described.
DISCUSSION
This 81-year-old man with multiple cardiac comorbidities presented with progressive dyspnea and chest tightness. Inability to engage the coronary ostium prompted further imaging, which revealed an unusual combination of anomalies — a single coronary ostium located above the aortic sinuses of Valsalva with a suspected interarterial RCA course. The absence of distinguishing symptoms underscores the diagnostic challenge of identifying congenital CAAs in elderly patients with competing cardiovascular conditions.
Coronary artery anomalies are uncommon in the general population but have been associated with sudden cardiac death (SCD) in young athletes, informing current guideline-based risk stratification.4,5 However, these data derive predominantly from younger cohorts, and their applicability to older adults remains uncertain.6 High take-off or anomalous coronary origins may result in difficulty achieving selective ostial engagement, prolonged procedural time, and increased contrast exposure.7
Beyond procedural difficulties in the catheterization lab, recognizing high take-off coronary arteries is paramount for surgical safety. During cardiac surgery, these anomalies pose significant technical risks, including inadvertent injury, transection, or accidental clamping during aortotomy, cross-clamping, or valve procedures, particularly when unrecognized preoperatively. Furthermore, an abnormal ostial location may compromise retrograde or antegrade cardioplegia delivery, potentially resulting in inadequate myocardial protection. Additional concerns include coronary compression, distortion, or suture impingement, especially during prosthetic valve implantation or transcatheter interventions. These considerations underscore the importance of precise anatomical identification before any invasive or surgical management.8
The prevalence of anomalous aortic origin of a coronary artery (AAOCA) ranges from 0.14% to 2.16% in contemporary imaging studies.4 High-risk anatomical features related to SCD include interarterial course, intramural segment, slit-like ostium, acute take-off angle, proximal vessel hypoplasia, and dynamic compression between the aorta and pulmonary artery — factors that are particularly concerning in physically active individuals.2 In contrast to younger cohorts, the natural history of AAOCA in older adults appears relatively benign, with lower observed rates of SCD, although the mechanisms underlying this age-related attenuation of risk remain unclear.6,9
In this elderly patient, the anomaly was suspected only after repeated difficulty in engaging the coronary ostium during invasive angiography. A systematic engagement strategy is essential when an anomalous coronary origin is suspected. In this case, after initial attempts with a diagnostic JR4 catheter failed to identify the right coronary ostium in its usual locations within the RCC and NCC, an anomalous origin from the LCC was suspected. Consequently, we switched to a JL catheter to explore the left sinus; however, it failed to engage or visualize an opening in the standard positions. To better approach a potentially anteriorly directed ostium — which is often difficult to reach with a JL curve — a CHAMP catheter was utilized for a more comprehensive search. Following these unsuccessful attempts with standard and specialized curves, we proceeded with an AOG to avoid excessive contrast consumption. This targeted approach ultimately provided the critical clue, revealing a high take-off origin of the coronary artery.
Prolonged attempts to locate an anomalous ostium increase radiation exposure and contrast load — a concern of particular relevance in elderly patients with underlying renal dysfunction. Early transition to CCTA may provide safer anatomical clarification in hemodynamically stable patients.
A key limitation is the absence of CCTA to definitively characterize the RCA course; the suspected interarterial trajectory was inferred from calcification patterns on non-contrast computed tomography.
Management of AAOCA and isolated high take-off coronary arteries should be individualized based on anatomical characteristics, ischemic burden, symptom profile, and patient age. Surgical intervention may be considered in symptomatic patients with high-risk anatomical features or documented ischemia, particularly in younger individuals. In the absence of clear ischemia, conservative management with lifestyle modification and optimized medical therapy is appropriate.1,10 This case identifies "high take-off" coronary arteries as a critical cause of failed ostial engagement. To optimize outcomes, clinicians should adopt a structured engagement strategy: if standard JR4 or JL3.5 catheters fail, perform early AOG to localize the anomalous origin. Subsequently, switching to specialized catheters such as AL1 or multipurpose (MP) can facilitate selective cannulation, thereby reducing unnecessary manipulation, contrast load, and radiation exposure. Ultimately, management must be individualized based on specific anatomical findings, functional ischemia, and patient-specific risk profiles.
LEARNING POINTS
When selective engagement fails within the expected sinus, clinicians should adopt a structured strategy: performing early AOG to localize potential high take-off ostia. Switching to specialized catheters, such as Amplatz Left or MP, may facilitate coaxial alignment with anomalous origins above the STJ. If engagement remains unsuccessful, early termination of the invasive procedure in favor of CCTA is recommended. This approach prevents excessive vessel manipulation and minimizes both contrast consumption and radiation exposure.
DECLARATION OF CONFLICT OF INTEREST
All authors declare no conflict of interest.
Acknowledgments
None.
FUNDING
No funding was required for this manuscript.
REFERENCES
- 1.Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2019;73:1494–1563. doi: 10.1016/j.jacc.2018.08.1028. [DOI] [PubMed] [Google Scholar]
- 2.Gentile F, Castiglione V, De Caterina R. Coronary artery anomalies. Circulation. 2021;144:983–996. doi: 10.1161/CIRCULATIONAHA.121.055347. [DOI] [PubMed] [Google Scholar]
- 3.Loukas M, Andall RG, Khan AZ, et al. The clinical anatomy of high take-off coronary arteries. Clin Anat. 2016;29:408–419. doi: 10.1002/ca.22664. [DOI] [PubMed] [Google Scholar]
- 4.Cheezum MK, Liberthson RR, Shah NR, et al. Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 2017;69:1592–1608. doi: 10.1016/j.jacc.2017.01.031. [DOI] [PubMed] [Google Scholar]
- 5.Harmon KG, Asif IM, Maleszewski JJ, et al. Incidence, cause, and comparative frequency of sudden cardiac death in National Collegiate Athletic Association Athletes: a decade in review. Circulation. 2015;132:10–19. doi: 10.1161/CIRCULATIONAHA.115.015431. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.Gräni C, Benz DC, Steffen DA, et al. Outcome in middle-aged individuals with anomalous origin of the coronary artery from the opposite sinus: a matched cohort study. Eur Heart J. 2017;38:2009–2016. doi: 10.1093/eurheartj/ehx046. [DOI] [PubMed] [Google Scholar]
- 7.Fretay XHD, Boudvillain O, Koutsoukis A, et al. Catheterization techniques for anomalous aortic origin of coronary arteries. Catheter Cardiovasc Interv. 2025;105:825–837. doi: 10.1002/ccd.31391. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Gaudino M, Di Franco A, Arbustini E, et al. Management of adults with anomalous aortic origin of the coronary arteries: state-of-the-art review. J Am Coll Cardiol. 2023;82:2034–2053. doi: 10.1016/j.jacc.2023.08.012. [DOI] [PubMed] [Google Scholar]
- 9.Amado J, Carvalho M, Ferreira W, et al. Coronary arteries anomalous aortic origin on a computed tomography angiography population: prevalence, characteristics and clinical impact. Int J Cardiovasc Imaging. 2016;32:983–990. doi: 10.1007/s10554-016-0849-5. [DOI] [PubMed] [Google Scholar]
- 10.Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42:563–645. doi: 10.1093/eurheartj/ehaa554. [DOI] [PubMed] [Google Scholar]

