Chest Pain Due to Anomalous Origin of the Left Circumflex Artery From the Opposite Sinus of Valsalva in a 70-Year-Old Woman

Introduction

Coronary artery anomalies are congenital developmental abnormalities of the coronary arteries and are typically classified according to their origin, course, and termination [1]. Among these categories, anomalies of origin are most common [2]. They generally involve abnormal origins of the 3 major coronary arteries. In the normal population, the right coronary artery (RCA) and left main coronary artery arise from the sinuses of Valsalva at the aortic root: the RCA originates from the right coronary sinus and courses within the right atrioventricular groove, whereas the left main coronary artery arises from the left coronary sinus, passes behind the pulmonary artery, and then divides into the left anterior descending artery and left circumflex (LCx) artery [3].

Anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) is the most common clinically significant coronary anomaly [4]. Population-based studies have revealed a prevalence of 1.5% to 6.3% among patients undergoing coronary angiography [4]. However, this population is subject to substantial selection bias, suggesting that the prevalence of ACAOS in the general population is lower.

Most patients with ACAOS are asymptomatic; only high-risk subtypes are associated with myocardial ischemia, arrhythmias, or sudden cardiac death, particularly in young adults and athletes [5]. Anomalous vessels can follow malignant courses, such as interarterial or intramural pathways, or arise from slit-like ostia; these factors increase the risk of ischemia during exertion [6,7].

Anomalous origin of the LCx from the right sinus of Valsalva is a rare subtype of ACAOS. This variant is often asymptomatic and typically becomes clinically apparent only in later adulthood, presenting with chest pain or being detected incidentally [8,9]. Diagnosis requires coronary computed tomographic angiography (CTA) and coronary angiography to define the anatomy and guide management [10]. Treatment is individualized according to symptoms, myocardial ischemia, and anatomical risk features [11,12]. This report describes an older woman who presented with chest pain as the first clinical manifestation of ACAOS [13–15].

Case Report

A 70-year-old woman was admitted with a 5-day history of persistent, worsening precordial chest tightness that was unrelieved by rest. She also reported a 20-day history of recurrent dizziness; each episode lasted approximately 30 minutes and resolved with rest. During these episodes, her blood pressure reached 200/100 mm Hg. She had no history of diabetes mellitus or smoking. Previous treatment with nifedipine and atorvastatin improved her dizziness but worsened the chest tightness.

On admission, physical examination revealed a blood pressure of 150/90 mm Hg and a heart rate of 80 bpm. Electrocardiography showed isolated QT interval prolongation without ST-T changes suggestive of myocardial ischemia (Figure 1). Transthoracic echocardiography demonstrated mild mitral and tricuspid regurgitation, with a left ventricular ejection fraction of 65% and preserved global function (Figure 2). Chest radiography revealed no cardiomegaly, normal pulmonary vascular markings, and no acute pulmonary infiltrates (Figure 3). Laboratory testing revealed mild hypokalemia (3.1 mmol/L), which was corrected medically.

After an insufficient response to medical therapy, coronary angiography was performed with informed consent. Coronary angiography demonstrated right coronary dominance (Figure 4A, 4B). The left main coronary artery was absent. The LCx artery originated independently from the right sinus of Valsalva and coursed between the aortic root and pulmonary vein root, without high-risk features such as an acute takeoff angle or slit-like ostium. Clinically significant stenosis (up to 85%) was present in the left anterior descending artery, LCx artery, and mid-to-distal RCA.

After shared decision-making with the patient and her family, optical coherence tomography, percutaneous transluminal coronary angioplasty, and percutaneous coronary intervention of the RCA were successfully performed. Postoperative distal RCA flow was TIMI grade 3 (complete perfusion) (Figure 4C), and the chest tightness resolved. Dual antiplatelet therapy (aspirin plus ticagrelor) and optimized antihypertensive treatment were continued. At the 1-month follow-up, the patient remained free of chest pain, and her blood pressure was controlled at approximately 140/75 mm Hg.

Discussion

Whether anomalous coronary arteries in patients with ACAOS are linked to an increased risk of atherosclerosis remains controversial. Early matched controlled studies by Click et al suggested that anomalous circumflex arteries had a higher prevalence of significant stenosis than normal coronary arteries (P = 0.02) [16]. Sidhu et al also reported that moderate-to-severe coronary artery disease was more common in anomalous vessels than in normal vessels (49.12% vs 33.21%, P = 0.003) [17]. However, Jiang et al found no significant difference in stenosis severity between anomalous and normal arteries (P = 0.8), although patients with anomalous circumflex arteries were older at diagnosis and had more severe overall coronary stenosis (odds ratio = 2.7, 95% confidence interval: 2.2–3.4, P < 0.0001) [18]. Similarly, Şahin et al found greater atherosclerotic involvement in anomalous coronary arteries (P = 0.005), but no significant differences in atherosclerotic burden according to anomaly subtype (P = 0.220) [19]. Overall, current evidence remains inconsistent. Large-scale, well-controlled studies are needed to determine whether anomalous circumflex arteries predispose patients to atherosclerosis after adjustment for age, sex, cardiovascular risk factors, and selection bias in angiographic or coronary CTA cohorts.

This case shows that ACAOS can initially present with chest pain in older patients and may pose specific challenges for coronary angiography and intervention. Our patient had an anomalous LCx artery originating from the right sinus of Valsalva with 3 separate coronary ostia, an unusual subtype that is rarely symptomatic before older age. Unlike previous reports of ACAOS in younger individuals, this case is notable because symptoms first developed at 70 years of age, consistent with reports that some ACAOS variants remain clinically silent until late adulthood [20]. Similar to the case reported by Rigatelli and colleagues [20], our patient lacked high-risk anatomical features, such as interarterial or intramural courses, which may have contributed to the delayed clinical presentation.

ACAOS complicates coronary angiography, increasing procedural difficulty, duration, and contrast use [20,21]. Preprocedural coronary CTA can delineate the coronary anatomy and reduce technical difficulty during intervention [10]. This was a key consideration in the present case. Percutaneous coronary intervention instead of surgery was justified given the absence of high-risk anatomical features and presence of focal atherosclerotic disease. Current expert recommendations support individualized management, with percutaneous coronary intervention favored for stable obstructive disease and surgical unroofing or reimplantation reserved for patients with high-risk anatomy or inducible ischemia [11,12]. This case highlights the importance of considering ACAOS in older patients with unexplained chest pain and emphasizes that accurate anatomical characterization is essential for safe and successful management.

Conclusions

This case represents an exceedingly rare presentation of ACAOS in an older woman with chest pain caused by an anomalous LCx artery arising from the right sinus of Valsalva in the setting of atherosclerotic coronary artery disease. Symptomatic presentation may occur late in life, even in the absence of high-risk anatomical features. Preoperative coronary CTA can improve procedural success by accurately delineating coronary anatomy. Management should be individualized according to the patient’s symptoms and anatomical characteristics.