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Anomalous Coronary Arteries

Rationale and Objectives

The study aimed to evaluate the association of a coronary interarterial course (IAC) with adverse outcomes in adults and to assess the benefit of coronary artery bypass grafting (CABG) in this population.

Materials and Methods

A retrospective review identified 172 adults over 40 years of age with an anomalous coronary artery origin from the opposite coronary sinus, including 79 patients with an IAC. Hard outcomes (myocardial infarction or cardiac death) and soft outcomes (persistent angina) were evaluated, with a mean follow-up time of 5 years.

Results

Although the frequency of coronary artery disease (CAD) was lower among patients with an IAC (43% vs 59%; P < 0.05), the frequency of CABG was higher among those with an IAC (21/79 = 26% vs 16/93 = 17%; P = 0.15). Hard outcomes were equally frequent among IAC patients (12/79 = 15%) compared to those without an IAC (16/93 = 17%) ( P = 0.7), and among patients with a right IAC (11/68 = 16%) compared to those with a left IAC (1/11 = 9%) ( P = 0.56). Among patients with an IAC, the frequency of hard outcomes was similar with CABG (4/21 = 19%) versus without CABG (8/59 = 14%) ( P = 0.55); the frequency of soft events was also similar with CABG (9/21 = 43%) versus without CABG (18/59 = 31%) ( P = 0.3). On multivariate analysis, the extent of coronary disease was the only significant predictor of hard or soft outcome (the presence of IAC, CABG, and left vs right IAC were not significant; P > 0.3).

Conclusions

The frequency of hard and soft outcomes among adult patients with anomalous coronary arteries is not significantly related to the presence of an IAC. No benefit was documented from the CABG treatment of an IAC in the absence of CAD.

Introduction

The incidence of anomalies of the coronary arteries is low, comprising up to 1.3% of the population in angiographic studies. Of these, less than half involve anomalous origins of the coronary arteries arising from the opposite coronary sinus (ACAOS). The most common anomalous coronary artery is a circumflex artery arising from the right coronary sinus or with a common origin with the right coronary artery (RCA). An anomalous circumflex artery always follows a “benign” course posterior to the aortic root. The more clinically significant anomalies include an anomalous RCA arising from the left coronary sinus, which is more common than an anomalous left main coronary artery (LMCA) arising from the right coronary sinus. Anomalous right and left main coronary arteries may follow either a “benign” course around the aorta and pulmonary artery, or a “malignant” interarterial course (IAC) between the aorta and pulmonary artery .

An anomalous coronary artery with an IAC is a recognized cause of sudden death in the pediatric population and young adults . Since the introduction of noninvasive coronary imaging with coronary computed tomography angiography (cCTA), these anomalies have been increasingly found in the adult population presenting to emergency rooms and doctor offices with symptoms of chest pain .

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Materials and Methods

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Figure 1, Anomalous right coronary artery with an interarterial course. The right coronary artery (RCA) (arrow) originates from the left sinus of Valsalva (*) and passes between the aortic root (Ao) and the right ventricular outflow tract (RVOT). The left main coronary artery is also visualized (arrowhead). (a) Volumetric rendering. (b) Angled axial maximum intensity projection image (MIP). The MIP is angled caudally to the right side to follow the interarterial course of the RCA, which follows that anterior margin of the aortic root below the sinotubular junction. (c) Coronal MIP again demonstrates the interarterial course of the RCA as it courses anterior to the aortic root.

Figure 2, Anomalous left main coronary artery with an interarterial course (septal variant). The left main coronary artery (LMCA) (arrow) originates at a right angle from the base of the RCA (arrowhead) at the right sinus of Valsalva (*), and passes between the aortic root (Ao) and the right ventricular outflow tract (RVOT). (a) Volumetric rendering. (b) Angled axial maximum intensity projection image (MIP). The MIP is angled caudally to the left side to follow the interarterial course of the LMCA, which follows that anterior margin of the aortic root down to the interventricular septum. (c) Coronal MIP again demonstrates the caudally directed interarterial course of the LMCA as it courses anterior to the aortic root.

Figure 3, Anomalous left main coronary artery with an interarterial course (septal variant). The left main coronary artery (LMCA) (arrow) demonstrates a widely patent origin from a common trunk with the right coronary artery (RCA) from the right sinus of Valsalva (*), and passes between the aortic root (Ao) and the right ventricular outflow tract (RVOT). The RCA is also visualized (arrowhead). (a) Volumetric rendering. (b) Angled axial maximum intensity projection image (MIP). The MIP is angled caudally to the left side to follow the interarterial course of the LMCA, which follows that anterior margin of the aortic root down to the interventricular septum. (c) Coronal MIP again demonstrates the caudally directed interarterial course of the LMCA as it courses anterior to the aortic root.

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Results

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Table 1

Patient Information for All ACAOS Patients with or without an IAC

All IAC No IAC ( n = 172) ( n = 79) ( n = 93) Demographics Age in years 63.8 ± 12.1 63.5 ± 12.4 64.1 ± 11.8 Male/Female 95/77 38/41 57/36 %Male 55.2 48.1 61.3 Anomalous artery Left main (LMCA) 22 (12.8%) 11 (13.9%) 11 (11.8%) Right (RCA) 79 (45.9%) 68 (86.1%) 11 (11.8%) Circumflex 71 (41.3%) 0 71 (76.3%) No. of patients with 0/1/2/3 diseased vessels 86/28/27/31 45/9/12/13 41/19/15/18 No. of patients with disease in anomalous vessel 60 (34.9%) 28 (35.4%) 32 (34.4%) Follow-up Average follow-up time (months) 59.3 ± 38.1 54.4 ± 39.2 63.5 ± 36.8 Number bypassed 37 (21.5%) 21 (26.6%) 16 (17.2%) Soft outcomes 52 (30.2%) 26 (32.9%) 26 (28.0%) Hard outcomes 28 (16.3%) 12 (15.2%) 16 (17.2%) Death (all cause) 20 (11.6%) 9 (11.4%) 11 (11.8%)

ACAOS, anomalous origin of the coronary artery arising from the opposite coronary sinus; IAC, interarterial course; LMCA, left main coronary artery; RCA, right coronary artery.

Table 2

Patient Information for ACAOS Patient Who Underwent Coronary Artery Bypass Grafting with or without an IAC

All IAC No IAC ( n = 37) ( n = 21) ( n = 16) Demographics Age in years 69.0 ± 13.4 66.8 ± 15.3 71.9 ± 9.9 Male/Female 26/11 13/8 13/3 %Male 70.3 61.9 81.3 Anomalous artery Left main (LMCA) 4 (10.8%) 4 (19.0%) 0 Right (RCA) 17 (45.9%) 17 (81.0%) 0 Circumflex 16 (43.2%) 0 16 (100%) No. of patients with 0/1/2/3 diseased vessels 8/2/9/18 8/1/4/8 0/1/5/10 No. of patients with disease in anomalous vessel 23 (62.2%) 11 (52.4%) 12 (75.0%) Follow-up Average follow-up time (months) 78.4 ± 39.9 79.1 ± 40.5 77.4 ± 40.4 Soft outcomes 15 (40.5%) 9 (42.9%) 6 (37.5%) Hard outcomes 7 (18.9%) 4 (19.0%) 3 (18.8%) Death (all cause) 5 (13.5%) 3 (14.3%) 2 (12.5%)

ACAOS, anomalous origin of the coronary artery arising from the opposite coronary sinus; IAC, interarterial course; LMCA, left main coronary artery; RCA, right coronary artery.

Table 3

Patient Information for All ACAOS Patients Managed Medically with or without an IAC

All IAC No IAC ( n = 135) ( n = 58) ( n = 77) Demographics Age in years 62.3 ± 11.4 62.4 ± 11.2 62.5 ± 11.6 Male/Female 69/66 25/33 44/33 %Male 51.1 43.1 57.1 Anomalous artery Left main (LMCA) 18 (13.3%) 7 (12.1%) 11 (14.3%) (all 7 septal variants) Right (RCA) 62 (45.9%) 51 (87.9%) 11 (14.3%) Circumflex 55 (40.7%) 0 55 (71.4%) No. of patients with 0/1/2/3 diseased vessels 77/26/18/14 37/8/8/5 40/18/10/9 No. of patients with disease in anomalous vessel 37 (27.4%) 17 (29.3%) 20 (26.0%) Follow-up Average follow-up time (months) 54.1 ± 36.0 45.4 ± 35.0 60.6 ± 35.6 Soft outcomes 37 (27.4%) 17 (29.3%) 20 (26.0%) Hard outcomes 21 (15.6%) 8 (13.8%) 13 (16.9%) Death (all cause) 15 (11.1%) 6 (10.3%) 9 (11.7%)

ACAOS, anomalous origin of the coronary artery arising from the opposite coronary sinus; IAC, interarterial course; LMCA, left main coronary artery; RCA, right coronary artery.

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Figure 4, Kaplan-Meier curve comparing overall survival in anomalous origin of the coronary artery arising from the opposite coronary sinus (ACAOS) patients with and without an interarterial course.

Figure 5, Kaplan-Meier curve comparing overall survival in anomalous origin of the coronary artery arising from the opposite coronary sinus (ACAOS) patients with an interarterial course treated medically to those treated with coronary artery bypass grafting.

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Table 4

Logistic Regression Analysis for Hard Outcomes Alone Using Single Variable and Multivariate Analysis

Odds Ratio Standard Error 95% Confidence Interval_P_ Value Single variable Extent of disease 1.67 0.29 1.19–2.34 0.003 IAC 0.86 0.36 0.38–1.95 0.72 Right IAC 0.99 0.42 0.43–2.26 0.98 Left IAC 0.50 0.53 0.06–4.04 0.51 CABG 1.27 0.61 0.49–3.26 0.62 Multivariate Extent of disease 1.82 0.36 1.23–2.68 0.002 IAC 0.99 0.43 0.42–2.31 0.98 CABG 0.59 0.33 0.20–1.76 0.34

CABG, coronary artery bypass grafting; IAC, interarterial course.

Table 5

Logistic Regression Analysis for Both Hard and Soft Outcomes Combined Using Single Variable and Multivariate Analysis

Odds Ratio Standard Error 95% Confidence Interval_P_ Value Single variable Extent of disease 1.37 0.19 1.04–1.80 0.02 IAC 1.26 0.42 0.65–2.43 0.48 Right IAC 1.48 0.50 0.77–2.86 0.24 Left IAC 0.49 0.39 0.10–2.37 0.38 CABG 1.81 0.70 0.85–3.85 0.13 Multivariate Extent of disease 1.34 0.21 0.99–1.83 0.06 IAC 1.33 0.46 0.68–2.61 0.41 CABG 1.21 0.53 0.51–2.87 0.66

CABG, coronary artery bypass grafting; IAC, interarterial course.

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Discussion

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Conclusions

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