ORIGINAL ARTICLE

Single Coronary Artery Anomaly: Classification and EvaluationUsing Multidetector Computed Tomography and MagneticResonance Angiography

Soma Mandal • Sameh S. Tadros • Shephaly Soni •

Shobhit Madan

Received: 14 May 2013 / Accepted: 14 September 2013 / Published online: 6 October 2013

� Springer Science+Business Media New York 2013

Abstract The aim of this study was to use multidetector

computed tomography (MDCT) and magnetic resonance

(MRA) angiography to illustrate the classification and clinical

characteristics of single coronary artery anomaly (SCAA).

Retrospective evaluation of 22 adult and pediatric patients

with SCAA by way of a medical archiving system was per-

formed between June 2001 and August 2012. Imaging

modalities used for coronary artery evaluation included MRA

and MDCT angiography. Of the 22 patients, the majority

(n = 8; 36 %) showed an interarterial course, the subtype

having the worst prognosis. The retroaortic course (n = 3;

14 %) and course anterior to the pulmonary trunk (n = 3;

14 %) were the next most frequent patterns. Additional types

(n = 8; 36 %) included the following: L-I, R-III, septal, and

combined. Four patients (18 %) showed atherosclerotic

involvement. SCAA anomaly was diagnosed as an incidental

finding in the majority of patients evaluated initially for car-

diovascular diseases (n = 19; 86 %). Two patients (9 %)

required surgical interventions solely for their anomaly. Nine

patients (41 %) were found to have coexisting congenital

heart disease. Although conventional catheter angiography is

responsible for the current classification of SCAA, advanced

imaging modalities are useful in the evaluation of morpho-

logical and clinical characteristics of single coronary arteries.

Keywords Multidetector computed tomography �Magnetic resonance imaging � Angiography � Single

coronary artery anomaly � Congenital heart disease

Introduction

Single coronary artery anomaly (SCAA), a rare condition in

which the right coronary artery (RCA) or left coronary artery

(LCA) is absent from its respective coronary sinus of Valsalva,

has been discovered at autopsy for[250 years. First identified

posthumously in 1699 by Fantoni [13], SCAA was only

identified antemortem in 1967 through conventional catheter

angiography [14]. Because this condition presents with vari-

ous morphological variations, the development of classifica-

tion schemas has been attempted since the 1950s through

largely postmortem analysis [24, 32]. The main classification

system used today was developed in 1979 from the work of

Lipton et al. [19] using catheter angiography. Depending on

whether the motivation was functional or anatomical, multiple

schemas have been developed [1, 4, 7, 18, 27, 28, 30, 32, 34,

37]; however, Lipton et al.’s schema, as modified by Yama-

naka and Hobbs [38] in the 1990, has proven to be widely

adopted. Technology in modern medicine has allowed us to

visualize coronary vasculature, especially the proximal course

of the coronary artery, with superior sensitivity and specificity,

thus allowing for concrete classification evaluation of mor-

phological characteristics. In this study, we illustrate the

classification of SCAA with advanced, noninvasive imaging

modalities, such as magnetic resonance (MRA) and multide-

tector computed tomography (MDCT) angiography. Further-

more, we explore clinical features of SCAA subtypes

regarding (1) the manner in which they are typically diagnosed

and (2) the associated congenital and arterial pathologies.

SCAA Classification

SCAA incidence ranges from 0.24 to 0.66 % [11], but the

pretest probability of developing the anomaly is unknown.

S. Mandal � S. S. Tadros � S. Soni � S. Madan (&)

Department of Radiology, Cardiac Imaging, Pediatric Imaging

Research Center, Children’s Hospital of Pittsburgh of University

of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh,

PA 15224, USA

e-mail: madans@upmc.edu; shobhitmd@gmail.com

123

Pediatr Cardiol (2014) 35:441–449

DOI 10.1007/s00246-013-0798-x

It is diagnosed based on two criteria [2]: (1) the presence

of a single ostium in one coronary sinus combined with

the absence of an ostium in the opposite sinus and (2) lack

of origination of another coronary artery from an ectopic

site. Lipton et al.’s anatomic classification concerns the

origin (whether the vessel originates from the left [L] or

right [R] coronary sinus) and course of the aberrant vessel.

As seen in Fig. 1, the main types of SCAA include the

following: R-I, L-I, R-II-A/B/P, L-II-A/B/P, and R-III

along with mixed (M), septal (S), and combined (C),

which are applicable to either L or R types. Specifically,

group I (R-I, L-I) refers to a solitary vessel arising from

either the left or right coronary cusp with terminating left

or right branches; for example, the LCA would be

responsible for yielding right terminal branches. Group II

(R-II, L-II) is divided into three types depending on the

relationship of the aberrant vessel to the great vessels.

R-II-A designates both the RCA and LCA emerging from

the right coronary sinus with the LCA coursing anterior to

the pulmonary trunk; likewise, L-II-A designates the

opposite. R-II-B characterizes both the RCA and the LCA

originating from the right coronary sinus with the LCA

traveling in between the aorta and pulmonary trunk; L-II-

B characterizes the opposite. The final type within group II

is R-II-P, which characterizes the LCA and RCA origi-

nating from the right coronary sinus with the LCA trav-

eling posterior to the aorta; L-II-P characterizes the

opposite. R-III defines an absent LCA with the left ante-

rior descending and circumflex arteries arising from the

common trunk originating from right coronary cusp. Fig-

ure 1 also shows adjustments to these types: M types are

indicated when either the RCA or LCA branches from the

other versus branching at the site of the single ostium

shortly after the emergence from a single ostium. S types

characterize more distal behavior in which the LCA or

RCA, after emerging from a single coronary artery or

cusp, follows a transseptal course. C types characterize

anomalies in which multiple courses exist: Fig. 1 shows

the combination of R-II-P and S subtype under the C

subtype. Figure 2 further shows potential paths that can be

taken by a type II anomaly originating from the right

coronary cusp [19].

Fig. 1 Classification of SCAA in 3D and axial view as characterized by Lipton et al. [19] and Yamanaka and Hobbs [38]. A aorta, PA pulmonary

artery, cX circumflex artery, LAD left anterior descending, M mixed, C combined, S septal

442 Pediatr Cardiol (2014) 35:441–449

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Methods

Study Population

Three hundred two consecutive patients presenting with

nonspecific symptoms (e.g., angina, arrhythmias) and sus-

pected of coronary artery aberrations based on initial

evaluation using echocardiography or catheter angiography

were referred for further evaluation with MDCT from

[130,000 records in a medical archiving system from a

single institution between June 2001 and August 2012. Of

the 302 records, 87 patients showed some form of coronary

artery anomaly. Patients with SCAA specifically were

studied under Institutional Review Board approval. A total

of 22 patients were identified to have SCAA, which was

diagnosed by way of a combination of electrocardiograph-

ically gated (ECG-gated) CT angiography, and/or cardiac

MRA, and/or echocardiography, and/or catheter angiogra-

phy. Fourteen (64 %) children (mean age 11 years) and 8

(36 %) adults (mean age 54 years) comprised a cohort with

a blood supply that was predominantly (78 %) right domi-

nant (with the posterior descending branching from the

RCA). Patient demographics, presenting symptoms, and

results of nuclear medicine/stress tests are listed in Table 1.

Clinical data were obtained from patients’ medical and

imaging records.

Imaging Techniques

Several imaging techniques exist in the characterization of

coronary artery anomalies, including transthoracic echo-

cardiography, catheter coronary angiography, intravascular

ultrasound, MRA, and MDCT angiography [3]. In this study,

the use of MDCT for 18 patients and contrast-enhanced (or

nonenhanced) MRA for four patients was considered opti-

mal for the evaluation of SCAA. Of the 22 patients, 10

(n = 5 children, n = 5 adults) underwent catheter coronary

angiography. All patients underwent echocardiography

before further imaging. MDCT angiography with prospec-

tive and retrospective ECG gating used an average radiation

dose of 2.5 and 8.5 mSv, respectively. Radiation dose values

were calculated using the dose-length product. For patients

in whom MDCT angiography is contraindicated (i.e.

tachycardia, radiation exposure concerns), MRA is the next

modality used in this institution. Curved planar reformats,

three-dimensional (3D) volume rendering, endoluminal

imaging, and double-oblique plane methods were used for

single ostial visualization and general morphological eval-

uation (see Fig. 3a).

MDCT Angiography

Image acquisition patients were scanned with a 64-slice

MDCT scanner (GE Medical Systems, Milwaukee, WI). A

bolus of 3 mL/kg of Isovue 370 (Bracco Diagnostics,

Princeton, NJ) was injected intravenously at rate of 5.0 and

4.0 cc/s for adults and children, respectively. A target heart

rate \65 beats per minute was achieved by administration

of beta blockers or calcium-channel blockers to provide a

stable heart rate and avoid motion artifacts during imaging.

For image acquisition of higher heart rates, burst and burst-

plus techniques were used. Respiration was suspended for

10 s during image acquisition, and imaging was performed

using a retrospective or prospective ECG-gated MDCT

protocol depending on subject heart rate at the time of

imaging for dose modulation.

MR Imaging and Angiography

Image acquisition MR imaging was performed using a 1.5-

Tesla MR scanner (CV/I; GE Medical Systems). Steady-

state free-precision pulse sequence with breath hold in

axial, sagittal, and coronal planes at the level of aortic root

was used for cinematographic coronary artery imaging. 3D

contrast-enhanced MRA was performed with intravenous

administration of gadolinium contrast agent, 0.15 mmol/kg

(Multihance; Bracco), and 3 mm–thick slices at 1.5-mm

spacing.

Results

Of the 22 patients with SCAA (Table 2), the majority

(n = 8, 36 %) showed an interarterial course or type II-B

anomaly (Fig. 4). Type II-P (n = 3, 14 %) patients showed

the aberrant vessel coursing posterior to the aorta (Fig. 5).

Three cases of R-II-A (14 %) were diagnosed (Fig. 6) with

Fig. 2 Example of subtypes of R-II SCAA. The origin of the single

artery in an R-II classification occurs at the left coronary sinus of

Valsalva and can travel anteriorly (R-II-A), interarterially (R-II-B), or

retroaortically (R-II-P). PA pulmonary artery, RCC right coronary

cusp of aorta, LCC left coronary cusp of aorta, NCC noncoronary cusp

of aorta

Pediatr Cardiol (2014) 35:441–449 443

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two of these cases showing a mixed trunk (Fig. 7). Other

subtypes (n = 8, 36 %) represented included L-I, S

(Figs. 8c, 9), and R-III subtype (Fig. 10). Nine patients

(41 %) showed a coexisting congenital heart disease

(CHD) (Table 2). Within our cohort, 4 (18 %) pediatric

patients showed atherosclerosis (Fig. 3b), and 50 % of the

adults assessed showed atherosclerosis. Half of the patients

(n = 11) were managed with medications tailored to their

presenting symptoms (e.g., antiarrhythmics) or were sent

home with plans to monitor the progression of cardiac

symptoms. Although nine patients were managed surgi-

cally for associated CHD, only two patients required

Table 1 Patient demographics and symptoms and nuclear medicine/stress test results

Patient

no.

Sex Age

(year)

Height

(in)

Weight

(lb)

Body surface

area (m2)

Blood flow

dominance

Reasons for

CT/MR imagingaNuclear medicine/

stress test results

1 F 0.5 24.4 11.0 0.3 L 9 NP

2 M 7 51.2 59.4 1.0 R 6 Normal

3 F 7 52.8 77.0 1.1 R 5 NP

4 F 7 48.8 39.6 0.8 R 5 NP

5 F 8 52.76 61.6 1.0 R 2 NP

6 M 10 58.8 123.2 1.5 R 10 Normal

7 M 12 63.6 105.6 1.5 L 11 NP

8 F 14 57.6 121.0 1.5 L 3 NP

9 M 14 60.8 121.0 1.5 R 4, 7 SA node disease

10 F 14 67.2 167.2 1.9 L 1, 2, 3, 4 No ischemia

11 F 15 66.8 103.4 1.5 R 4 BBB

12 M 16 66 127.6 1.6 R 5 No ischemia

13 M 16 71.6 222.2 2.0 R 12 NP

14 F 17 64 112.2 1.5 R 1, 3, 4 Sinus rhythm, BBB

15 M 23 71.2 189.2 2.0 L 1, 3, 4 PVB

16 F 40 65.2 158.4 1.8 R 3 Antero-apical ischemia

17 M 53 66.4 143.0 1.8 R 3 NP

18 F 57 62 150.0 1.7 R 3 NP

19 M 58 74 209.0 2.2 R 3, 4, 8 Mild inferior ischemia

20 M 62 71 216.0 2.2 R 3 Normal

21 M 65 69.6 212.5 2.2 R 13 NP

22 M 71 69.2 161.9 1.9 R 3 ST depression, inferior

ischemia

NP not performed, SA sinoatrial, BBB bundle branch block, PVB premature ventricular beatsa Symptoms key: 1 difficulty breathing, 2 syncope, 3 chest pain, 4 arrhythmia, 5 suspected coronary artery aberration from echocardiographic

evaluation, 6 status post-heart transplant, 7 cardiac arrest, 8 angina, 9 cyanosis, 10 family history of early cardiac death, 11 history of congenital

heart disease, 12 poor cardiac function, 13 history of coronary artery disease and anomalous coronary courses

Fig. 3 Techniques used in the preliminary evaluation of SCAA.

a Endoluminal view confirms the presence of a single ostium.

b Curved planar reformat shows atherosclerotic plaques in an adult

patient with SCAA. c Volume-rendered imaging showing the

common origin and the rest of the vascular tree. d Catheter

angiography showing SCAA

444 Pediatr Cardiol (2014) 35:441–449

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surgical intervention, one each of solely coronary unroo-

fing and bypass grafting, for their SCAA. Other patients

underwent surgery and stent placement for either CHD or

atherosclerosis. Of those patients who were medically

managed, classification types included the following: II-P

(n = 3), II-B (n = 2), and a mix of other subtypes (n = 6).

Of those surgically managed for associated conditions,

subtypes were II-B (n = 3) and a mix of other subtypes

Table 2 SCAA classification, morphological dimensions, and associated conditions and management

Patient

no.

Classification Associated conditions Cardiac cycle

phase (%)

CT dose

(mSv)

Atherosclerotic

involvement

Patient management

1 LII-P TOF, PA, HAA, COA 50 N/A None Routine follow-up

2 LII-B Isolated 75 1.5 None Routine follow-up

3 RII-B Functional murmur 84 2.1 None Routine follow-up

4 RIII Murmur 50 7.3 None OI

5 RII-S DCRV, VSD 80 5.9 None Surgical management, DCRV

6 RII-C Isolated 50 7.6 None Routine follow-up

7 LII-P Heterotaxy syndrome, COA,

BAV, VSD

75 8.2 None Routine follow-up

8 RIII TS, BAV, DAA MR MR None Routine follow-up

9 L-I DORV, DAR 75 4.1 None Surgical, dual-chamber pacemaker

10 LII-B Isolated 80 3.4 None Surgical-RCA unroofing

11 NA DORV, AA, VSD, DVAA, PCS MR MR None Routine follow-up

12 LII-B Isolated 75 13.5 None Surgical, coronary unroofing

13 RII-A TOF, VSD, PS MR MR None Surgical-PV replacement

14 RII-P DORV, VSD, BSCV MR MR None Routine follow-up

15 L-I SF, CSS 75 5.7 None Routine follow-up

16 RII-B Isolated 75 N/A None Surgical, CA bypass grafting

17 RII-B CAD, severe stenosis 78 7.5 Severe Surgical, CA revascularization

18 RII-B CAD, moderate stenosis 75 19.1 None OI

19 RII-A-M Isolated 75 33.9 None Routine follow-up

20 RII-A-M CAD, moderate/severe stenosis 75 NA Severe Surgical-stent placement

21 RII-S CAD, nonobstructive atherosclerosis 75 6.4 Moderate Routine follow-up

22 LII-B CAD, mild stenosis 75 9.6 Severe Surgical, stenting of RCA

SCAD single coronary artery ostial diameter, SCAOA single coronary artery ostial area, NA not available or not able to delineate, OI outside

institution, CA coronary artery, PV pulmonary vein, TOF tetralogy of Fallot, PA pulmonary atresia, AA aortic atresia, HAA hypoplastic aortic

arch, COA coarctation of aorta, DCRV double-chamber right ventricle, VSD ventricular septal defect, BAV bicuspid aortic valve, DAA dilated

ascending aorta, DAR dilated aortic root, DORV double-outlet right ventricle, TS Turner’s syndrome, DVAA double-vessel aortic arch, PCS

pulmonic conduit stenosis, PS pulmonary stenosis, CSS congenital subaortic stenosis, SF septal fibroma, CAD coronary artery disease, BSCV

bilateral superior caval veins

Fig. 4 Type II-B or interarterial courses of SCAA. a Contrast-

enhanced CT axial image of type LII-B SCAA with the LCA coursing

between the aorta (A) and the pulmonary artery (PA). b Volume-

rendered image of the same patient. c Contrast-enhanced MDCT axial

oblique view of RII-B SCAA with the RCA traversing interarterially

Pediatr Cardiol (2014) 35:441–449 445

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(n = 6). High-risk indication for surgery in two patients

(9 %) was present for classification types L-II-B and R-II-

B, which involve interarterial courses originating from the

left and right coronary sinus, respectively. In terms of

discovery, SCAA was diagnosed as (1) an incidental

finding in a patient with a CHD (n = 8, 36 %), (2) an

incidental finding in a patient (n = 5, 23 %) without

symptoms attributable to a coronary anomaly, (3) a pos-

sible or likely culprit in a patient with ischemic or

arrhythmic symptoms (n = 1, 5 %), and (4) an incidental

finding in a patient with ischemic or arrhythmic symptoms

and significant atherosclerosis (n = 5, 23 %).

Discussion

The goals of this study were to use MDCT and MR angi-

ography to (1) illustrate the classification and (2) determine

the clinical characteristics and correlations of SCAAs.

Regarding the first aim, the illustration of SCAA classifi-

cation, this study is one of the largest characterized to date.

Two other large series are notable: Fifty-four SCAA cases

were identified from [126,000 patients undergoing coro-

nary angiography between 1960 and 1988 [38], and 33

SCAA cases were identified from 50,000 patients under-

going coronary angiography between 1973 and 1991 [11].

Although catheter angiography has been classically used in

the diagnosis of SCAA [14, 19, 24, 32, 38], this study used

MDCT and MR angiography as the optimal diagnostic

tools in coronary artery evaluation. Of the pediatric cohort

(n = 14), 10 (71 %) children were evaluated solely with

MDCT angiography without the use of MRA.

In addition, the clinical correlations of SCAA were

profiled in the context of (1) associated congenital and

arterial pathologies and (2) the manner in which the

anomalies are typically diagnosed. Specifically, the pre-

sence of atherosclerosis is supported by our cohort, but its

overall association with SCAA is still controversial [21,

26]. In addition, SCAA is associated with several CHDs,

including tetralogy of Fallot, coronary arteriovenous fis-

tula, bicuspid aortic valve, truncus arteriosus, ventricular

septal defect, patent ductus arteriosus, patent foramen

Fig. 5 CT of type II-P SCAA or single coronary arteries posterior to

the aorta. a LII-P–classified anomaly with the proximal course of the

RCA arching posterior to the aorta (A). b Another LII-P–classified

SCAA seen with both the LCA and RCA branching from the common

origin. c The distal course an LII-P–classified anomaly is seen with

the RCA running posterior to the aorta

Fig. 6 Contrast-enhanced MRA of the RII-A subtype. a Axial view

of the proximal course of the LCA branching from the aorta (A) and

travelling anterior to the pulmonary artery (PA). b Oblique reformat-

ted image of the proximal and distal course of the LCA moving

anteriorly to the PA

Fig. 7 MDCT angiography of

mixed trunks or type II-M.

a Curved planar reformatted

image showing the LCA

branching from the RCA.

b Oblique view showing LCA

branching sharply curving from

the RCA

446 Pediatr Cardiol (2014) 35:441–449

123

ovale, and transposition of the great vessels [24, 29, 36].

Further investigation is needed to determine the genetic

association or causation between SCAA and other con-

genital abnormalities.

In terms of the clinical diagnosis of SCAA, or our

second part aim, SCAA was usually discovered in the

following ways: (1) an incidental finding in a patient

(usually a child) with a CHD (majority of the cohort); (2)

an incidental finding in a patient without symptoms

attributable to a coronary anomaly (n = 5, 23 %), often a

child evaluated for a murmur or for nonanginal chest pain;

(3) a possible or likely culprit in a patient with ischemic or

arrhythmic symptoms (n = 1, 5 %); and (4) an incidental

finding in a patient (usually an older adult) with ischemic

or arrhythmic symptoms and significant atherosclerosis,

which was more likely to be the cause of the symptoms

(n = 5, 23 %).

In terms of the clinical relevance and management of

subtypes, SCAA is clinically most significant when the

anomalous course of the vessel is either interarterial or

when the aberrant vessel traverses between the aorta and

the pulmonary arterial trunk (Figs. 1 [type II-B], 2). These

Fig. 8 Contrast-enhanced MDCT angiography of RII-S subtypes.

a Short-axis view showing the proximal and transseptal distal course

of the LCA. b Oblique reformatted image of the proximal course of

the LCA inferior to the pulmonary trunk. c MDCT angiography

allows the distal transseptal course of LCA to be clearly seen

Fig. 9 Contrast-enhanced MRA of combined subtypes for the RCA

or the R-II-C subtype. a Axial view showing the common origin of

the SCAA. b Proximally, the axial view shows an interarterial course

of the LCA. c Distally, the axial view shows the transseptal course of

the LCA for the same patient

Fig. 10 R-III subtype. a 3D

volume-rendered image of a

single coronary artery (SCA)

giving rise to an RCA, which

bifurcates into the left anterior

descending (LAD) and

circumflex (cX) arteries. b CT

angiography of the RCA

branching into the LAD and cX

arteries in axial view

Pediatr Cardiol (2014) 35:441–449 447

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arteries have been documented as causing angina or sudden

cardiac death due to the interarterial course and compres-

sion of the aberrant coronary artery by exertional expan-

sion (from moderate to heavy physical activity) of the great

vessels [5, 36]. Other possible contributors include vaso-

spasm of the aberrant vessel or an intramural course; sur-

gical options include ostioplasty and bypass grafting of the

aberrant vessel [39]. Moreover, the alternative anatomy of

coronary arteries affects surgical repair of other CHD

associated with the SCAA [16, 31]. In addition, patients

with a complete intraseptal coronary course may be at high

risk for ischemic myocardial injuries [10]. Although sud-

den death for type II-B has been documented [8, 17, 22],

the clinical relevance for other subtypes remains unclear

[4]. For these subtypes, good prognoses without a decrease

in life expectancy or need for surgery have been reported

[12, 20, 25, 29]. Current research supports that medical

therapy resolved most presenting symptoms for patients

with SCAA that was neither interarterial (or type II-B) nor

septal [6, 9, 33, 35]. The next step of this study in particular

would be to follow-up patients and track clinical outcomes

of various subtypes within the cohort. Given sample size

limitations and lack of data in long-term follow-up, it is

difficult to correlate subtype to clinical outcomes.

Multidetector computed tomography angiography post-

processing techniques, including multiplanar reconstruc-

tion, maximum intensity projection, and 3D volume

rendering, assist in the clinical interpretation of the proxi-

mal course of the artery, its relationship to the great ves-

sels, as well as the ostial morphology (e.g., whether it is

single or not) because they are better characterized com-

pared with conventional angiography. Endoluminal imag-

ing, which is not possible in catheter angiography, also is

critical for assessing ostial morphology, a key feature in

defining SCAA.

In addition to better visualization of the SCAA itself,

MDCT angiography also allows for diagnosis of obstruc-

tive coronary artery disease, and invasive angiography can

be safely avoided in the majority of these patients. There is

also the potential for significant radiation dose decrease

using new emerging CT techniques, such as iterative

image-reconstruction technique [23] and dual-source

MDCT angiography [15].

Conclusion

Antemortem classification and characterization of SCAA

was devised within the last 30 years despite the discovery

of the arterial anomaly centuries ago. Conventional cath-

eter angiography is responsible for the current classifica-

tion schema and understanding of SCAA. MDCT and MR

angiography are advanced imaging modalities useful in the

exploration of the morphological dimensions as well as the

associated clinical pathologies. As a class of anomaly,

SCAA is considered relatively benign but warrants clinical

monitoring if an interarterial course is identified on cardiac

imaging. Cardiac MDCT and MR angiography allow for

accurate evaluation of coronary ostial morphology, coro-

nary course (especially proximal), endoluminal assessment,

and plaque characterization compared with conventional

imaging modalities.

Limitations

Although an association was found between SCAA and

CHD, it is possible that some selection bias occurred

because patients with CHD are more likely to be referred to

imaging compared with asymptomatic patients. Although

this association has been well shown, our findings of 41 %

of SCAA patients presenting concurrently with CHD may

be overstated due to the inability to capture more patients

who have isolated SCAA with no obvious presentation.

Furthermore, when compared with all coronary artery

anomalies, the prevalence of SCAA may be overstated due

to overrepresentation of specialized pediatric disorders at

this institution.

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