Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva
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JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. 69, NO. 12, 2017
PUBLISHED BY ELSEVIER ON BEHALF OF THE AMERICAN ISSN 0735-1097/$36.00
COLLEGE OF CARDIOLOGY FOUNDATION http://dx.doi.org/10.1016/j.jacc.2017.01.031
THE PRESENT AND FUTURE
STATE-OF-THE-ART REVIEW
Anomalous Aortic Origin of a
Coronary Artery From the
Inappropriate Sinus of Valsalva
Michael K. Cheezum, MD,a,b Richard R. Liberthson, MD,c Nishant R. Shah, MD, MPH, MSC,d Todd C. Villines, MD,e
Patrick T. O’Gara, MD,a Michael J. Landzberg, MD,f Ron Blankstein, MDa
ABSTRACT
Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized
by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death
with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to
identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classi-
fication, prevalence, risk stratification, and management. We comprehensively reviewed 77 studies reporting the prev-
alence of AAOCA among >1 million patients, and 20 studies examining outcomes of interarterial ALCA/ARCA patients.
Observational data suggests that interarterial ALCA is rare (weighted prevalence ¼ 0.03%; 95% confidence interval [CI]:
0.01% to 0.04%) compared with interarterial ARCA (weighted prevalence ¼ 0.23%; 95% CI: 0.17% to 0.31%).
Recognizing the challenges in managing these patients, we review cardiac tests used to examine AAOCA and knowledge
gaps in management. (J Am Coll Cardiol 2017;69:1592–608) Published by Elsevier on behalf of the American College of
Cardiology Foundation.
C ongenital coronary artery anomalies (CAA)
are rare and may be broadly classified as ab-
normalities of coronary artery origin, course,
destination, and size or number of vessels (1,2). This
and anomalous right coronary artery (ARCA) arising
at or above the left sinus of Valsalva. Rarely, AAOCA
vessels may also arise from the “noncoronary” sinus.
AAOCA are further characterized by 1 of 5 course sub-
review focuses on anomalous aortic origin of a coro- types as interarterial, subpulmonic (intraconal or
nary artery (AAOCA) arising at or above the inappro- intraseptal), pre-pulmonic, retroaortic, or retrocar-
priate sinus of Valsalva. Although classification of diac (Central Illustration). Additionally, AAOCA may
these cases varies (1–4), AAOCA arise from the aorta have an early intramural segment (within the aortic
by a separate ostium, shared or common ostium, or wall), as seen in the majority of interarterial cases.
as a branch vessel (5). Among subtypes, our discus- Among course subtypes, the potential for sudden car-
sion will focus on anomalous left coronary artery diac death (SCD) has been largely attributed to an
(ALCA) arising at or above the right sinus of Valsalva interarterial course between the aorta and pulmonary
From the aDepartments of Medicine and Radiology, Cardiovascular Division, Brigham and Women’s Hospital, Harvard Medical
Listen to this manuscript’s School, Boston, Massachusetts; bDepartment of Medicine, Cardiology Service, Fort Belvoir Community Hospital, Ft. Belvoir,
audio summary by Virginia; cDepartment of Medicine, Division of Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston,
JACC Editor-in-Chief Massachusetts; dLifespan Cardiovascular Institute, Division of Cardiovascular Medicine, Department of Medicine, Brown Uni-
Dr. Valentin Fuster. versity Alpert School of Medicine, Providence, Rhode Island; eDepartment of Medicine, Cardiology Service, Walter Reed National
Military Medical Center, Bethesda Maryland; and the fDepartment of Cardiology, Boston Children’s Hospital, Boston, Massa-
chusetts. The opinions and assertions herein are those of the authors alone, and do not represent the views of the U.S. Army,
Office of the Surgeon General, Department of Defense, or the U.S. Government. The authors have reported that they have no
relationships relevant to the contents of this paper to disclose.
Manuscript received October 7, 2016; revised manuscript received December 5, 2016, accepted January 3, 2017.
JACC VOL. 69, NO. 12, 2017 Cheezum et al. 1593
MARCH 28, 2017:1592–608 AAOCA Review
artery (Figure 1A) (6). By comparison, a subpulmonic for AAOCA in the absence of a clinical indi- ABBREVIATIONS
course exits the aorta below the pulmonic valve and cation for testing (Online Appendix A, Online AND ACRONYMS
traverses the right ventricular outflow tract, pulmo- Table 1) (8–12). Consequently, the true prev-
AAOCA = anomalous aortic
nary infundibulum, and interventricular septum alence of AAOCA in the general population origin of a coronary artery
(Figure 1B) (7). Although prior studies with invasive remains unknown.
ALCA = anomalous left
angiography and echocardiography provide limited In Figure 3, we focus specifically on the coronary artery
visualization of these course subtypes, coronary observed rate of interarterial ARCA and ANOCOR = anomalous
computed tomography angiography (CTA), magnetic interarterial ALCA cases among included connections of coronary
arteries
resonance angiography (MRA), and intravascular ul- studies. As shown, the frequency of inter-
trasound (IVUS) are improving characterization of arterial ALCA is rare (weighted prevalence ¼ ARCA = anomalous right
coronary artery
AAOCA vessels. 0.03%; 95% confidence interval [CI]: 0.01% to
CAA = coronary artery
Although evidence demonstrates that interarterial 0.04%) by comparison with interarterial
anomaly
ALCA and ARCA may be associated with an increased ARCA (0.23%; 95% CI: 0.17% to 0.31%). The
CI = confidence interval
risk of SCD among AAOCA subtypes (6), the preva- observed prevalence of the remaining course
CTA = computed tomography
lence of AAOCA and their associated absolute risk of subtypes is listed in Online Table 2, with a angiography
SCD in the general population is unknown. Thus, retroaortic course comprising the most com-
FFR = fractional flow reserve
controversy remains regarding the optimal approach mon subtype (prevalence ¼ 0.28%; 95% CI:
ICA = invasive coronary
to risk stratify and manage these patients. With 0.21% to 0.35%). angiography
increasing recognition of AAOCA, we aimed to review IVUS = intravascular
EVALUATION OF AAOCA
the following: 1) the observed prevalence of AAOCA ultrasound
arising at or above the inappropriate sinus of Valsalva MRA = magnetic resonance
TRANSTHORACIC AND TRANSESOPHAGEAL
with attention to the interarterial course subtype; 2) angiography
ECHOCARDIOGRAPHY. TTE is a common
the use of cardiac testing to examine AAOCA; 3) out- PCI = percutaneous coronary
technique used to evaluate young patients intervention
comes of interarterial ALCA and ARCA patients; and
with suspected or known cardiac disease, as a SCD = sudden cardiac death
4) recommendations and knowledge gaps in current
noninvasive, rapid, and widely available test
management. TTE = transthoracic
with low cost (Figure 4). Yet TTE has limited echocardiography
OBSERVED PREVALENCE OF AAOCA IN accuracy to detect AAOCA, requiring experi-
CLINICAL PRACTICE enced operators to identify coronary ostia. In a study
by Thankavel et al. (13), a standardized TTE protocol
We performed a comprehensive review of published improved AAOCA detection from 0.02% to 0.22% of
reports to examine the observed prevalence of patients. TTE also depends on patient habitus for
AAOCA arising at or above the inappropriate sinus of optimal image quality. Across studies designed to
Valsalva on cardiac testing with the course subtypes visualize AAOCA, 6% to 10% of patients were excluded
shown in the Central Illustration. Studies were on the basis of an uninterpretable TTE (8,9). Even after
included if they examined patients by invasive coro- excluding unsatisfactory cases, Pelliccia et al. (8)
nary angiography (ICA), transthoracic echocardiogra- were unable to visualize the RCA ostium in 20% of
phy (TTE), coronary CTA, or MRA. Detailed methods, young athletes, a population expected to have good
and inclusion and exclusion criteria are described in image quality. Lastly, TTE has limited spatial resolu-
Online Appendix A, incorporating evidence from 77 tion and lacks detailed characterization of AAOCA
studies and >1 million patients undergoing cardiac features and surrounding structures (14). Among 159
testing (Online Figure 1). AAOCA patients in the CHSS (Congenital Heart
In Figure 2, we summarize the observed prevalence Surgeon’s Society) registry, there was limited agree-
of AAOCA arising at or above the inappropriate sinus ment (weighted kappa) between institutional and
of Valsalva as the combined rate of all course sub- expert TTE reports and surgical findings of AAOCA
types (interarterial, subpulmonic, pre-pulmonic, ret- measures (i.e., interarterial course, intramural course,
roaortic, and retrocardiac) across included studies. As and acute angle takeoff) (15).
shown, there is significant variability in the observed Transesophageal echocardiography has been used
prevalence of AAOCA, which may be attributed to to identify AAOCA (15–23) and may be useful to
inherent referral bias, differences in age groups and visualize CAA perioperatively (24). With the addition
presentation of various cohorts, variable inclusion of 3-dimensional transesophageal echocardiography,
criteria and AAOCA course descriptions, and limita- visualization of AAOCA and their relation to sur-
tions in the ability of each modality to examine rounding anatomy may improve (25). At this time,
AAOCA. To date, few studies have screened patients however, transesophageal echocardiography is not a
1594 Cheezum et al. JACC VOL. 69, NO. 12, 2017
AAOCA Review MARCH 28, 2017:1592–608
C E NT R AL IL L U STR AT IO N AAOCA: Course Subtypes
Cheezum, M.K. et al. J Am Coll Cardiol. 2017;69(12):1592–608.
The 5 main course subtypes of anomalous aortic origin of a coronary artery (AAOCA) arising from the inappropriate sinus are shown: blue ¼ pre-pulmonic;
red ¼ interarterial; orange ¼ subpulmonic; green ¼ retroaortic; purple ¼ retrocardiac. Figure prepared by Robert Cheezum and Chris Shearin (DesignVis Studios Inc.,
Indianapolis, Indiana), and adapted with permission from Angelini et al. (80). Ao ¼ aorta; MV ¼ mitral valve; PV ¼ pulmonic valve; TV ¼ tricuspid valve.
routine tool to image AAOCA, considering the ability expertise, availability, and the strengths and limita-
of alternative noninvasive techniques to visualize tions of these techniques (Figure 4). In many centers,
CAA. CTA is preferred to image AAOCA due to rapid scan
times, high spatial resolution, and lower cost in
CORONARY CTA/MRA. Currently, coronary CTA and comparison to MRA. CTA has also been shown to have
MRA are the only Class I–indicated tests used to im- a high diagnostic accuracy to detect coronary artery
age AAOCA (26). The choice between these tech- stenosis when compared with ICA (27) and has the
niques depends on multiple factors, including local ability to characterize multiple AAOCA featuresJACC VOL. 69, NO. 12, 2017 Cheezum et al. 1595 MARCH 28, 2017:1592–608 AAOCA Review F I G U R E 1 Interarterial Versus Subpulmonic Course Subtypes (A) Three-dimensional volume rendering (top) and multiplanar image reconstruction (bottom) demonstrate an anomalous left main (LM) coronary artery with an interarterial course above the pulmonic valve (PV); (B) 3-dimensional volume rendering (top) and multiplanar image reconstruction (bottom) demonstrate an anomalous LM coronary artery arising from the right coronary cusp and following a subpulmonic course below the PV. Reprinted with permission from Cheezum et al. (5). Ao ¼ aorta; PA ¼ pulmonary artery; RV ¼ right ventricle. (Figure 5) (5). Although CTA incurs iodinated contrast iodinated contrast agents, but incurs lower spatial agents and radiation exposure, dose reduction stra- resolution, increased scan times, and higher cost. In tegies (28) and CT advancements continue to improve experienced centers, free-breathing MRA visualizes patient safety (29). Newer scanners routinely permit the coronary takeoff and course in nearly all patients very low radiation exposures (
1596
F I G U R E 2 Observed Prevalence of AAOCA Arising at or Above the Inappropriate Sinus of Valsalva on Cardiac Testing
Observed Prevalence of AAOCA on Cardiac Testing
AAOCA Review
Cheezum et al.
Study # AAOCA/Total Prevalence [95% Confidence Interval] Weight (%) Study # AAOCA/Total Prevalence [95% Confidence Interval] Weight (%)
Krasuski 2011 301/210,700 0.14% ICA 2.98 Werner 2001 8/62,320 0.01% Echo 3.76
Correia 2010 6/3,906 0.15% 2.48 Thankavel2015 14/6,428 0.22% 3.67
Tuncer 2006 109/70,850 0.15% 2.96 Lytrivi 2008 51/14,546 0.35% 3.73
Weighted = 0.15% [0.00%-0.54%]
Yildiz 2010 24/12,457 0.19% 2.81 Pooled: 73/83,294 0.09% 2 11.16
I = 98.4%, p < 0.001
Tuo 2013 7/3,026 0.23% 2.36
Turkmen 2013 137/53,655 0.26% 2.95
Ghadri 2014 25/9,782 0.26% 2.76 Srinivasan 2008 4/1,495 0.27% CTA 3.35
Gol 2002 156/58,023 0.27% 2.95 Sato 2005 4/1,153 0.35% 3.24
Ouali 2009 20/7,330 0.27% 2.69 Knickelbine 2009 16/4,543 0.35% 3.62
Yuksel 2013 47/16,573 0.28% 2.85 Yang 2010 22/6,014 0.37% 3.66
Harikrishnan 2002 21/7,400 0.28% 2.70 Komatsu 2008 17/3,910 0.43% 3.60
Click 1989 73/24,959 0.29% 2.90 Namgung 2014 48/8,864 0.54% 3.70
Altin 2015 17/5,548 0.31% 2.61 Xu 2012 73/12,145 0.60% 3.72
Kaku 1996 56/17,731 0.32% 2.86 Cheng 2010 23/3,625 0.63% 3.59
Pillai 2000 46/14,424 0.32% 2.83 Fujimoto 2011 38/5,869 0.65% 3.66
Aydinlar 2005 39/12,059 0.32% 2.80 Park 2013 11/1,582 0.70% 3.37
Correia 2004 13/3,660 0.36% 2.45 Krupinski 2014 54/7,115 0.76% 3.68
Akpinar 2013 101/25,368 0.40% 2.90 Graidis 2015 20/2,572 0.78% 3.52
Barriales-Villa 2006 98/23,300 0.42% 2.89 Opolski 2013 73/8,522 0.86% 3.69
Donaldson 1982 42/9,153 0.46% 2.75 Pan 2015 67/7,469 0.90% 3.68
Karaca 2007 35/7,574 0.46% 2.70 Turkvatan 2013 23/2,375 0.97% 3.50
Sohrabi 2012 30/6,065 0.49% 2.64 Shabestari 2012 28/2,697 1.04% 3.53
Aydar 2011 39/7,810 0.50% 2.71
Erol 2011 22/2,096 1.05% 3.46
Tuccar 2002 25/5,000 0.50% 2.58
Zhang 2010 20/1,879 1.06% 3.43
Kardos 1997 39/7,694 0.51% 2.71
Andreini 2010 30/2,757 1.09% 3.53
Tuo 2013 41/7,960 0.52% 2.72
Nasis 2015 107/9,774 1.09% 3.70
Cieslinski 1993 22/4,016 0.55% 2.49
Schmitt 2005 25/1,758 1.42% 3.41
Topaz 1992 78/13,010 0.60% 2.82
Clark 2014 22/1,518 1.45% 3.36
Kimbris 1978 44/7,000 0.63% 2.68
Cheezum 2017 103/5,991 1.72% 3.66
Sivri 2012 92/12,844 0.72% 2.81
Ghadri 2014 38/1,759 2.16% 3.41
Eid 2009 34/4,650 0.73% 2.55
Pooled: 888/107,482 0.83% Weighted = 0.82% [0.68-0.99%] 85.08
Wilkins 1988 80/10,661 0.75% 2.78 2
I = 86.6%, p < 0.001
Chaitman 1976 31/3,750 0.83% 2.46
Garg 2000 34/4,100 0.83% 2.50
Engel 1975 41/4,250 0.96% 2.51 Ripley 2014 116/59,844 0.19% 3.76
Ugalde 2010 129/10,000 1.29% 2.77 MRA
Angelini 1999 34/1,950 1.74% 2.12
Weighted = 0.44% [0.37-0.53%]
Pooled: 2,166/708,238 0.31% 2 Weighted = 0.70% [0.48-0.95%]
Overall: I = 95.6%, p < 0.001
I2 = 97.7%, p < 0.001
Invasive Coronary 0% 0.5% 1.0% 1.5% 2.0% 2.5% Noninvasive 0% 0.5% 1.0% 1.5% 2.0% 2.5%
Angiography Cardiac Imaging
JACC VOL. 69, NO. 12, 2017
MARCH 28, 2017:1592–608
Forest plot of anomalous aortic origin of a coronary artery (AAOCA) prevalence demonstrates the combined rate of all subtypes (interarterial, subpulmonic, pre-pulmonic, retroaortic, and retrocardiac) observed by
various cardiac testing methods. As described in Online Appendix A, weighted transformations and 95% confidence intervals are shown in patients undergoing coronary computed tomography angiography (CTA),
echocardiography (Echo), invasive coronary angiography (ICA), and magnetic resonance angiography (MRA).MARCH 28, 2017:1592–608
JACC VOL. 69, NO. 12, 2017
F I G U R E 3 Observed Prevalence of Interarterial ALCA and ARCA on Cardiac Testing
Interarterial ALCA Interarterial ARCA
Study N / Total Prevalence [95% CI] Weight (%) Study N / Total Prevalence [95% CI] Weight (%)
Pelliccia 1993 0/1,273 0.00% 1.04% Pelliccia 1993 0/1,273 0.00% 1.78%
Thankavel 2015 1/6,428 0.02% 2.65% Zeppilli 1998 2/3,150 0.06% 2.13%
Echo Echo
Lytrivi 2008 4/14,546 0.03% 3.37% Davis 2001 2/2,388 0.08% 2.05%
Labombarda 2014 1/3,229 0.03% 1.92% Lytrivi 2008 23/14,546 0.16% 2.39%
Zeppilli 1998 1/3,150 0.03% 1.90% Thankavel 2015 13/6,428 0.20% 2.29%
Davis 2001 2/2,388 0.08% 1.61% Labombarda 2014 8/3,229 0.25% 2.14%
2 2
Pooled: 9/31,014 0.03% Weighted = 0.02%; I =0%, p=NS 12.5% Pooled: 40/31,014 0.13% Weighted = 0.13%; I =40%, p=NS 12.78%
Krasuski 2011 36/210,700 0.02% 2.46%
Garg 2000 0/4,100 0.00% 2.18%
Click 1989 7/24,959 0.03% 2.42%
Correia 2004 0/3,660 0.00% ICA 2.06%
Harikrishnan 2002 7/7,400 0.09%
ICA 2.31%
Mavi 2004 0/10,042 0.00% 3.08%
Ayalp 2002 5/5,253 0.10% 2.26%
Kaku 1996 1/17,731 0.01% 3.51%
Sohrabi 2012 6/6,065 0.10% 2.28%
Click 1989 2/24,959 0.01% 3.71%
Correia 2004 4/3,660 0.11% 2.18%
Krasuski 2011 18/210,700 0.01% 4.22% Chaitman 1976 6/3,750 0.16% 2.18%
Topaz 1992 4/13,010 0.03% 3.29% Tuccar 2002 8/5,000 0.16% 2.25%
Donaldson 1983 3/9,153 0.03% 3.00% Kimbiris 1978 12/7,000 0.17% 2.31%
Kimbiris 1978 3/7,000 0.04% 2.74% Eid 2008 9/4,650 0.19% 2.23%
Sohrabi 2012 3/6,065 0.05% 2.59% Kaku 1996 44/17,731 0.25% 2.40%
Wilkins 1988 6/10,661 0.06% 3.13% Topaz 1992 36/13,010 0.28% 2.38%
Tuccar 2002 3/5,000 0.06% 2.39% Wilkins 1988 30/10,661 0.28% 2.36%
Chaitman 1976 4/3,750 0.11% 2.08% Garg 2000 15/4,100 0.37% 2.20%
Pooled: 47/325,831 0.01% 2 38.0% Berdoff 1986 12/2,145 0.56% 2.01%
Weighted = 0.02%; I = 69%* 2
Weighted = 0.16%; I = 96%*
Pooled: 237/326,084 0.07% 34.23%
Sato 2005 0/1,153 0.00% 0.96 Srinivasan 2008 1/1,495 0.07% 1.86%
Komatsu 2008 0/3,910 0.00% 2.13
CTA Graidis 2015 3/2,572 0.12% CTA 2.07%
Zhang 2010 0/1,879 0.00% 1.38 Yang 2010 9/6,014 0.15% 2.28%
Fujimoto 2011 0/5,869 0.00% 2.56 Krupinski 2014 11/7,115 0.15% 2.31%
Park 2013 0/1,582 0.00% 1.22 Turkvatan 2013 4/2,375 0.17% 2.04%
Krupinski 2014 0/7,115 0.00% 2.76 Andreini 2010 6/2,757 0.22% 2.09%
Clark 2014 0/1,518 0.00% 1.19 Knickelbine 2009 10/4,543 0.22% 2.23%
Graidis 2015 0/2,572 0.00% 1.69 Cheng 2010 8/3,625 0.22% 2.17%
Namgung 2014 1/8,864 0.01% 2.97 Lee 2012 53/22,925 0.23% 2.42%
Yang 2010 1/6,014 0.02% 2.59 Opolski 2013 20/8,522 0.23% 2.33%
Knickelbine 2009 1/4,543 0.02% 2.29 Park 2013 4/1,582 0.25% 1.88%
Ashrafpoor 2015 1/4,160 0.02% 2.20 Sato 2005 3/1,153 0.26% 1.73%
Opolski 2013 3/8,522 0.04% 2.93 Ashrafpoor 2015 14/4,160 0.34% 2.21%
Turkvatan 2013 1/2,375 0.04% 1.61 Komatsu 2008 14/3,910 0.36% 2.19%
Erol 2011 1/2,096 0.05% 1.48 Nasis 2015 36/9,774 0.37% 2.35%
Cheezum 2017 3/5,991 0.05% 2.58 Shabestari 2012 11/2,697 0.41% 2.90%
Cheng 2010 2/3,625 0.06% 2.05 Erol 2011 9/2,096 0.43% 2.00%
Schmitt 2005 1/1,758 0.06% 1.32 Fujimoto 2011 27/5,869 0.46% 2.28%
Nasis 2015 8/9,774 0.08% 3.05 Namgung 2014 41/8,864 0.46% 2.34%
Ghadri 2014 2/1,759 0.11% 1.32 Ghadri 2014 11/1,759 0.63% 1.93%
Srinivasan 2008 2/1,495 0.13% 1.17 Schmitt 2005 11/1,758 0.63% 1.93%
Andreini 2010 4/2,757 0.15% 1.76 Cheezum 2017 40/5,991 0.67% 2.28%
Torres 2010 10/6,000 0.17% 2.58 Zhang 2010 13/1,879 0.69% 1.95%
Shabestari 2012 5/2,697 0.19% 1.74 Clark 2014 12/1,518 0.79% 1.86%
2 2
Pooled: 46/98,028 0.05% Weighted = 0.03%; I = 54%* 47.5% Pooled: 371/114,953 0.32% Weighted = 0.32%; I =73%* 50.82%
Angelini 2015 6/3,529 0.17% MRA 2.0% Angelini 2015 12/3,529 0.34% MRA 2.17%
Weighted = 0.03% [0.01%-0.04%] Weighted = 0.23% [0.17%-0.31%]
Summary 2 Summary 2
I = 68%, p < 0.001 I = 94%, p < 0.001
0% 0.25% 0.5% 0.75% 1.0% 1.25% 0% 0.25% 0.5% 0.75% 1.0% 1.25%
AAOCA Review
Cheezum et al.
Forest plot of interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA) prevalence observed by various cardiac testing methods. Weighted transformations and 95% confidence
intervals (CI) are shown in patients undergoing CTA, Echo, ICA, and MRA. Abbreviations as in Figure 2.
15971598 Cheezum et al. JACC VOL. 69, NO. 12, 2017
AAOCA Review MARCH 28, 2017:1592–608
F I G U R E 4 Anatomic Tests Used to Characterize AAOCA Vessels
Echo CTA MRA ICA IVUS
Indication for
- Class I Class I Class IIa Class IIa
AAOCA Imaging
0.8 × 1.5 mm
Spatial Resolution 0.5 mm (isotropic) 1.0 mm (volumetric) 0.3 mm 0.15 × 0.25 mm
(4-MHz transducer)
Temporal
30 msec 75-175 msec 60 – 120 msec 7-20 msec Variable
Resolution
Visualize surround
Limited X X
structures
Dynamic imaging Limited Limited Limited
(Limited at ostium)
Noninvasive, rapid Noninvasive, rapid Noninvasive Availability Dynamic imaging
Widely available Visualize takeoff + Visualize takeoff + Improved spatial and Evaluation of
Low cost course + surrounding course + surrounding temporal resolution proximal narrowing
structures structures Ancillary techniques
Strengths Evaluate CAD Evaluate cardiac (IVUS, OCT, FFR)
Examine multiple function, perfusion and
AAOCA features * prior MI
Avoid radiation &
iodinated contrast
Limited accuracy for Limited availability Limited availability Invasive; Cost Invasive
detection of AAOCA lodinated contrast Cost and scan-time Contrast and radiation Cost
Dependent on body Radiation (low dose, increased vs. CTA Limited visualization of Difficulty engaging
Limitations
habitus and operator e.g. 2-8 mSv now Spatial resolution ostium, proximal anomalous vessel
technique routine) decreased vs. CTA course, surrounding
structures
Comparison of available anatomic tests used to characterize AAOCA vessels. Adapted with permission from Angelini and Flamm (44). Echocardiography image courtesy
of Daniel Shindler and Sudha Patel (Rutgers–Robert Wood Johnson Medical School, New Brunswick, New Jersey). *See Figure 5 for CTA-identified AAOCA features.
CAD ¼ coronary artery disease; FFR ¼ fractional flow reserve; IVUS ¼ intravascular ultrasound; MI ¼ myocardial infarction; OCT ¼ optical coherence tomography; other
abbreviations as in Figures 1, 2, and 3.
AAOCA vessels. In a registry of 13 hospitals and typically measured at the point of maximal narrowing
23,300 ICA cases, the initial course of anomalous in diastolic phase imaging. Yet systolic compression
coronary arteries was not identified in 41% of patients of proximal AAOCA vessels may be observed in cases
(n ¼ 40 of 98) (40). When ICA detects AAOCA, many with an early intramural course. As in patients with
patients are referred for CTA or MRA to improve deep myocardial bridging, prolonged pressure on
visualization. In our previously reported experience, coronary arteries during systole and early diastole
44% of AAOCA cases (n ¼ 45 of 103) were referred for may decrease coronary blood flow. In these cases,
CTA after a prior ICA (5). Nonetheless, more recent IVUS offers superior resolution to image coronary
use of specialized ICA catheters may improve the arteries throughout the cardiac cycle and is accord-
detection and characterization of AAOCA, particularly ingly designated as having a Class IIa indication to
when combined with IVUS. identify mechanisms of coronary flow restriction (26).
As a technique with high spatial and temporal Although IVUS is low risk, engaging AAOCA vessels
resolution, IVUS offers excellent dynamic imaging may be difficult in cases with ostial narrowing, an
(41,42) (Figure 4). Considering that the majority of ostial ridge, or an acute angle takeoff. Additional care
coronary artery perfusion in left-sided epicardial is needed during IVUS to distinguish vessel spasm
vessels occurs during diastole, stenosis grading is from true narrowing (43). In a review by Angelini andJACC VOL. 69, NO. 12, 2017 Cheezum et al. 1599 MARCH 28, 2017:1592–608 AAOCA Review F I G U R E 5 CTA-Identified AAOCA Features (A) Multiplanar axial computed tomography reconstruction at level of the coronary artery takeoff demonstrating AAOCA ostia types (separate ostium, shared ostium, and branch vessel). (B) Proximal vessel morphology in double oblique view using the percentage of lumen diameter narrowing compared with normal distal reference (not shown), stratified by normal, oval shape (
1600 Cheezum et al. JACC VOL. 69, NO. 12, 2017
AAOCA Review MARCH 28, 2017:1592–608
T A B L E 1 Autopsy Studies of SCD in ARCA/ALCA Patients
Total Number of Autopsy
% of ARCA/ALCA % of ARCA/ALCA
Patients With ARCA/ALCA Coronary-Related SCD
Deaths During Asymptomatic
First Author, Year (Ref. #) ARCA ALCA ARCA (%) ALCA (%) Exercise Before Death
Cheitlin et al., 1974 (55) 18 33 0 of 18 (0) 9 of 33 (27) 78 *
Kragel and Roberts, 1988 (56) 25 7 8 of 25 (32) 5 of 7 (71) * 38
Taylor et al., 1992 (57) 24 28 * 23 of 28 (82) * 66
Taylor et al., 1997 (58) 21 9 4 of 21 (19) 8 of 9 (89) 83 66
Frescura et al., 1998 (59) 7 4 4 of 7 (57) 4 of 4 (100) 75 50
*Not reported. Adapted with permission from Mirchandani and Phoon (6).
ALCA ¼ anomalous left coronary artery; ARCA ¼ anomalous right coronary artery; SCD ¼ sudden cardiac death.
may be associated with more transient mechanisms of risk of SCD (Table 1). By these studies, the risk of SCD
ischemia (46,47). Recognizing rare, but potential appears highest in young individuals and particu-
difficulties with invasive testing, Lee et al. (45) noted larly in interarterial ALCA, during or following a
that 1 patient developed an ostial ARCA dissection period of strenuous exertion. A significant portion
during ICA, and placement of a guiding catheter was (38% to 66%) of ALCA and ARCA patients have no
not possible in 2 patients. reported symptoms before sudden death, thus
NONINVASIVE FUNCTIONAL TESTING. Several limiting efforts to detect these patients antemortem.
studies have assessed the functional significance of Despite this, the incidence of SCD from CAA in large
AAOCA by exercise treadmill testing and stress cohorts of young athletes and screening populations
myocardial perfusion imaging (5,48–53). Gräni et al. is exceedingly rare (Table 2). Given the rarity of
(54) examined 46 adults (mean age 56 12 years) with these cases, current guidelines do not support uni-
CTA-identified AAOCA (26 interarterial, 20 other) by versal pre-participation cardiac testing to screen for
single-photon emission computed tomography and AAOCA in asymptomatic athletes (63). This remains
found that myocardial ischemia or scar was only an evolving issue, with studies and clarification
present in patients with concomitant coronary artery needed to consider screening options. Currently,
disease. Notably, vasodilator testing was used in 50% the American Heart Association and American
of patients. Yet exercise stress is preferable for eval- Academy of Pediatrics offer various tools for indi-
uating patients with AAOCA when considering that a vidualized risk assessment (64,65). Additionally, a
majority of SCD cases attributed to AAOCA occur with multidisciplinary task force held by the National
strenuous exercise (Table 1) (6,55–60). Nonetheless, Collegiate Athletic Association provided a recent
both exercise treadmill testing and stress myocardial consensus statement on cardiovascular care of col-
perfusion imaging may yield false-positive and false- lege athletes (66).
negative results (46,47,61). Among 27 young athletes
OUTCOME STUDIES: REVASCULARIZATION VERSUS
with AAOCA (23 ALCA, 4 ARCA) described by Basso
CONSERVATIVE MANAGEMENT. To examine out-
et al. (62), 6 patients had a normal exercise treadmill
comes of interarterial ALCA and ARCA patients, we
test before SCD. Consequently, the absence of
performed a comprehensive review of published data
ischemia during stress testing cannot be viewed as
using detailed methods and inclusion and exclusion
reassuring currently, particularly when potentially
criteria as described in Online Appendix B. Among
high-risk anatomic features are present (i.e., proximal
5,459 abstracts in our initial search of published re-
vessel narrowing) (5,26). Ongoing studies are needed
ports, we identified 20 studies reporting outcomes
to define the optimal approach to risk stratification
(death, revascularization, symptoms, and/or
of these patients and to compare the accuracy of
myocardial infarction) with at least 20 ALCA/ARCA
various tests used to detect AAOCA narrowing and
patients and >1 year of follow-up (Online Figure 2). As
ischemia.
described in Table 3, the incidence of AAOCA-related
OUTCOMES death in available follow-up is rare, incorporating
evidence from 8 imaging cohorts, 10 selected revas-
SUDDEN CARDIAC DEATH. To date, several autopsy cularization cohorts (9 surgical, 1 percutaneous cor-
studies have demonstrated that interarterial ALCA onary intervention [PCI]), and 2 systematic reviews.
and ARCA patients are associated with an increased Notably, only 2 included studies in adult cohorts ofJACC VOL. 69, NO. 12, 2017 Cheezum et al. 1601
MARCH 28, 2017:1592–608 AAOCA Review
T A B L E 2 Population Studies of SCD
Deaths Attributed
Sudden Deaths to CAA
Study
First Author, Year (Ref. #) Population N Duration, yrs Total, n (%) Cardiac, n n Incidence
Wren et al., 2000 (81) England, children 1 to 806,000 10 270 (0.03) 26 0 0
20 years of age
Eckart et al., 2004 (82) U.S. military recruits 6,300,000 25 126 (0.002) 64 21 1 in 300,000
Corrado et al., 2006 (83) Italy, population 12–35 yrs of 4,379,900 26 * 320 21 1 in 208,567
age
Redelmeier and Greenwald, Runners from 26 U.S. 3,292,268 30 26 (0.0008) 21 2 1 in 1,646,134
2007 (84) marathons
Maron et al., 2009 (85) U.S. competitive athletes * 27 1,866 (*) 1,049 119 *
Chugh et al., 2009 (86) Oregon county, children 660,486† 3 8 (*) 3 0 *
#17 years of age
Harris et al., 2010 (87) U.S. triathletes 959,214 3 14 (0.001) 7 1 1 in 959,214
Harmon et al., 2011 (88) NCAA athletes 393,932 5 80 (0.02) 45 * *
*Not reported. †Total population of Multnomah County, Oregon, including children and adults. Adapted with permission from Peñalver et al. (89).
CAA ¼ coronary artery anomalies (comprising all types of CAA; anomalous aortic origin of a coronary artery subtype not reported); NCAA ¼ National Collegiate Athletic
Association; SCD ¼ sudden cardiac death.
predominantly ARCA patients (>97%) have examined management remain an evolving topic, with partic-
a primary approach of conservative therapy, ular debate regarding the indications for surgical
observing a very low mortality rate (1602
T A B L E 3 Outcomes of Interarterial ARCA/ALCA Patients (Studies With $20 Patients and at Least 1 Year of Follow-Up)
First Author, Year Total N Surgery (n) PCI (n)
AAOCA Review
Cheezum et al.
(Ref. #) Year Population Age, yrs (ARCA/ALCA) Follow-Up, yrs (ARCA/ALCA) (ARCA/ALCA) Comments/Morbidity
Imaging Cohorts
Kaku et al. (48) 1996 17,731 adults referred for 56 12 44 (44/0) 5.6 4.2 0 (0/*) * All patients treated conservatively and no deaths
ICA attributed to ARCA.
Lytrivi et al. (90) 2008 14,546 children referred 45 27 (23/4) 2.5 3.0 6 (5/1) * No deaths attributed to ARCA/ALCA among
for TTE 22 patients with follow-up.
Krasuski et al. (91) 2011 210,700 adults referred 58 14 54 (36/18) 9.2 [4.5–16.1] 28 (20/8) * Among 301 AAOCA, similar all-cause mortality
for ICA with surgery (n ¼ 36 of 94, 38%) vs. no
surgery (n ¼ 95 of 207, 46%). Among 54 IAC,
lower all-cause mortality with surgery (n ¼ 5
of 28, 18%) vs. no surgery (n ¼ 12 of 26,
46%), but underpowered for comparison. No
perioperative deaths occurred.
Lee et al. (92) 2012 22,925 adults referred for 56 12 53 (53/*) 2.5 [0.8–3.9] 8 (8/*) * 1 CV death, 3 nonfatal MI, 8 UA in follow-up
CTA among 53 with IAC. Angina more common in
53 IAC vs. 34 subpulmonic IAC group vs. subpulmonic course (43% vs.
ARCA 6%, p ¼ 0.001)
Opolski et al. (67) 2013 8,522 adults referred for 56 13 22 (20/2) 1.3 1.0 2 (1/1) * Among 72 AAOCA, 97% (n ¼ 70 of 72)
CTA conservatively managed, with no deaths or MI
attributed to AAOCA; 27% had worsened
symptoms. Syncope more common in IAC
compared with no IAC (33% vs. 9%,
p ¼ 0.04).
Ripley et al. (38) 2014 59,844 adults referred 54 [40–64] 64 (†/†) 4.3 [2.5–7.8] 23 (†/†) * 13 single coronaries with unclear course.†
for MRA 3 deaths not attributed to IAC.
Nasis et al. (49) 2015 9,774 adults referred for 58 13 44 (36/8) 2.3 1.3 9 (6/3) * No cardiac deaths or acute coronary syndrome in
CTA follow-up.
Cheezum et al. (5) 2017 5,992 adults referred for 52 17 43 (40/3) 5.8 [3.8–7.8] 13 (12/1) * 2 late ($90-day) CV deaths in ARCA patients, not
CTA attributed to ARCA vessel.
Revascularization Cohorts
Osaki et al. (53) 2008 31 ARCA/ALCA patients; 67 31 (18/13) 4.8†/1.9† 7 (0/7) * 1 patient with symptoms after surgery; 1 cardiac
subset of 7 ALCA with death (care withdrawn POD1 for neurological
surgery impairment after resuscitation); No surgery/
surgery.†
Davies et al. (93) 2009 36 ARCA/ALCA surgical 44 16 36 (21/15†) 1.1 2.8 36 (21/15) * 2 LAD included;† 1 (3%) recurrent symptoms;
repair 1 death from subdural bleed.
Frommelt et al. 2011 27 ARCA/ALCA surgical 13 4 27 (20/7) 1.8 [*] 27 (20/7) * No ischemic symptoms or deaths in follow-up.
(94) repair 7 patients with trivial AI.
Mumtaz et al. (95) 2011 22 ARCA/ALCA unroofing 15 [*] 22 (15/7) 1.4 [*] 22 (15/7) * 1 (5%) with chest pain but normal ICA post-
operatively. No deaths in follow-up.
Kaushal et al. (96) 2011 27 ARCA/ALCA surgical 14 12 27 (25/2) 1.2 0.1 27 (25/2) * 2 (7%) with noncardiac symptoms. No deaths or
JACC VOL. 69, NO. 12, 2017
repair significant morbidity.
MARCH 28, 2017:1592–608
Mainwaring et al. 2014 74 ARCA/ALCA surgical 15 [*] 74 (47/27) 6.0 [*] 74 (47/27) * 1 heart transplant; remaining patients free of
(51) repair cardiac symptoms. No deaths.
Wittlieb-Weber 2014/2007 24 ARCA/ALCA surgical 12 [*] 24 (16/8) 5.3 [*] 24 (16/8) * 13 (54%) with follow-up symptoms, none with
et al. (97)/ repair ischemia. Normal quality of life when reported
Brothers (n ¼ 12). No deaths, 1 emergent reoperation
et al. (52) POD1, 2 post-operative infections, 4 mild AI,
11 effusions (1 requiring drainage).
Continued on the next pageJACC VOL. 69, NO. 12, 2017 Cheezum et al. 1603
MARCH 28, 2017:1592–608 AAOCA Review
and, in selected individuals, the benefits of revascu-
AAOCA ¼ anomalous aortic origin of the coronary artery; AI ¼ aortic insufficiency; BMS ¼ bare-metal stent; CTA ¼ computed tomography angiography; CV ¼ cardiovascular; DES ¼ drug-eluting stent; F/U ¼ follow-up; IAC ¼ interarterial course; ICA ¼ invasive
coronary angiography; IVUS ¼ intravascular ultrasound; LAD ¼ left anterior descending artery; MRA ¼ magnetic resonance angiography; MI ¼ myocardial infarction; PCI ¼ percutaneous coronary intervention; POD ¼ post-operative day; SD ¼ standard deviation; TTE ¼
related deaths. n ¼ 10 had clinically indicated
restenosis (n ¼ 2 BMS, n ¼ 2 DES) and n ¼ 1
otherwise no significant operative morbidity.
follow-up ICA, including n ¼ 4 with in-stent
No deaths in follow-up. Weighted average.†
2.2% symptoms, 9.2% morbidity, 0.6% cardiac
larization likely outweigh the benefits of surgery.
97% free of symptoms attributed to coronary
prosthetic valve. No perioperative deaths.
Symptoms improved in PCI subset. No ARCA
1 (2%) with severe AI after ALCA unroofing,
anomaly; 1 death from endocarditis of a
Conversely, a conservative approach may be reason-
1 (1%) late noncardiac death. No cardiac
able in asymptomatic individuals with interarterial
death (n ¼ 2 of 325) in follow-up
ARCA, no proximal vessel narrowing, and no evi-
with late-stent thrombosis.
dence of ischemia. Ultimately, we recognize that the
symptoms at follow-up.
optimal management strategy likely varies as a
function of individual age, presentation, anatomy,
and physiology.
SURGICAL REPAIR. When a decision is made to pur-
sue surgical repair, evidence suggests that coronary
artery bypass graft should be avoided in the absence
of concomitant obstructive coronary artery disease,
given the potential for competitive flow from native
42 (42/*)
4 (*/4)
vessels to cause graft failure. Although a discussion of
*
*
*
surgical repair techniques is beyond the scope of this
review, coronary unroofing is generally preferred in
patients with an early intramural course, when
326 (*/326)
75 (69/6)
29 (24/5)
feasible. Alternatively, coronary reimplantation,
57 (*/57)
3 (3/*)
fenestration, neo-ostia formation, or combination
techniques provide additional options. In all cases,
care must be taken to avoid iatrogenic injury to the
aortic valve commissure and its support.
3.8 0.8
5.0 2.9
1.6 0.2
PERCUTANEOUS CORONARY INTERVENTION. Limited
1.7*†
0–14
Revascularization Cohorts
evidence exists regarding the use of PCI in patients
with interarterial ALCA or ARCA. In a study of 42
Reviews
predominantly adult patients (mean age 48 years,
range 12 to 73 years) with interarterial ARCA under- 326 (*/326)
75 (69/6)
29 (24/5)
67 (67/*)
72 (*/72)
going PCI, the rate of in-stent restenosis was 13% by
serial IVUS (42). In that study, 29% of patients had
recurrent symptoms during a median follow-up
period of 5 years. Although coronary artery bypass
Values are mean SD [IQR] unless otherwise indicated. *Not applicable or not reported. †Median value.
graft guidelines acknowledge that PCI has been used
40 20
36 22
48 12
43 3
in adults with anomalous coronary arteries (71), a
*
recent American College of Cardiology/American
Heart Association Task Force recommends, “surgical
transthoracic echocardiogram; UA ¼ unstable angina; other abbreviations as in Table 1.
procedures are the only therapies available for cor-
259 patients with AAOCA;
62 with no follow-up,
75 ARCA/ALCA unroofing
326 ALCA surgical repair
264 ALCA (130 autopsy,
recting these anomalies” (72). Consequently, PCI is
72 with follow-up)
42 PCI, 3 surgery, 22
currently not considered a routine option for revas-
medical therapy
61 with surgery, 29
67 ARCA w/IVUS;
cularization in these patients.
ARCA/ALCA
EXERCISE RESTRICTION
Recommendations for exercise and disqualification
from competitive sports were the subjects of the 2015
American College of Cardiology/American Heart As-
2008
2016
2014
2015
2012
sociation updated scientific statement (Table 4,
Available Recommendations section) (72). In ARCA
T A B L E 3 Continued
patients with symptoms, arrhythmias, or ischemia on
Angelini et al. (42)
Nguyen et al. (68)
Sharma et al. (98)
exercise testing, restriction from all competitive sports
Feins et al. (99)
Moustafa et al.
is recommended while awaiting surgical repair.
(100)
Conversely, in ARCA patients without symptoms or a
positive exercise stress test, “permission to compete
can be considered after adequate counseling of the1604 Cheezum et al. JACC VOL. 69, NO. 12, 2017
AAOCA Review MARCH 28, 2017:1592–608
T A B L E 4 Management of Interarterial ALCA/ARCA and Key Knowledge Gaps
Available Recommendations in Interarterial ALCA/ARCA Management
2011 ACC/AHA Guideline for CABG (71) and 2015 ACC/AHA Scientific Statement for Competitive Athletes
Class 2008 ACC/AHA Guidelines for Management of ACHD (26) With Cardiovascular Abnormalities (72)
I Surgical coronary revascularization for: —
—Anomalous left main with an interarterial course
—Ischemia due to coronary compression (when coursing between
the great arteries or in intramural fashion)
—Interarterial ARCA and evidence of ischemia.
IIa Surgical revascularization can be beneficial if there is vascular ARCA patients should be evaluated by an exercise stress test. If
wall hypoplasia or obstruction to coronary flow, regardless of normal stress test and no symptoms, permission to
inability to document ischemia. compete can be considered after counseling and
considering uncertain accuracy of a negative stress test.
IIb Surgical coronary revascularization may be reasonable in patients After successful surgical repair of ALCA/ARCA, athletes may
with anomalous left anterior descending with a course consider participation in all sports 3 months after surgery if
between the aorta and PA. free of symptoms and an exercise stress test shows no
evidence of ischemia or cardiac arrhythmias.
III — Restrict ALCA patients from all competitive sports, regardless
of symptoms, before surgical repair, with possible
exception of Class IA sports.*
Restrict ARCA patients from all competitive sports before
surgical repair if symptoms, arrhythmias, or signs of
ischemia on exercise stress testing, with possible exception
of Class IA sports.*
Key Knowledge Gaps in AAOCA patients
1. Do surgical repair, medical management, and/or exercise restriction affect the natural history of interarterial ALCA and ARCA?
2. Do the risks of surgical repair outweigh potential long-term benefits?
3. What is the optimal technique for AAOCA repair considering individual anatomy (e.g., intramural course, relation to commissure)?
4. How should anatomic vs. functional testing guide risk stratification and management?
5. Is there a role for other testing (e.g., MRI evaluation for scar, event monitoring) to risk stratify interarterial ALCA and ARCA patients?
6. What is the comparative accuracy of tests used to identify AAOCA origin, course, and anatomic features (i.e., intramural course)?
7. What degree of proximal vessel narrowing defines obstruction in AAOCA vessels?
8. What is the true prevalence of AAOCA and the absolute risk of SCD associated with it in the general population?
9. Consensus terminology and precise characterization of AAOCA are needed throughout published reports.
10. Objective and clearly defined endpoints are needed to examine outcomes in AAOCA patients with prospective follow-up.
*IA sports: for example, bowling, cricket, golf, curling, riflery, yoga (101).
ACC ¼ American College of Cardiology; ACHD ¼ adult congenital heart disease; AHA ¼ American Heart Association; CABG ¼ coronary artery bypass graft; MRI ¼ magnetic
resonance imaging; PA ¼ pulmonary artery; other abbreviations as in Tables 1 and 3.
athlete and/or the athlete’s parents as to the risk and collaborations are underway to improve our under-
benefit, taking into consideration the uncertainty of a standing of these patients. The CHSS Registry of
negative stress test” (72). Additionally, exceptions AAOCA is a multicenter study examining outcomes of
may be made for participation in Class IA sports (i.e., patients (age #30 years) with AAOCA managed by
bowling, cricket, golf, curling, riflery, yoga). observation or surgery (73). CHSS includes AAOCA
In athletes with ALCA, “especially cases where the patients with an interarterial, intraseptal, and/or
artery passes between the pulmonary artery and intramural course. The registry consists of a retro-
aorta” (72), restriction from all competitive sports is spective cohort (diagnosis between January 1998 and
recommended (with the possible exception of Class January 2009), and a prospective cohort diagnosed
IA sports) while awaiting surgical repair. After sur- after January 2009. As of January 2014, 378 patients
gery, a return to intense activities may be considered with AAOCA are included from 35 institutions (74),
if the patient remains asymptomatic and an exercise incorporating patients from the Society of Thoracic
stress test shows no evidence of ischemia or cardiac Surgeons Congenital Heart Surgery Database (75).
arrhythmias. The registry of proximal anomalous connections of
coronary arteries (ANOCOR) of the French Society of
FUTURE DIRECTIONS Cardiology is a prospective multicenter observational
study of patients $15 years of age with ANOCOR
In considering available evidence and recommenda- diagnosed by ICA or CTA (76). Aims are to examine
tions in AAOCA management, we recognize that strategies used to treat ANOCOR and their impact on
several key knowledge gaps remain and require morbidity and mortality at 5-year follow-up with
further study (Table 4, Key Knowledge Gaps section). scheduled completion in January 2018. As of February
To examine these knowledge gaps, several 2015, the registry is composed of 472 patientsJACC VOL. 69, NO. 12, 2017 Cheezum et al. 1605
MARCH 28, 2017:1592–608 AAOCA Review
(mean age 63 13 years, range 16 to 95 years of age), AAOCA patients remains uncertain. Recognizing the
including 451 (91%) with AAOCA among various inherent variability in patient factors, as well as
ANOCOR subtypes (77). institutional and provider preferences, an individu-
Incorporating AAOCA among other causes of SCD alized approach is recommended.
in young patients, the Sudden Death in the Young ACKNOWLEDGMENTS The authors are grateful to
Registry (78) will further aid studies of the incidence Robert Cheezum and Chris Shearin (DesignVis Studios
and mechanisms of SCD (79). With these collabora- Inc., Indianapolis, Indiana) for their contribution of
tions, key knowledge gaps may be addressed to the Central Illustration; Daniel Shindler and Sudha
inform public efforts and consensus guidelines, with Patel (Rutgers–Robert Wood Johnson Medical School,
a common goal to improve AAOCA recognition and New Brunswick, New Jersey) for their contribution of
management and prevent SCD. the echocardiography image in Figure 4; and Dr.
CONCLUSIONS Justin Dunn (Summa Health System, Akron, Ohio)
and Hunain Shiwani (Leeds General Infirmary, Leeds,
The risk of SCD appears highest among patients with United Kingdom) for their feedback and assistance in
interarterial ALCA and, in selected individuals, the the preparation of the manuscript.
benefits of revascularization likely outweigh the risks
of surgery. However, in many individuals with ARCA ADDRESS FOR CORRESPONDENCE: Dr. Michael K.
and an interarterial course, a conservative approach Cheezum, Brigham and Women’s Hospital, Harvard
may be reasonable, particularly in older patients with Medical School, Departments of Medicine and Radi-
no proximal vessel narrowing and no signs or symp- ology (Cardiovascular Division), 75 Francis Street,
toms of ischemia. Nevertheless, the optimal approach Boston, Massachusetts 02115. E-mail: mcheezum@
to risk stratification and management for many gmail.com.
REFERENCES
1. Angelini P. Coronary artery anomalies: an entity 10. Park JH, Kwon NH, Kim JH, et al. Prevalence of artery from the left coronary sinus: delineation by
in search of an identity. Circulation 2007;115: congenital coronary artery anomalies of Korean omniplane transesophageal echocardiogram. Can J
1296–305. men detected by coronary computed tomography. Cardiol 1996;12:529–31.
Korean Circ J 2013;43:7–12.
2. Jacobs ML, Mavroudis C. Anomalies of the 17. Kaku B, Shimizu M, Kita Y, Yoshio H, Ino H,
coronary arteries: nomenclature and classification. 11. Angelini P, Cheong B, Uribe C, et al. Results of Takeda R. Detection of anomalous origin of the
Cardiol Young 2010;20 Suppl 3:15–9. systematic MRI-based pre-participation screening left coronary artery by transesophageal echocar-
of school children: the sample size issue (abstr). diography and magnetic resonance imaging. Jpn
3. Brothers JA, Gaynor JW, Jacobs JP, et al. The
J Am Coll Cardiol 2015;65:A1305. Heart J 1994;35:383–8.
registry of anomalous aortic origin of the coronary
artery of the Congenital Heart Surgeons’ Society. 12. Prakken NH, Cramer MJ, Olimulder MA, 18. Fernandes F, Alam M, Smith S, Khaja F. The
Cardiol Young 2010;20 Suppl 3:50–8. Agostoni P, Mali WP, Velthuis BK. Screening for role of transesophageal echocardiography in
proximal coronary artery anomalies with 3- identifying anomalous coronary arteries. Circula-
4. Lipton MJ, Barry WH, Obrez I, Silverman JF,
dimensional MR coronary angiography. Int J Car- tion 1993;88:2532–40.
Wexler L. Isolated single coronary artery: diag-
diovasc Imaging 2010;26:701–10. 19. Giannoccaro PJ, Sochowski RA, Morton BC,
nosis, angiographic classification, and clinical sig-
nificance. Radiology 1979;130:39–47. 13. Thankavel PP, Lemler MS, Ramaciotti C. Utility Chan KL. Complementary role of trans-
and importance of new echocardiographic oesophageal echocardiography to coronary angi-
5. Cheezum MK, Ghoshhajra B, Bittencourt MS, ography in the assessment of coronary artery
screening methods in diagnosis of anomalous
et al. Anomalous origin of the coronary artery anomalies. Br Heart J 1993;70:70–4.
coronary origins in the pediatric population:
arising from the opposite sinus: prevalence and
assessment of quality improvement. Pediatr Car- 20. Alam M, Brymer J, Smith S. Transesophageal
outcomes in patients undergoing coronary CTA.
diol 2015;36:120–5. echocardiographic diagnosis of anomalous left
Eur Heart J Cardiovasc Imaging 2017;18:224–35.
14. Brothers JA, Whitehead KK, Keller MS, coronary artery from the right aortic sinus. Chest
6. Mirchandani S, Phoon CK. Management of 1993;103:1617–8.
Fogel MA, Paridon SM, Weinberg PM, Harris MA.
anomalous coronary arteries from the contralat-
Cardiac MRI and CT: differentiation of normal 21. Henson KD, Geiser EA, Billett J, Alexander JA,
eral sinus. Int J Cardiol 2005;102:383–9.
ostium and intraseptal course from slitlike ostium Akins EW, Bopitiya C. Use of transesophageal
7. Liberthson RR, Dinsmore RE, Bharati S, et al. and interarterial course in anomalous left coronary echocardiography to visualize an anomalous right
Aberrant coronary artery origin from the aorta: artery in children. AJR Am J Roentgenol 2015;204: coronary artery arising from the left main coronary
diagnosis and clinical significance. Circulation W104–9. artery (single coronary artery). Clin Cardiol 1992;
1974;50:774–9. 15:462–5.
15. Lorber R, Srivastava S, Wilder TJ, et al., for the
8. Pelliccia A, Spataro A, Maron BJ. Prospective AAOCA Working Group of the Congenital Heart 22. Gaither NS, Rogan KM, Stajduhar K, Banks AK,
echocardiographic screening for coronary artery Surgeons Society. Anomalous aortic origin of cor- Hull RW, Whitsiff T, Vernalis MN. Anomalous
anomalies in 1,360 elite competitive athletes. Am onary arteries in the young: echocardiographic origin and course of coronary arteries in adults:
J Cardiol 1993;72:978–9. evaluation with surgical correlation. J Am Coll identification and improved imaging utilizing
Cardiol Img 2015;8:1239–49. [Published correc- transesophageal echocardiography. Am Heart J
9. Zeppilli P, dello Russo A, Santini C, et al. In vivo
tion appears in J Am Coll Cardiol Img 2016;9:217.] 1991;122:69–75.
detection of coronary artery anomalies in asymp-
tomatic athletes by echocardiographic screening. 16. Sasson Z, Grande P, Lorette I, McEwan P. 23. Salloum JA, Thomas D, Evans J. Trans-
Chest 1998;114:89–93. Proximal narrowing of anomalous right coronary oesophageal echocardiography in diagnosis of1606 Cheezum et al. JACC VOL. 69, NO. 12, 2017
AAOCA Review MARCH 28, 2017:1592–608
aberrant coronary artery. Int J Cardiol 1991;32: an expert consensus document of the Society of 45. Lee SE, Yu CW, Park K, et al. Physiological and
106–8. Cardiovascular Computed Tomography (SCCT). clinical relevance of anomalous right coronary ar-
J Cardiovasc Comput Tomogr 2015;9:493–513. tery originating from left sinus of Valsalva in
24. Balaguru D, Auslender M, Colvin SB,
adults. Heart 2016;102:114–9.
Rutkowski M, Artman M, Phoon CK. Intraoperative 34. National Cancer Institute. Radiation Risks and
myocardial ischemia recognized by trans- Pediatric Computed Tomography (CT): A Guide for 46. Brothers J, Carter C, McBride M, Spray T,
esophageal echocardiography monitoring in the Health Care Providers. 2012. Available at: http:// Paridon S. Anomalous left coronary artery origin
pediatric population: a report of 3 cases. J Am Soc www.cancer.gov/about-cancer/causes-prevention/ from the opposite sinus of Valsalva: evidence of
Echocardiogr 2000;13:615–8. risk/radiation/pediatric-ct-scans. Accessed January intermittent ischemia. J Thorac Cardiovasc Surg
25. Yilmaz H, Gungor B, Sahin S, Bolca O. A case of 19, 2017. 2010;140:e27–9.
anomalous origin of circumflex artery from right 35. Taylor AJ, Cerqueira M, Hodgson JM, et al. 47. Maddoux GL, Goss JE, Ramo BW, et al. Angina
sinus of Valsalva recognized by three-dimensional ACCF/SCCT/ACR/AHA/ASE/ASNC/NASCI/SCAI/SCMR and vasospasm at rest in a patient with an
transesophageal echocardiography and coronary 2010 appropriate use criteria for cardiac computed anomalous left coronary system. Cathet Car-
computed tomography angiography. Heart Views tomography: a report of the American College of diovasc Diagn 1989;16:95–8.
2014;15:57–9. Cardiology Foundation Appropriate Use Criteria Task
48. Kaku B, Shimizu M, Yoshio H, et al. Clinical
26. Warnes CA, Williams RG, Bashore TM, et al. Force, the Society of Cardiovascular Computed To-
features of prognosis of Japanese patients with
ACC/AHA 2008 guidelines for the management of mography, the American College of Radiology, the
anomalous origin of the coronary artery. Jpn Circ J
adults with congenital heart disease: a report of American Heart Association, the American Society of
1996;60:731–41.
the American College of Cardiology/American Echocardiography, the American Society of Nuclear
Cardiology, the North American Society for Cardio- 49. Nasis A, Machado C, Cameron JD, Troupis JM,
Heart Association Task Force on Practice Guide-
vascular Imaging, the Society for Cardiovascular Meredith IT, Seneviratne SK. Anatomic character-
lines (Writing Committee to Develop Guidelines on
Angiography and Interventions, and the Society for istics and outcome of adults with coronary arteries
the Management of Adults With Congenital Heart
Cardiovascular Magnetic Resonance. J Am Coll Car- arising from an anomalous location detected with
Disease): developed in collaboration with the
diol 2010;56:1864–94. coronary computed tomography angiography. Int
American Society of Echocardiography, Heart
J Cardiovasc Imaging 2015;31:181–91.
Rhythm Society, International Society for Adult 36. Angelini P. Novel imaging of coronary artery
Congenital Heart Disease, Society for Cardiovas- anomalies to assess their prevalence, the causes of 50. Uebleis C, Groebner M, von Ziegler F, et al.
cular Angiography and Interventions, and Society clinical symptoms, and the risk of sudden cardiac Combined anatomical and functional imaging us-
of Thoracic Surgeons. J Am Coll Cardiol 2008;52: death. Circ Cardiovasc Imaging 2014;7:747–54. ing coronary CT angiography and myocardial
e143–263. perfusion SPECT in symptomatic adults with
37. Rajiah P, Setser RM, Desai MY, Flamm SD, abnormal origin of a coronary artery. Int J Car-
27. Budoff MJ, Dowe D, Jollis JG, et al. Diagnostic
Arruda JL. Utility of free-breathing, whole-heart, diovasc Imaging 2012;28:1763–74.
performance of 64-multidetector row coronary
three-dimensional magnetic resonance imaging in
computed tomographic angiography for evalua- 51. Mainwaring RD, Reddy VM, Reinhartz O,
the assessment of coronary anatomy for congen-
tion of coronary artery stenosis in individuals Petrossian E, Punn R, Hanley FL. Surgical repair of
ital heart disease. Pediatr Cardiol 2011;32:418–25.
without known coronary artery disease: results anomalous aortic origin of a coronary artery. Eur J
from the prospective multicenter ACCURACY 38. Ripley DP, Saha A, Teis A, et al. The distribu- Cardiothorac Surg 2014;46:20–6.
(Assessment by Coronary Computed Tomographic tion and prognosis of anomalous coronary arteries
identified by cardiovascular magnetic resonance: 52. Brothers JA, McBride MG, Seliem MA, et al.
Angiography of Individuals Undergoing Invasive
15 year experience from two tertiary centres. Evaluation of myocardial ischemia after surgical
Coronary Angiography) trial. J Am Coll Cardiol
J Cardiovasc Magn Reson 2014;16:34. repair of anomalous aortic origin of a coronary
2008;52:1724–32.
artery in a series of pediatric patients. J Am Coll
28. Halliburton SS, Abbara S, Chen MY, et al., for 39. Feltes TF, Bacha E, Beekman RH 3rd, et al. Cardiol 2007;50:2078–82.
Society of Cardiovascular Computed Tomography. Indications for cardiac catheterization and inter-
53. Osaki M, McCrindle BW, Van Arsdell G,
SCCT guidelines on radiation dose and dose- vention in pediatric cardiac disease: a scientific
Dipchand AI. Anomalous origin of a coronary ar-
optimization strategies in cardiovascular CT. statement from the American Heart Association.
tery from the opposite sinus of Valsalva with an
J Cardiovasc Comput Tomogr 2011;5:198–224. Circulation 2011;123:2607–52.
interarterial course: clinical profile and approach
29. Chinnaiyan KM, Boura JA, DePetris A, et al., for 40. Barriales-Villa R, Morís C, Sanmartín JC, to management in the pediatric population.
the Advanced Cardiovascular Imaging Consortium Fernandez E, Pajin F, Ruiz Nodar JM. [Anomalous Pediatr Cardiol 2008;29:24–30.
Coinvestigators. Progressive radiation dose coronary arteries originating in the contralateral
reduction from coronary computed tomography 54. Gräni C, Benz DC, Schmied C, et al. Hybrid
sinus of Valsalva: registry of thirteen Spanish
angiography in a statewide collaborative quality CCTA/SPECT myocardial perfusion imaging find-
hospitals (RACES)]. Rev Esp Cardiol 2006;59:
improvement program: results from the Advanced ings in patients with anomalous origin of coronary
620–3.
Cardiovascular Imaging Consortium. Circ Car- arteries from the opposite sinus and suspected
diovasc Imaging 2013;6:646–54. 41. Angelini P. Sudden death and coronary concomitant coronary artery disease. J Nucl Car-
anomalies: the importance of a detailed descrip- diol 2017;24:226–34.
30. Ghoshhajra BB, Lee AM, Engel LC, et al. Ra-
tion. Tex Heart Inst J 2011;38:544–6.
diation dose reduction in pediatric cardiac 55. Cheitlin MD, De Castro CM, McAllister HA.
computed tomography: experience from a tertiary 42. Angelini P, Uribe C, Monge J, Tobis JM, Sudden death as a complication of anomalous left
medical center. Pediatr Cardiol 2014;35:171–9. Elayda MA, Willerson JT. Origin of the right cor- coronary origin from the anterior sinus of Valsalva:
onary artery from the opposite sinus of Valsalva in a not-so-minor congenital anomaly. Circulation
31. Bischoff B, Meinel FG, Del Prete A, Reiser MF,
adults: characterization by intravascular ultraso- 1974;50:780–7.
Becker HC. High-pitch coronary CT angiography in
nography at baseline and after stent angioplasty. 56. Kragel AH, Roberts WC. Anomalous origin of
dual-source CT during free breathing vs. breath
Catheter Cardiovasc Interv 2015;86:199–208. either the right or left main coronary artery from
holding in patients with low heart rates. Eur J
Radiol 2013;82:2217–21. 43. Pflederer T, Marwan M, Ropers D, Daniel WG, the aorta with subsequent coursing between aorta
Achenbach S. CT angiography unmasking and pulmonary trunk: analysis of 32 necropsy
32. Long CM, Long SS, Johnson PT, Mahesh M,
catheter-induced spasm as a reason for left main cases. Am J Cardiol 1988;62:771–7.
Fishman EK, Zimmerman SL. Utility of low-dose
high-pitch scanning for pediatric cardiac coronary artery stenosis. J Cardiovasc Comput 57. Taylor AJ, Rogan KM, Virmani R. Sudden car-
computed tomographic imaging. J Thorac Imaging Tomogr 2008;2:406–7. diac death associated with isolated congenital
2015;30:W36–40. coronary artery anomalies. J Am Coll Cardiol 1992;
44. Angelini P, Flamm SD. Newer concepts for
20:640–7.
33. Han BK, Rigsby CK, Leipsic J, et al. Computed imaging anomalous aortic origin of the coronary
tomography imaging in patients with congenital arteries in adults. Catheter Cardiovasc Interv 58. Taylor AJ, Byers JP, Cheitlin MD, Virmani R.
heart disease, part 2: technical recommendations: 2007;69:942–54. Anomalous right or left coronary artery from theYou can also read
