Coronary Artery Anomalies: A Comprehensive Review

DOI: 10.1111/jocs.14228
REVIEW ARTICLE
Comprehensive literature review of anomalies of the
coronary arteries
| Arish Noshirwani MBChB1 | Ozhin Karadakhy2 |
Amer Harky MBChB, MRCS, MSc1
Juliana Ang3
1
Department of Cardiothoracic Surgery,
Liverpool Heart and Chest Hospital, Liverpool,
UK
Abstract
Coronary artery anomalies (CAA) are vanishingly rare, affecting less than 1% of the
2
School of Medicine, Manchester University,
UK
general population. While the majority of anomalies do not cause significant
3
symptoms; those that do, have devastating outcomes on the patient. Seventeen
School of Medicine, University of Liverpool,
Liverpool, UK
Correspondence
Amer Harky, MBChB, MRCS, MSc,
Department of Cardiothoracic Surgery,
Liverpool Heart and Chest Hospital, Thomas
Drive, L14 3PE, Liverpool, UK.
Email: [email protected]
percent of deaths from exercise is attributed to CAA, and over half of these present
as sudden death making CAA the second most common cause of sudden cardiac
death in individuals. Computed tomography is generally regarded as the first‐line
investigation due to its superior ability to delineate the course of the coronary vessels
and the surrounding structures, while intravascular coronary angiography can be
helpful in assessing the vessels if there is evidence of stenosis. A multidisciplinary
approach is adopted with patient expectations at the core of the management. Once
the decision to operate has been made, there are multiple techniques available to the
surgeon for the management of anomalous vessels. Surgical repair forms the key
management step in such patients. Currently, surgery in elective cases is associated
with extremely low morbidity and mortality and it is considered a safe option with a
fantastic long‐term prognosis. The ideal approach for assessment and risk stratification remains uncertain, and the inherent variability of coronary anomalies and patient
factors demands a multidisciplinary team with an individualized approach.
KEYWORDS
ALCAPA, cardiac surgery, congenital heart disease, coronary anomalies
1 | INTRODUCTION
The LCA originates from the LSV and it passes between the
pulmonary trunk and left atrial appendage, then bifurcating into the
The normal coronary arteries consist of the right and left coronary
LAD and LCX arteries. Anteriorly, it descends toward the apex of the
arteries; arising from the right coronary sinus of Valsalva (RSV) and
heart as LAD. The LAD also gives rise to the diagonal branches and
left coronary sinus of Valsalva (LSV), respectively. The left coronary
septal perforator branches. The LCX arising from the LCA bifurcation
artery (LCA) begins with the left main stem, which then bifurcates into
winds around the left border to the left AV groove before reaching
left circumflex (LCX) and left anterior descending arteries (LAD).1
the posterior interventricular sulcus and the crux of the heart. The
The right coronary artery (RCA) traverses between the pulmonary
LCX may give rise to the obtuse marginal branches (Figure 1).3
trunk and the right atrial appendage which then descends through the
According to the guiding principles proposed by Angelini et al,4 an
right atrioventricular (AV) groove, after which it divides into its two
anomaly is an abnormality which affects less than 1% of the general
main branches: acute marginal branch and posterior descending artery.2
population. The incidence of coronary artery anomalies (CAAs) can
be recognized as coronary features or patterns that are appreciated
Amer Harky and Arish Noshirwani are equal contributors.
J Card Surg. 2019;1–16.
in less than 1% of the general population. There are still variabilities
wileyonlinelibrary.com/journal/jocs
© 2019 Wiley Periodicals, Inc.
|
1
2
|
HARKY ET AL.
F I G U R E 1 Coronary circulation. Source: Reproduced by permission from Coronary.pdf: Patrick J. Lynch; medical illustrator derivative work:
Fred the Oyster (talk); adaption and further labeling: Mikael Häggström
in the definitions of “anomalous” and “normal variant”, and as a
clinical consequences of CAAs are myocardial infarction (MI), congestive
result, the incidence of CAAs may vary in different literature.
heart failure (CHD), ventricular aneurysms, cardiomyopathy, or SCD.
Incidence of CAAs may also vary due to the unlikelihood of CAAs
Even though echocardiography does not assess the functional
being discovered in healthy individuals. Thus, it is possible that there
status of coronary arteries, it is still used to evaluate the anatomy
may be an overrepresentation of populations that were diagnosed on
and to identify clinically significant coronary anomalies, especially in
invasive angiography and by autopsy.
young patients.8
The most common CAA is the separate origin of the LAD and LCX
The purpose of this literature review is to examine current
arteries, with an incidence of 0.41%, followed by LCX arising from
literature about various forms of coronary anomalies and their
RCA, with an incidence of 0.37%.5
current challenges in diagnosis and surgical management.
Seventeen percent of exercise‐related deaths in athletes are
attributable to CAAs, with half of these cases presenting as sudden
cardiac death (SCD), making it the second most common cause of
SCD among competitive athletes.6 An anomalous origin of a coronary
artery (AOCA) is the CAA most frequently associated with SCD,
2 | CL ASSIFICAT ION OF CORONA RY
ANOMA LIES
particularly a course between the aorta and the pulmonary artery
(PA). Myocardial bridging is another common anomaly with a
The first comprehensive classification of coronary anomalies was
prevalence of 0.15% to 25% on coronary angiography, and with a
attempted by Ogden9 in 1969, in which he classified them into three
prevalence of 5% to 86% at autopsy. The proximal LAD artery is the
main groups as outlined in Table 1. Although this classification was
most common coronary artery involved in myocardial bridging.7
very comprehensive at that time, it lacked the clinical significance of
The abnormal origin and course of anomalous coronary arteries,
some of the critical anomalies.
including left coronary artery from the right sinus of Valsalva (ALCA‐R)
In 1989, Angelini10 introduced the concept of normal variation vs
and anomalous right coronary artery from the left sinus of Valsalva
anomaly using statistical data, which defined an anomaly as coronary
(ARCA‐L), may cause myocardial ischemia, ultimately leading to SCD.
artery anatomy present in less than 1% of the population. Though
The pathophysiology behind ischemia has not been fully determined. It
this was an arguably detailed and logical approach, the most
has been proposed that such CAAs cause myocardial ischemia as the
preferred classification system is the one that was proposed by
arteries are more prone to atherosclerosis. Another theory suggests
Dodge‐Khatami et al11 which has been deemed as the most simplistic
that myocardial ischemia could be due to spasm of the anomalous
and precise classification so far. This was first proposed as part of the
artery. Different CAAs may also cause complications during aortic
international congenital heart surgery nomenclature and database
valve surgery or coronary angioplasty. Patients with coronary artery
project of the Society of Thoracic Surgeons—Congenital Heart
fistulas or aneurysms can be found with aortic root distortion, while
Surgery Database Committee and the European Association of
fistulas can also cause volume overload.5
Cardiothoracic Surgeons. This classification system utilizes a hier-
On the basis of the studies on autopsy patients, coronary anomalies
can cause clinical symptoms such as angina, dyspnea, and syncope. The
archy system to classify coronary anomalies. This is briefly
summarized in Table 2.
| 3
HARKY ET AL.
T A B L E 1 Abbreviations
RSV
Right coronary sinus of Valsalva
LSV
Left coronary sinus of Valsalva
LCA
Left coronary artery
LMS
Left main stem artery
T A B L E 2 Hierarchy of coronary artery classification
Hierarchy
level
Anomalies
1
Coronary artery anomaly
2
Coronary anomaly: anomalous pulmonary origins
of the coronaries (APOC)
LMCA
Left main coronary artery
LCX
Left circumflex artery
LAD
Left anterior descending arteries
RCA
Right coronary artery
PDA
Posterior descending artery
CAA
Coronary artery anomaly
AOCA
Anomalous origin of a coronary artery
PA
Pulmonary artery
Coronary anomaly: coronary artery aneurysms
(CAN)
ALCA‐R
Anomalous left coronary artery from the right sinus of
Valsalva
Coronary anomaly: coronary stenosis
ARCA‐L
Anomalous right coronary artery from the left sinus of
Valsalva
described in the literature,9 and Angelini et al6 found 13 cases of this
CALM
Congenital atresia of the left main coronary artery
variation from 1950 angiograms; an instance of 0.67%.
ALCAPA
Anomalous left main coronary artery from the
pulmonary artery
matic, complaining of angina, dyspnea, syncope, acute myocardial
MI
Myocardial infarction
infarction, and even sudden death.12-14
SCD
Sudden cardiac death
CHF
Congestive heart failure
TTE
Transthoracic echocardiography
CTA
Computed tomography angiography
CT
Computed tomography
(ALCAPA) is also known as Bland‐White‐Garland syndrome and was
MRA
Magnetic resonance angiography
first described in 1956 (Figure 2).
ICA
Invasive coronary angiography
IVUS
Intravascular ultrasound
very rare anomaly. it is a dangerous anomaly as 90% of infants die within
APOCA
Anomalous pulmonary origins of the coronary arteries
the first year of life without definitive surgical intervention.7,16,17 The
CPB
Cardiopulmonary bypass
pulmonary arterial pressure falls soon after birth, and there is
AAOCA
Anomalous aortic origins of the coronary arteries
LVEF
Left ventricle ejection fraction
ECG
Electrocardiogram
Coronary anomaly: anomalous aortic origins of
the coronaries (AAOC)
Coronary anomaly: congenital atresia of the left
main coronary artery (CALM)
Coronary anomaly: coronary arteriovenous
fistulas (CAVF)
Coronary anomaly: coronary artery bridging (CB)
Many patients who presented with this anomaly were sympto-
3.2 | Anomalous left main coronary artery from the
pulmonary artery
An anomalous left coronary artery from the pulmonary artery
With a frequency of 0.008% of the general population, ALCAPA is a
left‐to‐right shunting from the higher pressure LCA to the lower pressure
main PA. This left‐to‐right shunting is known as the steal phenomenon
and causes abnormal left ventricular perfusion. Subsequently, it leads to
myocardial ischemia and infarction. The severity of myocardial ischemia is
determined by the extent of collateral circulation between the RCA and
3 | ANOMA LIES O F TH E LE FT C O RO NARY
ARTERY
The anomalies of the left coronary artery can be divided into
congenital atresia of the left main coronary artery (CALM), anomalous
origin of the left main coronary artery (LMCA), LAD, and LCX.
LCA. There are two types of ALCAPA: adult‐type and infant‐type.18
In the adult‐type, there is adequate development of collaterals
between the LCA and RCA. Patients with adult‐type ALCAPA
syndrome generally survive beyond infancy. Over a long period of
time, the increased volume would lead to prominently dilated
collateral arteries and coronary veins resulting in myocardial
ischemia, left ventricular dysfunction, mitral regurgitation, or
ventricular dysrhythmias.18 These clinical manifestations may be
3.1 | Congenital atresia of the left main coronary
artery
malignant and could potentially lead to SCD.19
In the infant‐type ALCAPA syndrome, infants do not have well‐
established intercoronary collaterals. As a result, the backflow in the
CALM is an extremely rare coronary artery anomaly characterized by
LCA does not have sufficient blood supply to the left ventricular
the ostium of the left coronary artery to be absent or having a closed
myocardium, leading to CHF and mitral insufficiency secondary to
aortic end. The left side of the heart relies on the collateral blood
myocardial infarction.19 Many present with a failure to thrive,
supply form RCA. In Ogden’s study in 1969, only 40 cases were
profuse sweating, dyspnea, pallor, and atypical chest pain while
4
|
HARKY ET AL.
Myocardial bridging is a common anomaly with the proximal LAD
being the most common coronary artery involved in myocardial
bridging and is mostly asymptomatic in patients with no abnormalities observed in functional stress testing.7
For the LCX artery, it could anomalously arise from RSV of the
aorta, and RCA. LCX anomalously originating from the proximal RSV
is a common anomaly (0.32%‐0.67%) which may present with
coronary atherosclerosis. It is considered a benign anomaly if
coronary atherosclerosis is not present.13 The LCX arising from the
proximal branch of the RCA has a frequency of around 0.37%.22
F I G U R E 2 Cardiac magnetic resonance image of an anomalous
circumflex artery arising from the right pulmonary artery (ALCAPA).
Cx, circumflex artery; RPA, right pulmonary artery. Reproduced with
permission15
eating or crying. Without treatment, more than 90% of patients die
4 | AN OMAL IE S O F T HE RIGHT
CORONARY ARTERY
The anomalies of the RCA can be divided into anomalous origin from
PA, LSV, and posterior sinus of Valsalva (PSV).
during the first year of life.19
3.3 | Anomalous left coronary artery from the right
sinus of Valsalva
4.1 | Right coronary artery from the pulmonary
artery
RCA anomalies originating from the PA is an extremely rare anomaly
affecting 0.002% of the population and is far less common than the
ALCA‐R is a rare anomaly with a prevalence of 0.02% to 0.05%. The
primary risk factor is the course the anomalous artery took, with SCD
being common when the artery courses between the aorta and main
PA.7 Taylor et al20 hypothesized that this could be due to the acute
angle take‐off, intramural, or interarterial course of the CAA, finding
origin of the LMCA from the PA.7 Collateral vessels allow blood to
flow from the LCA to RCA, then retrograde to the PA. Syncope,
congestive heart failure, and SCD have been reported with this
anomaly, but generally, patients remain asymptomatic with no
evidence of myocardial damage.13,14
that 84% of patients died suddenly when the anomalous artery
traveled between the great vessels (Figure 3).
4.2 | Anomalous right coronary artery from the left
sinus of Valsava
3.4 | Left main coronary artery from posterior
sinus of Valsalva
ARCA‐L is a rare phenomenon occurring in the range of 0.03% to 0.92%
The origin of the LMCA from the posterior sinus of Valsalva is
which could lead to angina pectoris, acute MI, or even SCD (Figure 4).
of the general population.7,24 It courses between the aorta and PA,
extremely rare (0.0008%) with no fatal clinical consequences and is
considered a benign anomaly.22
4.3 | Anomalous right coronary artery from the
posterior sinus of Valsalva
3.5 | Anomalous origins of the left anterior
descending artery
RCA with an ectopic origin from the PSV is extremely rare (0.003%).
The anomalous origin of LAD includes PA, RSV of the aorta, and RCA.
anomaly has no significant clinical manifestations and is considered a
Another anomaly regarding the intercoronary communications of the
benign CAA.13
LAD is myocardial bridging. While it is very rare for LAD to originate
from the PA, this anomaly could lead to MI and SCD.
Besides the ectopic origin, it has a normal anatomical course. This
A summary of the clinical presentation of the coronary anomalies
is given in Table 3.
An anomalous origin of the LAD from the RSV and the proximal
RCA is extremely rare and has been considered functionally
insignificant but potentially serious.
5 | DIAGNOSIS
Anomalous origin of LAD from the RCA has a strong association
with hypoplasia of the pulmonary infundibulum. This is because the
As coronary artery anomalies are exceedingly rare, diagnosis is most
LAD plays a significant role in the development of the pulmonary
commonly made during further assessment of chest pain, incidentally
conus. Patients may present with dyspnea, chest pain, and palpitation
during the perioperative phase, or at autopsy on patients who suffer
on effort, syncope or epigastric pain, or they may be asymptomatic.23
from SCD.
| 5
HARKY ET AL.
F I G U R E 3 Anomalous origin of the left coronary artery (LCA) from the right coronary sinus of Valsalva (ALCA‐R) with an interarterial
course. A, CT coronary angiogram, (C‐E) 3D CT reconstruction showing the coronary system in various views, (B) schematic representation of
the anomalous LCA. Ao acs, ascending aorta; CT, computed tomography; L, left coronary sinus of Valsalva; LCA, left coronary artery; LCx, left
circumflex artery; LV, left ventricle; P, posterior sinus; R, RCS, right coronary sinus of Valsalva; RCA, right coronary artery; R.i.m., ramus
intermedius; RIVA, ramus interventricularis anterior; TP, truncus pulmonalis. Reproduced with permission21
According to the American College of Cardiology and American
It is not without its shortcomings, and the use of TTE to detect
Heart Association (ACC/AHA) 2018 Guidelines for the management
coronary anomalies is only as accurate as of the operator performing
of adults with congenital heart disease,25 there is no universal
the task. TTE is dependent on patient body habitus for ideal image
consensus regarding the assessment of coronary artery anomalies.
quality and even then, there is difficulty in accurately identifying the
The general principle agreed upon is that if coronary anomalies
necessary vessels. Pelliccia et al26 reported that RCA ostium was
diagnosed or suspected using echocardiography, then it should be
visualized in only 80% of young elite athletes with ideal body habitus.
supported by furthermore detailed scanning techniques.
Brothers et al27 described limited spatial resolution and a lack of
As such, the next recommended investigation is computed
characterization of the anomalous coronaries when compared to CTA
tomography angiography (CTA), followed by invasive coronary
and magnetic resonance angiography (MRA) scanning. The recent
angiography (ICA) and intravascular ultrasound (IVUS) for assess-
(ACC/AHA guidelines of 2018 disregarded TTE, advocating instead for
ment of stenosis or the need for shunting.
25
This is summarized in
Table 4.
CTA or MRA.25 As such, alternative noninvasive methods are a better
choice given their accuracy of characterizing the coronary vessels,
which will also aid to assist in planning interventions.
5.1 | Transthoracic echocardiography
Transthoracic echocardiography (TTE) is a common noninvasive
5.2 | Coronary computed tomography angiography
imaging technique which can be used to evaluate the chambers of the
With the advancement of modern medicine, computed tomography
heart and their functions, valves, and coronary arteries by identifying
(CT) scanning has developed into an ideal diagnostic modality, with
the area of possible ischemia with abnormal motion. This test is a
its rapid and high accuracy in identifying vessels and their
rapid assessment which is widely available and affordable when
surrounding structures. The main advantages of coronary CTA are
compared with the rest of the investigations.
its noninvasive nature while allowing for the visualization of the
6
|
HARKY ET AL.
F I G U R E 4 Anomalous origin of the right coronary artery (RCA) from the left coronary sinus of Valsalva (ARCA‐L) with an interatrial course.
A, Computed tomography, CT coronary angiogram, (B, D‐F) Three‐dimensional CT reconstruction showing the coronary system in various views,
(C) schematic representation of the anomalous RCA (further abbreviations Figure 3). Reproduced with permission21
T A B L E 3 Coronary artery anomalies and their common presenting symptoms
Coronary artery anomalies
Common presentation
Left coronary anomalies
ALCAPA
Adult‐type: myocardial ischemia, left ventricular dysfunction, mitral regurgitation, and ventricular dysrhythmias
Infant‐type: failure to thrive, profuse sweating, dyspnea, pallor, and atypical chest pain while eating or crying,
CHF, and mitral insufficiency secondary to MI.
ALCA‐R
MI, SCD, atherosclerosis
LMCA from PSV
–
LAD from PA
MI, SCD
LAD from RSV
–
LAD from RCA
–
Myocardial bridging in LAD
Asymptomatic
Right coronary anomalies
RCA arising from PA
Asymptomatic (usually), syncope, CHF, and SCD
ARCA‐L
SCD without prior symptoms, coronary atherosclerosis, angina pectoris, syncope, MI, ventricular tachycardia
RCA arising from PSV
Asymptomatic
Abbreviations: ALCAPA, anomalous left main coronary artery from the pulmonary artery; ARCA‐L, right coronary artery from the left sinus of Valsalva;
ALCA‐R, artery from the right sinus of Valsalva; CHF, congestive heart failure; LAD, left anterior descending arteries; LMCA, left main coronary artery;
MI, myocardial infarction; PA, pulmonary artery; PSV, posterior sinus of Valsalva; RCA, right coronary artery; RSV, right coronary sinus of Valsalva.
| 7
HARKY ET AL.
T A B L E 4 Comparison of investigative techniques for visualization of anomalous coronary vessels
Availability
Echocardiogram
Coronary CTA
MRA
ICA
IVUS
Widely available
Readily available
Limited availability
Readily available
Limited availability
Scan time
Rapid
Rapid
Prolonged
Prolonged
Prolonged
Cost
Low
Average
High
High
High
Invasive/ noninvasive
Noninvasive
Noninvasive
Noninvasive
Invasive
Invasive
Visualization of
surrounding structures
Limited
Highly visualized
Good visualization
Poor
visualization.
Poor visualization.
Limitations
Limited accuracy.
Operator dependent.
Dependent on body
shape.
Radiation Iodinated,
nephrotoxic contrast.
Poor resolution vs CTA
Expensive
Limited availability.
Dependent on the patient
following breathing
commands
Radiation
Use of contrast.
Difficulty engaging
anomalous vessel.
Abbreviations: CTA, computed tomography angiography; ICA, invasive coronary angiography; IVUS, intravascular ultrasound; MRA, magnetic resonance
angiography.
vessel, the wall and lumen, and simultaneous assessment of
coronary arteries of the heart. To achieve these images, a small
surrounding vessels, valves, and heart chambers. This combined with
catheter is inserted into a large artery of the body and advanced until
its higher diagnostic accuracy when compared to invasive techniques
the tip is visualized within one of the openings of the coronary
makes it an effective noninvasive alternative.
arteries. Once in position, the contrast agent is injected and X‐rays of
Coronary CTA is not without its disadvantages, which are its cost,
the agents traveling through the coronary arteries and then veins are
availability, reliance on reporter expertise, and ionizing radiation,
obtained. This allows for the visualization of the coronary vessels
especially when used in the investigation of children.28
with a high spatial and temporal resolution.
According to the National Cancer Institute “for an individual
Although viewed as a relatively low‐risk invasive procedure, it has
child, the risks of CT scans are small and the individual risk‐benefit
its limitations when assessing for coronary anomalies. In a large study
balance favors the benefit when used appropriately.”29 Combined
of 23 300 patients undergoing ICA, 98 anomalous coronary arteries
with advances in CT scanners and imaging strategies now providing
were detected (incidence of 0.4%) and in 40.8% the proximal course
low levels of energy exposure and rapid scanning techniques, the
of the anomalous artery could not be identified resulting in patients
risks of radiation exposure to adults and children have been
being referred for further diagnostic imaging to identify the proximal
minimized.
30
course.34 This was also true for Cheezum et al35 who stated that 44%
of anomalous cases were referred for coronary CTA following ICA
(n = 45 of 103).
6 | MAGNETIC RE SO NANCE
AN GIOGRAPH Y
The use of ICA in children is a controversial one, but one that has
been performed for the past few decades.36 It is not without its
complications, from minor issues such as bradycardia, electrocardio-
MRA is best utilized to identify and assess the coronary arteries and
gram changes, and vascular access complications, to serious
provide a functional image of the heart, with the added benefit of no
complications such as ventricular fibrillation.37,38 It is recommended
ionizing radiation or contrast agents, but at the detriment of image
that it be reserved for selective cases where noninvasive methods
quality, longer scan times, and increased costs.
are inconclusive, or for interventional procedures.
When assessed against ICA, multiple studies found that MRA was
It has been hypothesized that ICA has been underestimating the
consistently found to produce highly accurate results, with some
true prevalence of coronary anomalies in the population. A study
studies finding it superior to ICA.31-33
comparing the prevalence of coronary anomalies in a population
When deciding between coronary CTA or MRA as the imaging
undergoing coronary CTA or ICA was carried out by Ghadri et al39
modality of choice, the deciding factor would be based on choices
and found that of 1759 patients, 138 had coronary anomalies which
such as availability, cost, and whether spatial resolution or functional
corresponded to a prevalence of 7.9%, compared to ICA which was
imaging would be of greater benefit.
performed on 9732 patients and 203 anomalies were identified
resulting in a prevalence of 2.1%. The team concluded that coronary
CTA was superior in identifying coronary anomalies and ICA was
7 | IN VASIVE CORON ARY A NGIOGRAP HY
underestimating the true prevalence of anomalies in the general
population. When combined with specialized ICA catheters and IVUS,
ICA is a minimally invasive procedure which utilizes a blood
the detection and characterization of coronary anomalies may
compatible radiocontrast agent and multiple X‐rays to visualize the
improve.35
8
|
HARKY ET AL.
F I G U R E 5 The reimplantation procedure for an anomalous left coronary artery (LCA) from the pulmonary artery, onto the aorta. A, Above
the pulmonary valve, the pulmonary artery (PA) is transected. B, The ostium of the anomalous LCA is resected. C, It is mobilized and
anastomosed end‐to‐end to the aorta. D, To avoid tension, the reimplanted LCA is moved posterior to the pulmonary artery. E, The pulmonary
artery is closed, which is done with a pericardial patch (not shown). Reproduced with permission49
8 | IN TRAVA SC UL AR ULTRASOUND
become an integral part in the assessment of anomalous coronaries at
specialist centers.41,44
IVUS is an imaging technique which utilizes a catheter with a miniature
ultrasound probe attached to the end of it, which is inserted into a large
artery of the body and allows for the visualization of the vessel’s
endothelium from the inside.
9 | MANAGEMENT OF CORONARY
ANOMA LIES
It is frequently used for visualizing the coronary arteries, and
specifically to assess atheromatous plaque build‐up at any one point.
The management of patients with coronary artery anomalies has
As its main advantage is its ability to assess potential mechanisms of
remained controversial and lacks definitive guidelines. Recommenda-
ischemia, a few authors40-43 have recommended its use in assessing
tions have been made by the ACC/AHA in their 2018 guidelines for
for ischemia in coronary anomalies. It is superior from other imaging
the management of adults with congenital heart disease,25 and
techniques in accurately delineating the ostium, its intramural course,
further by Brothers et al.45 Considerable debate remains regarding
and atherosclerosis of the vessel which are not adequately visualized
the surgical management of such patients and is largely based on
using other modalities.
multiple modifiable and nonmodifiable factors.
Although low‐risk, its greatest disadvantage is like all novel
techniques: cost, availability, increase in procedural time, and
interpreter expertise. Unique to IVUS is the difficulty in engaging
the anomalous vessel, given their potential acute angle take‐off, and
9.1 | Anomalous pulmonary origins of the coronary
arteries
ostial narrowing. One must also be aware of the risks of refractory
When a main coronary artery originates from PA, a left‐to‐right
ischemia following insertion of an IVUS catheter into vessel‐causing
shunt is formed from the LCA to the main PA. This results in
spasms, which were only relieved through stenting or removal of the
myocardial perfusion being dependent on collateral circulation, and if
catheter, as described by Angelini and Flamm.44
an adequate collateral system is not developed shortly after birth,
Due to its various limitations and complexity, better alternatives to
myocardial ischemia results.
imaging are available which provide an accurate assessment of the
It is well‐established that the treatment for such presentations
coronary vessel anomalies. Multiple studies are calling for IVUS to
is surgery, and the role of medical therapy is in the stabilization
| 9
HARKY ET AL.
and optimization of the patient and as a bridge to definitive
surgical management.
The development of a dual‐coronary system has been
recognized as the ideal surgical technique of anomalous
pulmonary origin of the coronary arteries (APOCA), and
since 1995, the direct reimplantation of the anomalous artery
into
the
ascending
aorta
has
become
the
technique
of
choice.17,46-48
9.3 | Intrapulmonary tunnel (Takeuchi) technique
for ALCAPA repair
If the LCA arises from a location too distant from the aorta or upon
mobilization is of insufficient length to ensure a tension‐ and kink‐
free anastomosis, the intrapulmonary tunnel (Takeuchi) procedure
can be utilized.
Once CPB is achieved, the origin of the anomalous LCA is established
by performing a transverse incision through PA below its bifurcation.
Following aortic cross‐clamping, a box‐shaped flap is developed from the
transverse incision of PA, and an inverse “L” shape flap is created on the
9.2 | Translocation technique
A median sternotomy is utilized to approach the pericardium,
which is then divided. Cardiopulmonary bypass (CPB) is established and an aortic cross‐clamp is applied high. The PA is
transected high above the pulmonary valve, to allow for access to
the anomalous LCA. The LCA ostium is resected out with a
ascending aorta. A hole is punched out in a face‐to‐face fashion, on both
the pulmonary flap and the aortic wall. The holes are sutured together to
achieve a window between the aorta and PA, and the flap from PA is
used to construct a tunnel from the aortopulmonary window to the
ostium of the LCA. The anterior wall of the PA is reconstructed with an
autologous pericardial patch (Figure 7).16,17,48,50
generous rim of PA tissue, before being mobilized adequately to
avoid tension and kinking of the vessel. A hole is made in the
aorta and the ostium is anastomosed end‐to‐end on the side of
the aorta. The mobilized LCA is pushed posterior to PA, after
which PA is closed with a pericardial patch, as is routine
(Figures 5 and 6).
17,49
9.4 | Oblique coronary artery prolongation with an
aortic flap
Franco et al51 describe an alternative technique for the management
of ALCAPA. In their report, the distance between the origin of the
F I G U R E 6 An anomalous left coronary artery arising from the pulmonary artery (ALCAPA), postsurgery and reimplantation.
Volume‐rendered CT cardiac angiogram images of the corrected left coronary artery, now arising from the aorta with significant dilatation of
the LAD (11.4 mm) and main stem (14.0 mm). CT, computed tomography; Cx, left circumflex artery; LAD, left anterior descending artery.
Reproduced with permission39
10
|
HARKY ET AL.
anomalous LCA and aorta was too large to perform a direct
left lateral side to the posterior aspect. The two flaps were sutured
reimplantation, and an intrapulmonary tunnel would have compro-
together to ensure a tubular extension of the LCA while using the
mised the small main PA. As such, they describe the formation of an
distal end of the PA flap to seal the aortic defect. The PA defect was
oblique flap and formation of a tubular prolongation to ensure a
closed with an autologous pericardial flap (Figure 8).
tension‐free anastomosis.
A median sternotomy is performed and CPB established. The
branches of the pulmonary arteries are snared and the pulmonary
trunk transected. The LCA origin is identified and a coronary button
created with an oblique flap from the posterior wall of the PA. A
10 | SUMMARY OF SURGICAL OPTIONS
FOR APOCA
similar oblique flap is created on the aortic root and the two flaps are
constructed into a tube creating an anastomosis between the LCA
and aorta. The aorta and PA are patched with the autologous
pericardium, and the transected PA is reconnected.
The intended outcome for patients diagnosed with APOCA is to establish
a dual‐coronary system to prevent myocardial ischemia. The techniques
described above provide surgeons with an option for the presentations of
anomalous coronary arteries arising from pulmonary origins. If the
anomalous vessel arises from a reasonable distance from the aorta which
9.5 | Swinging‐door flap technique
does not compromise on tension or kinking, then the translocation
technique can be utilized to good effect. If the anomalous vessel is of
Another technique, described by Sese et al52 and Murthy et al,53 for
insufficient length to ensure a tension‐ and kink‐free anastomosis, the
the surgical management of anomalous origin of the LCA arising from
intrapulmonary tunnel technique, oblique aortic flap technique, and
the lateral, or left anterior aspect of the PA is the swinging‐door
swinging‐door technique can provide a suitable surgical option.
technique. This utilizes the formation of an autologous tube to ensure
a tension‐ and kink‐free anastomosis.
Postoperatively, patient outcomes are primarily dependent on
the extent of myocardial damage. A study by Reul et al17 showed that
A median sternotomy is performed, CPB established, and
of the 65 patients with APOCA in their study, four of them died
myocardial protection achieved with cold blood cardioplegia. The
postoperatively (mortality 6.2%); this was due to severe left
origin of the anomalous LCA is identified. A coronary button along
ventricular failure and they showed that overall the mortality rate
with a flap from the pulmonary wall is created transversely anteriorly
was dependent on the degree of irreversible left ventricular
without damaging the valve commissure. A flap of the aortic wall was
dysfunction. Kazmierczak et al47 and Hoashi et al16 also showed
cut in a similar fashion, starting anteriorly and continuing around the
that the leading cause of operative mortality rates was dependent on
F I G U R E 7 The Takeuchi technique. A, The aorta and main PA are incised as shown. B, An aortopulmonary window is created. C, The PA flap
is sutured as per the dashed lines allowing for (D) blood flow into the LCA orifice. The main PA is reconstructed with a pericardial patch.
Ao, aorta; AP, aortopulmonary; LCA, left coronary artery; PA, pulmonary artery. Reproduced with permission16
| 11
HARKY ET AL.
F I G U R E 8 Swinging‐door flap
technique. (A, top) Dotted lines showing
the flap incisions on the aorta (AO) and
pulmonary artery (PA). (A, bottom) The
anomalous LCA can be seen arising from
the left posterior sinus of PA. B, The AO
and PA flaps are dissected out and (C)
sutured together accordingly to form a
tubular structure (GA Tube). D, It is
implanted into the wall of the Ao and the
defects repaired with a pericardial patch
(PC). GA, great arterial. Reproduced with
permission53
the preoperative left ventricular function, with patients with a left
recent expert consensus guidelines,45 recommends that symptomatic
ventricle ejection fraction less than 30% faring worse.
individuals of AAOCA should be restricted from the competitive
Vouhe et al
54
55
and Levitsky et al
showed that patients did well
at both early and late follow‐up, with Vouhe et al reporting no late
deaths, and a symptom‐free lifestyle for all but one patient.
activity and be offered surgery.
The ACC/AHA 2018 guidelines for the management of adults
with congenital heart disease,25 recommend surgical revasculariza-
It must be remembered that the long‐term impact of surgery is
tion for ALCA‐R with a course between the aorta and PA regardless
still relatively unknown and ongoing assessments and studies must
of symptoms, and ARCA‐L “when coursing between the great arteries
be undertaken to establish both the short and long‐term risks.
or in an intramural fashion” with documented evidence of ischemia.
It is crucial to mention that practices vary from center to center
and must be individualized based on patient factors.
11 | A N O M A L O U S AO R T I C O R I G I N S O F
T HE CO RO NARY ARTE RI E S
The literature is less clear with regard to patients who are
asymptomatic and lead sedentary lifestyles. Most authors would
recommend revascularization in this scenario as well unless the patient
Given the potentially devastating outcome of SCD in patients with
wishes to avoid surgery and adopt a conservative approach. Annual
anomalous aortic origins of the coronary arteries (AAOCA), the most
follow‐up and the adoption of a sedentary lifestyle is recommended.
12
|
HARKY ET AL.
In older individuals, the risk of SCD is insignificant and hence the
management should be like that of ischemia. Each patient should be
assessed individually through appropriate investigations, a multidisciplinary approach, and a thorough discussion with the patient.
Once the decision to operate has been made, multiple techniques
are available.
11.1 | Unroofing
For anomalous vessels traveling in an intramural or interarterial
manner, “unroofing” is the procedure of choice.
After CBP is achieved, an anterior aortotomy is performed before
incising the common wall of the anomalous vessel and the aortic wall
and debulking the common wall to create a new ostium from the
anatomically correct sinus.
This technique has many benefits in that it relocates
the origin of the vessel to the correct sinus, creates a larger
orifice, and eliminates the intramural or interarterial segment
(Figure 9).56
Such a procedure is not without its pitfalls. Aggressive unroofing
can result in an accidental incision being made through the aortic or
coronary wall, resulting in troublesome bleeding. This is particularly
true of short intramural segments.
Another potential complication is the formation of a localized
dissection from exposure of the post unroofing neo‐ostium’s aortic
walls to systemic pressure. This complication can be managed by
the application of simple “tacking” sutures at the neo‐ostium to
ensure the layers of the aortic wall approximate appropriately.
There is a risk of aortic insufficiency as the intramural course
typically courses behind the intracoronary commissure, which is at
risk of being damaged during the procedure. Methods to
circumnavigate this complication involve identifying the pathway
of the anomalous vessel and only unroofing the portion which does
F I G U R E 9 Unroofing technique for an anomalous left coronary
artery from the right sinus of Valsalva with an intramural course. A,
The anomalous origin of the LCA can be seen. B, A neo‐orifice is
formed from the unroofing of the intramural segment. C, Sometimes,
the base of the commissure between the right and left cuff is
involved requiring resuspension. Reproduced with permission56
11.3 | Reimplantation
not put the commissure at risk or repairing the injured commissure
through resuspension.45
An anomalous vessel which has a limited or no intramural course
would best be managed through reimplantation of the anomalous
Ostia into the appropriate sinus of Valsalva.59-62
After achieving CPB and establishing aortic cross‐clamping, the
11.2 | Pulmonary artery translocation
anomalous vessel is mobilized, and its ostium excised with a generous
For anomalous arteries which course between the great arteries in
button of the aortic wall before being anastomosed into the correct
AAOCA, translocation of the PA prevents the anomalous vessel being
sinus of Valsalva.59,60
compressed which could result in ischemia.57
Initially, the proximal pulmonary root is thoroughly mobilized
through dissection, followed by transection of the right PA at its
This is a technically challenging procedure as it requires the
complete mobilization of the anomalous vessel to prevent kinking,
along with precise reimplantation.45
origin. It is then relocated anterior to the aorta where it is
reanastomosed, thereby moving the main PA away from the
anomalous vessel (Figure 10).58
11.3.1 | Ostioplasty
In another version of the operation, the main PA is divided and
The formation of a neo‐ostium for an anomalous vessel in the sinus
relocated onto the left PA through the formation of a neo‐
from which it should have originally exited from is seen as the most
confluence. The original confluence is patched. This has the effect
technically challenging of the surgical options.63,64
of distancing the PA away from the anomalous coronary artery by
moving it left of its original position.45
A matching incision is made in both the aortic sinus and coronary
artery, before joining these and developing the opened areas with a
| 13
HARKY ET AL.
F I G U R E 1 0 Methods of pulmonary artery (PA) translocation. A, The PA is divided distally. B, It is anastomosed to the left PA to allow for
free flow through the anomalous vessel. Alternatively, (C) the right PA is transected near its origin, and (D) brought in front of the aorta (Ao)
where it is reattached with a pericardial patch. Reproduced with permission45
pericardial patch. In this method, a neo‐ostium is fashioned, and the
As such, bypass grafting should be used in limited settings, where
course of the artery is no longer between the great vessels or
the anomalous vessel is already compromised by narrowing from
intramural (Figure 11).
atherosclerosis or in which other techniques would be unlikely.
To facilitate this approach, the main PA is transected to optimize
exposure, and thorough reconstruction of the coronary vessel is
necessitated, giving voice to the claim for being the most technically
challenging procedure.
11.4 | Bypass grafting
12 | SUMMARY OF SURGICAL OPTIONS
FOR AAOCA
For the management of AAOCA, multiple surgical modalities exist.
The general goal of surgery is to provide adequate arterial flow to the
As a final method for managing AAOCA, standard coronary artery
anomalous vessel to ensure perfusion of the myocardium. In patients
bypass techniques may be employed.17 This technique has yielded
presenting with an anomalous vessel taking a short intramural
unsatisfactory results as the anomalous vessel is only compromised
course, ostial reconstruction, or reimplantation would be the
during moments of exercise or stress, and as such would present a
treatment of choice. In patients presenting with an anomalous vessel
competitive pathway for flow for most of the time. This was
with a significant intramural course, unroofing would be the
described by Fedoruk et al,65 where two of the five patients in their
preferred treatment option. Coronary bypass grafting would be
study who underwent coronary artery bypass grafting to treat
appropriate for a vessel with significant atherosclerotic narrowing, or
AAOCA presented with complete occlusion of the grated vessel at
if an alternative technique has failed.
follow‐up.
Some have advocated the ligation of the anomalous vessel to
eliminate the competitive flow66,67; this presents the risk that the
To supplement the primary reconstruction, PA can be translocated, as it is relatively low‐risk and a simple accompanying
procedure.
grafted vessel may not be able to provide adequate flow early on to
Among the published literature, the risk of surgery has been found
replace the flow a nonstenotic native coronary artery would
to be low, with fantastic long‐term follow‐up.58,64 Mainwaring’s team69
65,68
provide.
conducted a study on 115 patients who underwent surgical repair of
14
|
HARKY ET AL.
a mortality rate of up to 90%.9,16 The definitive management of
choice is surgery.16,17,73,74
Patients who survive to adulthood have done so due to the
formation of collateral circulation from right to left coronary
artery.16,17 Harky et al15 demonstrated this example in their report
of a 34‐year‐old woman who presented with ventricular fibrillation
and was found to have an anomalous circumflex artery originating
from the PA. Such patients develop angina and coronary steal and
can present with ST‐elevated myocardial infarction requiring primary
coronary intervention and stenting.73,75 The management for the late
presentation of APOCA is controversial: asymptomatic, high‐risk
patients should be managed with definitive surgery, while those with
an established collateral coronary system with mild evidence of
symptoms can be managed medically or conservatively.17,73,74
AAOCA is largely asymptomatic and does not cause myocardial
ischemia. It is found incidentally on cardiac investigation and certain
types of anomalies have been described as life‐threatening congenital malformations.76,77 An example of this is a case report by
Panda et al76 on a 14‐year‐old patient with ARCA‐L. The patient
presented with exertional chest pain and dizziness. Emergency
coronary artery bypass grafting and surgical repair were carried
out. Meissner et al77 also report on a case of a patient with
anomalous origin of both coronary arteries from the right sinus of
Valsalva, who presented as an emergency with sudden onset
cardiogenic shock. The patient had recurrent ventricular fibrillation
and was treated with direct current cardioversion.
The definitive management for symptomatic patients is surgery,
F I G U R E 1 1 An osteoplasty procedure for an anomalous left
coronary artery from the right sinus of Valsalva. A, The pulmonary
artery and aorta are transected above their commissures. B, The
anomalous left coronary artery has been opened to its bifurcation
from its origin in the aorta. C, A neo‐ostium is formed using a
pericardial patch. Reproduced with permission45
with medical management aiding the optimization of the patient.45
14 | CO N CL US I O N
Coronary artery anomalies are asymptomatic in the vast majority of
AAOCA and found no early or late deaths. Furthermore, 97% of all
the population, thus interventions are varying. Depending on the
symptomatic patients were symptom‐free postoperatively, a fact
severity of anomaly and the age at presentation, surgical intervention
70
which was also found by Romp et al.
We do not know yet if such procedures place the patient’s
is the definite method of treatment and should be imposed to prevent
catastrophic, unexpected major cardiac complications, and death.
coronary vessels at an increased risk of stenosis from scarring or
atherosclerosis, and hence ongoing investigations and assessments
CON F LI CT OF IN TE RES T S
must be undertaken to establish both the short‐ and long‐term risks
of such procedures.
The authors declare that there are no conflict of interests.
ORCI D
13 | E M E R GEN CY P RES E N T ATI O N S
Emergency presentations for coronary anomalies either manifest
Amer Harky
http://orcid.org/0000-0001-5507-5841
R E F E R E N CE S
themselves in early life, as is the case for APOCA, or later in life as
angina, myocardial infarction, congestive heart failure, ventricular
aneurysms, cardiomyopathy, or SCD.17,45
APOCA is most often seen within the first year of life, where a
dual‐coronary network has failed to develop resulting in respiratory
distress, cardiogenic shock, and SCD. If left without treatment, it has
1. Cademartiri F, Marano R, Luccichenti G, et al. Normal anatomy of the
vessels of the heart with 16‐row multislice computed tomography.
Radiol Med. 2004;107(1‐2):11‐21.
2. James TN. Anatomy of the coronary arteries in health and disease.
Circulation. 1965;32(6):1020‐1033.
HARKY ET AL.
3. Levin DC, Fallon JT. Significance of the angiographic morphology of
localized coronary stenoses: histopathologic correlations. Circulation.
1982;66(2):316‐320.
4. Angelini P. Coronary artery anomalies—current clinical issues:
definitions, classification, incidence, clinical relevance, and treatment
guidelines. Tex Heart Inst J. 2002;29(4):271‐278.
5. Young PM, Gerber TC, Williamson EE, Julsrud PR, Herfkens RJ.
Cardiac imaging: Part 2, normal, variant, and anomalous configurations
of the coronary vasculature. Am J Roentgenol. 2011;197(4):816‐826.
6. Angelini P. Coronary artery anomalies: an entity in search of an
identity. Circulation. 2007;115(10):1296‐1305.
7. Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A.
Coronary artery anomalies overview: The normal and the abnormal.
World J Radiol. 2016;8(6):537‐555.
8. Davis JA, Cecchin F, Jones TK, Portman MA. Major coronary artery
anomalies in a pediatric population: incidence and clinical importance.
J Am Coll Cardiol. 2001;37(2):593‐597.
9. Ogden JA. Congenital anomalies of the coronary arteries. Am J
Cardiol. 1970;25(4):474‐479.
10. Angelini P. Normal and anomalous coronary arteries: definitions and
classification. Am Heart J. 1989;117(2):418‐434.
11. Dodge‐Khatami A, Mavroudis C, Backer CL. Congenital Heart
Surgery Nomenclature and Database Project: anomalies of the
coronary arteries. Ann Thorac Surg. 2000;69(4 suppl):270‐297.
12. Musiani A. Left main coronary artery atresia: literature review and
therapeutical considerations. Eur J Cardio‐Thorac Surg. 1997;11(3):
505‐514.
13. Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595
patients undergoing coronary arteriography. Cathet Cardiovasc Diagn.
1990;21(1):28‐40.
14. Roberts WC. Major anomalies of coronary arterial origin seen in
adulthood. Am Heart J. 1986;111(5):941‐963.
15. Harky A, Bashir M, Garner M, Hsia T. Anomalous origin of the
circumflex coronary artery presenting with ventricular fibrillation
cardiac arrest. BMJ CaseRep. 2017:pii: bcr2016219184. 10.1136/bcr‐
2016‐219184
16. Hoashi T, Kagisaki K, Okuda N, Shiraishi I, Yagihara T, Ichikawa H.
Indication of Takeuchi technique for patients with anomalous origin
of the left coronary artery from the pulmonary artery. Circ J.
2013;77(5):1202‐1207.
17. Reul RM, Cooley DA, Hallman GL, Reul GJ. Surgical Treatment of
Coronary Artery Anomalies. Tex Heart Inst J. 2002;29(4):299‐307.
18. Peña E, Nguyen ET, Merchant N, Dennie C. ALCAPA syndrome: not
just a pediatric disease. Radiographics. 2009;29(2):553‐565.
19. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left
coronary artery from the pulmonary trunk. Its clinical spectrum,
pathology, and pathophysiology, based on a review of 140 cases with
seven further cases. Circulation. 1968;38(2):403‐425.
20. Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated
with isolated congenital coronary artery anomalies. J Am Coll Cardiol.
1992;20(3):640‐647.
21. Heermann P, Heindel W, Schülke C. Coronary artery anomalies:
diagnosis and classification based on cardiac CT and MRI (CMR):
from ALCAPA to anomalies of termination. RoFo. 2017;189(1):29‐38.
22. Frescura C, Basso C, Thiene G, et al. Anomalous origin of coronary
arteries and risk of sudden death: a study based on an autopsy
population of congenital heart disease. Hum Pathol. 1998;29(7):
689‐695.
23. Shyu KG, Tseng CD, Chiu IS, et al. Infundibular pulmonic stenosis
with intact ventricular septum: a report of 15 surgically corrected
patients. Int J Cardiol. 1993;41(2):115‐121.
24. Sloan K, Majdalany D, Connolly H, Schaff H. Inferior wall myocardial
infarction caused by anomalous right coronary artery. Can J Cardiol.
2008;24(12):S102‐S103.
| 15
25. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline
for the management of adults with congenital heart disease:
executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice
Guidelines. J Am Coll Cardiol. 2019;73(12):1494‐1563.
26. Pelliccia A, Spataro A, Maron BJ. Prospective echocardiographic
screening for coronary artery anomalies in 1,360 elite competitive
athletes. Am J Cardiol. 1993;72(12):978‐979.
27. Brothers JA, Whitehead KK, Keller MS, et al. Cardiac MRI and CT:
differentiation of normal ostium and intraseptal course from slitlike
ostium and interarterial course in anomalous left coronary artery in
children. Am J Roentgenol. 2015;204(1):W104‐W109.
28. Budoff MJ, Dowe D, Jollis JG, et al. Diagnostic performance of
64‐multidetector row coronary computed tomographic angiography
for evaluation of coronary artery stenosis in individuals without
known coronary artery disease. J Am Coll Cardiol. 2008;52(21):
1724‐1732. Accessed July 28, 2019.
29. National Cancer Institute [Internet]. 2002. Radiation Risks and
Pediatric Computed Tomography. https://www.cancer.gov/about‐
cancer/causes‐prevention/risk/radiation/pediatric‐ct‐scans
30. Ghoshhajra BB, Lee AM, Engel L‐C, et al. Radiation dose reduction in
pediatric cardiac computed tomography: experience from a tertiary
medical center. Pediatr Cardiol. 2014;35(1):171‐179.
31. McConnell MV, Ganz P, Selwyn AP, Li W, Edelman RR, Manning WJ.
Identification of anomalous coronary arteries and their anatomic
course by magnetic resonance coronary angiography. Circulation.
1995;92(11):3158‐3162.
32. Taylor AM, Thorne SA, Rubens MB, et al. Coronary artery imaging in
grown up congenital heart disease: complementary role of magnetic
resonance
and
X‐ray
coronary
angiography.
Circulation.
2000;101(14):1670‐1678.
33. Post JC, van Rossum AC, Bronzwaer JGF, et al. Magnetic resonance
angiography of anomalous coronary arteries: a new gold standard for
delineating the proximal course? Circulation. 1995;92(11):3163‐3171.
34. Barriales‐Villa R, Morís C, Sanmartín JC, Fernández E, Pajín F, Ruiz
Nodar JM. Registro de anomalías congénitas de las arterias
coronarias con origen en el seno de Valsalva contralateral en 13
hospitales españoles (RACES). Rev Esp Cardiol. 2006;59(6):620‐623.
35. Cheezum MK, Ghoshhajra B, Bittencourt MS, et al. Anomalous origin
of the coronary artery arising from the opposite sinus: prevalence
and outcomes in patients undergoing coronary CTA. Eur Heart J
Cardiovasc Imag. 2017;18(2):224‐235.
36. Lederlin M, Thambo J‐B, Latrabe V, et al. Coronary imaging techniques
with emphasis on CT and MRI. Pediatr Radiol. 2011;41(12):1516‐1525.
37. Vranicar M, Hirsch R, Canter CE, Balzer DT. Selective coronary
angiography in pediatric patients. Pediatr Cardiol. 2000;21(3):285‐288.
38. Goo HW. Coronary artery imaging in children. Korean J Radiol.
2015;16(2):239‐250.
39. Ghadri JR, Kazakauskaite E, Braunschweig S, et al. Congenital
coronary anomalies detected by coronary computed tomography
compared to invasive coronary angiography. BMC Cardiovasc Disord.
2014;14(1):81.
40. Angelini P, Walmsley RP, Libreros A, Ott DA. Symptomatic
anomalous origination of the left coronary artery from the opposite
sinus of valsalva. Tex Heart Inst J. 2006;33(2):171‐179.
41. Yildiz O, Karabay KO, Akman C, Aytekin V. Anomalous origin of the
left main coronary artery from the right coronary artery with a
preaortic course. Tex Heart Inst J. 2015;42(3):243‐245.
42. Schrale RG, Channon KM, Ormerod OJ. IVUS‐guided evaluation and
percutaneous intervention in an anomalous left main coronary artery.
J Invasive Cardiol. 2007;19(7):E195‐E198.
43. Driesen BW, Warmerdam EG, Sieswerda G‐JT, et al. Anomalous
coronary artery originating from the opposite sinus of Valsalva
(ACAOS), fractional flow reserve‐ and intravascular ultrasound‐guided
16
|
management in adult patients. Catheter Cardiovasc Interv. 2018;92:
68‐75.
44. Angelini P, Flamm SD. Newer concepts for imaging anomalous aortic
origin of the coronary arteries in adults. Catheter Cardiovasc Interv.
2007;69(7):942‐954.
45. Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD,
Tweddell JS. Expert consensus guidelines: Anomalous aortic origin of
a coronary artery. J Thorac Cardiovasc Surg. 2017;153(6):1440‐1457.
46. Turley K, Szarnicki RJ, Flachsbart KD, Richter RC, Popper RW,
Tarnoff H. Aortic implantation is possible in all cases of anomalous
origin of the left coronary artery from the pulmonary artery. Ann
Thorac Surg. 1995;60(1):84‐89.
47. Kazmierczak PA, Ostrowska K, Dryzek P, Moll JA, Moll JJ. Repair of
anomalous origin of the left coronary artery from the pulmonary
artery in infants. Interact Cardiovasc Thorac Surg. 2013;16(6):797‐801.
48. Fagan T, Palacios‐Macedo A, Nihill M, Fraser CJ, Cooley D. Coronary
artery anomalies in pediatric patients. Coronary artery anomalies: a
comprehensive approach. Philadelphia, PA: Lippincott Williams &
Wilkins; 1999:151‐171.
49. Grace RR, Angelini P, Cooley DA. Aortic implantation of anomalous
left coronary artery arising from pulmonary artery. Am J Cardiol.
1977;39(4):609‐613.
50. Takeuchi S, Imamura H, Katsumoto K, et al. New surgical method for
repair of anomalous left coronary artery from pulmonary artery.
J Thorac Cardiovasc Surg. 1979;78(1):7‐11.
51. Franco JJ, Mesia CI, Escarcega RO, Stevens R, Moulick A. Oblique
coronary artery prolongation approach in anomalous left coronary
artery from the pulmonary artery in a low‐birth‐weight neonate.
World J Pediatr Congenit Heart Surg. 2015;6(2):328‐331.
52. Sese A, Imoto Y. New technique in the transfer of an anomalously
originated left coronary artery to the aorta. Ann Thorac Surg.
1992;53(3):527‐529.
53. Murthy KS, Krishnanaik S, Mohanty SR, Varghese R, Cherian KM. A
new repair for anomalous left coronary artery. Ann Thorac Surg.
2001;71(4):1384‐1386.
54. Vouhé PR, Baillot‐Vernant F, Trinquet F, et al. Anomalous left
coronary artery from the pulmonary artery in infants. Which
operation? When? J Thorac Cardiovasc Surg. 1987;94(2):192‐199.
55. Levitsky S, van der Horst RL, Hastreiter AR, Fisher EA. Anomalous
left coronary artery in the infant: recovery of ventricular function
following early direct aortic implantation. J Thorac Cardiovasc Surg.
1980;79(4):598‐602.
56. Frommelt PC, Frommelt MA, Tweddell JS, Jaquiss RDB. Prospective
echocardiographic diagnosis and surgical repair of anomalous origin
of a coronary artery from the opposite sinus with an interarterial
course. J Am Coll Cardiol. 2003;42(1):148‐154.
57. Gulati R, Reddy VM, Culbertson C, et al. Surgical management of
coronary artery arising from the wrong coronary sinus, using
standard and novel approaches. J Thorac Cardiovasc Surg. 2007;
134(5):1171‐1178.
58. Mainwaring RD, Reddy VM, Reinhartz O, et al. Anomalous aortic
origin of a coronary artery: medium‐term results after surgical repair
in 50 patients. Ann Thorac Surg. 2011;92(2):691‐697.
59. Rogers SO, Leacche M, Mihaljevic T, Rawn JD, Byrne JG. Surgery for
anomalous origin of the right coronary artery from the left aortic
sinus. Ann Thorac Surg. 2004;78(5):1829‐1831.
60. Lee MK, Choi JB, Kim KH, Kim KS. Surgery for anomalous origin
of the left main coronary artery from the right sinus of valsalva,
in association with left main stenosis. Tex Heart Inst J.
2009;36(4):309‐312.
61. Jaggers J, Lodge AJ. Surgical therapy for anomalous aortic origin of
the coronary arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg
Annu. 2005;8(1):122‐127.
HARKY ET AL.
62. Law T, Dunne B, Stamp N, Ho KM, Andrews D. Surgical results and
outcomes after reimplantation for the management of anomalous
aortic origin of the right coronary artery. Ann Thorac Surg.
2016;102(1):192‐198.
63. Han WS, Park PW, Cho SH. Neo‐ostium formation in anomalous
origin of the left coronary artery. Korean J Thorac Cardiovasc Surg.
2011;44(5):355‐357.
64. Alphonso N, Anagnostopoulos PV, Nölke L, et al. Anomalous coronary
artery from the wrong sinus of valsalva: a physiologic repair strategy.
Ann Thorac Surg. 2007;83(4):1472‐1476.
65. Fedoruk LM, Kern JA, Peeler BB, Kron IL. Anomalous origin of the
right coronary artery: right internal thoracic artery to right coronary
artery bypass is not the answer. J Thorac Cardiovasc Surg.
2007;133(2):456‐460.
66. Sabik JF, Lytle BW, Blackstone EH, Khan M, Houghtaling PL,
Cosgrove DM. Does competitive flow reduce internal thoracic artery
graft patency? Ann Thorac Surg. 2003;76(5):1490‐1497.
67. Berger A, MacCarthy PA, Siebert U, et al. Long‐term patency of
internal mammary artery bypass grafts: relationship with preoperative severity of the native coronary artery stenosis. Circulation.
2004;110(11 suppl 1):36‐40.
68. Navia D, Cosgrove DM, Lytle BW, et al. Is the internal thoracic artery
the conduit of choice to replace a stenotic vein graft? Ann Thorac Surg.
1994;57(1):40‐44.
69. Mainwaring RD, Murphy DJ, Rogers IS, et al. Surgical repair of 115
patients with anomalous aortic origin of a coronary artery from a single
institution. World J Pediatr Congenit Heart Surg. 2016;7(3):353‐359.
70. Romp RL, Herlong JR, Landolfo CK, et al. Outcome of unroofing
procedure for repair of anomalous aortic origin of left or right
coronary artery. Ann Thorac Surg. 2003;76(2):589‐596.
71. Mainwaring RD, Reddy VM, Reinhartz O, Petrossian E, Punn R,
Hanley FL. Surgical repair of anomalous aortic origin of a coronary
artery. Eur J Cardiothorac Surg. 2014;46(1):20‐26.
72. Law W, DiBardino DJ, Raviendren R, Devaney E, Davis C. Anomalous
aortic origin of the left coronary artery: in‐hospital cardiac arrest and
death despite bed rest. World J Pediatr Congenit Heart Surg.
2014;5(4):580‐582.
73. Ali R, Tahir A, Shakhatreh MI, Posina KV. Anomalous origin of
coronary arteries arising from the right coronary cusp: a rare
presentation. Cureus. 2018;10(4):e2535. https://doi.org/10.7759/
cureus.2535
74. Moodie DS, Fyfe D, Gill CC, et al. Anomalous origin of the left
coronary artery from the pulmonary artery (Bland‐White‐Garland
syndrome) in adult patients: long‐term follow‐up after surgery. Am
Heart J. 1983;106(2):381‐388.
75. Marchesini J, Campo G, Righi R, Benea G, Ferrari R. Coronary artery
anomalies presenting with ST‐segment elevation myocardial infarction.
Clin Pract. 2011;1(4):e107. https://doi.org/10.4081/cp.2011.e107
76. Panda BR, Prabhu A, Provenzano S, Karl T. Use of extracorporeal life
support for emergency coronary artery bypass grafting. Interact
Cardiovasc Thorac Surg. 2013;16(6):897‐899.
77. Meissner A, Trappe HJ, Plehn G. Acute cardiogenic shock with
inferior myocardial infarction associated with an abnormal origin of
the coronary arteries. Dtsch Med Wochenschr. 2007;132(4):145‐148.
How to cite this article: Harky A, Noshirwani A, Karadakhy O,
Ang J. Comprehensive literature review of anomalies of the
coronary arteries. J Card Surg. 2019;1‐16.
https://doi.org/10.1111/jocs.14228