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imaging modality as well. Due to the complexity of CHD,
imaging specialists should understand the importance of
proper patient and protocol selection for CCTA and, ideally,
collaborate with adult congenital specialists to help plan and
successfully execute the CCTA examination.
Coronary Artery Anomalies
Incidence and Defi nitions
Congenital coronary anomalies affect between 0.2 and 5.6 %
of the general population [ 108 – 112 ]. The incidence varies con-
siderably between studies due to a number of factors including
referral bias, fewer autopsies being performed and absence of
strict diagnostic criteria. In addition, coronary anomalies can
vary widely in severity. The presenting symptom for some
anomalies may be sudden cardiac death; others may be entirely
benign and never diagnosed. Some authors have reported that
coronary anomalies may cause anywhere between 12 and 19 %
of deaths among young athletes [ 113 – 115 ].
In the normal coronary circulation, the left main coronary
artery arises from the left ostium (located centrally within
the left sinus of Valsalva) and gives rise to the left anterior
descending artery and circumfl ex artery. The left anterior
descending artery courses along the anterior interventricular
groove, gives off septal perforators and supplies most of the
septum, anterior wall and apex while the circumfl ex artery
wraps posteriorly around the left atrioventricular groove.
The right coronary artery arises from the right ostium (centrally located within the right sinus of Valsalva), giving off
the marginal branch to the right ventricle and then curving
posteriorly in the atrioventricular groove. Angelini and
colleagues [ 116 ] suggest, in the context of known normal
coronary anatomy, the following defi nitions: normal (seen in
>1 % of the general population), normal variant (uncommon
variant but seen in >1 % of the population) and anomaly
(observed in <1 % of the population).
Clinical Signifi cance
Congenital coronary anomalies have been reported as the
cause of chest pain, arrhythmias, myocardial infarction and
sudden cardiac death. Yamanaka and colleagues [ 108 ], in a
survey of 126,595 angiograms, classifi ed 80 % of anomalies
as “benign” and 20 % as being potentially responsible for
serious sequelae. The literature has consistently reported an
association between certain coronary anomalies (such as
anomalous left coronary artery from the pulmonary artery or
origin of the left coronary artery from the right sinus of
Valsalva) and adverse clinical outcomes. Such coronary
variants may account for up to 15 % of sudden cardiac deaths
in athletes [ 114 , 116 ]. Outcomes in milder forms of coronary
anomalies (i.e. “normal variants”) are less well defi ned.
Coronary artery anomalies may also occur in concert with
congenital heart defects (discussed later in this chapter).
Selected Lesions
Abnormal Coronary Origins: ALCAPA
and ARCAPA
Anomalous origin of the left coronary from the pulmonary
artery (ALCAPA, also called the Bland-White-Garland syndrome) is a serious anomaly in which the left coronary artery
arises from the pulmonary artery [ 117 ] (See Fig. 23.16 ).
During fetal life, due to elevated pulmonary vascular resistance, myocardial perfusion is thought to be normal.
However, upon birth, the pulmonary vascular resistance
begins to fall, leading to insuffi cient perfusion of the left
ventricle. Typically there is vigorous collateral formation
from the right coronary artery but despite this, the majority
present in infancy with symptoms of congestive heart failure.
Others may never have symptoms and there are reports of
patients reaching adulthood [ 118 – 121 ] though it appears to
still confer a risk of sudden cardiac death.
Anomalous origin of the right coronary artery from the
pulmonary artery (ARCAPA) is a much rarer anomaly which
also has been associated with sudden cardiac death [ 122 ].
Coronary Artery Origin from the Improper Sinus
Including Single Coronary Arteries
Coronary arteries may be seen to arise anomalously from the
incorrect sinus, with an incidence of 0.92 % for origin of the
right coronary artery from the left sinus and 0.15 % for left
coronary artery from the right sinus [ 123 ]. Single coronary
arteries are even rarer, occurring in approximately 0.024–
0.066 % of the population [ 108 , 124 , 125 ]. Although single
coronary arteries are an isolated fi nding in the majority of
cases, they may be associated with other cardiac defects or
cause sudden cardiac death [ 125 , 126 ].
In broad terms, coronary arteries arising from the incorrect sinus (including single coronary arteries) may take a
number of routes including retrocardiac, retroaortic, infraseptal (supracristal), pre-pulmonary (crossing the right ventricular outfl ow tract) or intra-aortic (between the aorta and
pulmonary artery) [ 123 ] (See Figs. 23.17 , 23.18 , 23.19 and
23.20 ). Of these, the intra-aortic course has been most
consistently implicated as a possible cause of sudden cardiac
death. The ostium of the anomalously placed artery may be
slit-like, possibly predisposing to compression between the
great arteries [ 127 – 129 ]. Sudden cardiac death, when it
occurs, is often precipitated by effort [ 129 ], which likely
explains the increased incidence in athletes [ 113 , 115 ,
130 – 133 ].
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Normal Variants and Minor Coronary Anomalies
Other coronary variants with minor or no signifi cant clinical
sequelae include intramural course (also known as
“myocardial bridging”), “absent” left main (better described
as dual origin of the left anterior descending and left circumfl ex arteries) and geographical variations of the coronary
ostia within the appropriate sinus (See Fig. 23.21 ).
Coronary Anomalies in Congenital Heart Disease
Multiple coronary anomalies have been reported in patients
with d-transposition of the great arteries (d-TGA) [ 134 ].
Most frequently, the left coronary artery arises from the
“anterior facing” sinus and the right coronary artery arises
from the “posterior facing” sinus. As in normal hearts,
there may be single coronary arteries, multiple coronary
ostia or coronary arteries arising from an improper cusp. In
patients who have undergone the arterial switch procedure
(in which the coronary arteries are re-implanted into the
neo-aorta), coronary events are not infrequent with a prevalence of 2–11 % [ 135 – 140 ]. Thus, it is critically important
to evaluate on CCTA the coronary arterial origins and
course to exclude kinking, compression and intimal thickening [ 135 , 141 , 142 ].
Patients with tetralogy of Fallot also commonly have coronary anomalies including single coronary arteries in 5 %,
coronary arteries with anomalous origins in 5 % and a large
conus artery in anywhere from 20 to 40 % [ 73 , 74 , 143 ]. Of
particular concern is the variant in which the left anterior
descending artery arises from the right coronary artery,
crossing the right ventricular outfl ow tract in the process.
This can pose diffi culties during surgery as the left anterior
artery will be at risk when the chest is opened. Careful
assessment for coronary anomalies in this population can
ab
Fig. 23.16 A 2 month old infant presented with failure to thrive. Axial
views ( a ) and ( b ) demonstrating the LAD arising from the pulmonary
artery (anomalous left coronary artery arising from the pulmonary
artery or ALCAPA) (Note that the LCX arises normally from the aorta
(though its origin is posteriorly displaced) and the right coronary artery
is dilated. AO aorta, PA pulmonary artery, LAD left anterior descending
artery, RCA right coronary artery, LCX left circumfl ex artery
Fig. 23.17 This three-dimensional reconstruction demonstrates a single coronary artery, arising from the right coronary cusp. RCC right
coronary cusp, LAD left anterior descending artery, RCA right coronary
artery
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449
help guide surgical repair [ 144 ] and should therefore be
excluded prior to any intervention to the right ventricular
outfl ow tract.
Congenitally corrected transposition (l-TGA) typically
follows a similar pattern to d-TGA, in which the two main
coronary ostia arise from the “facing” sinus. As the coronary arteries not infrequently arise from the improper sinus,
it may be helpful to describe both the origin of the anomalous artery in addition to the territory supplied. The left
coronary artery will, as in normal individuals, supply the
ab
Fig. 23.18 ( a , b ) ( a ) Shown in this three-dimensional reconstruction is
an anomalous right coronary artery originating from the left coronary
cusp. ( b ) Curved multi-planar reconstruction demonstrates the slit-like
origin ( arrow ) of the right coronary artery. LCC left coronary cusp,
LAD left coronary artery, RCA right coronary artery
ab
Fig. 23.19 ( a , b ) Three-dimensional reconstructions demonstrates a
single coronary artery variant in which the left anterior descending
artery courses anterior to the pulmonary artery and the circumfl ex
artery travels behind the aorta. AO aorta, PA pulmonary artery, LCX left
circumfl ex artery, LAD left coronary artery, RCA right coronary artery
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morphologic left ventricle but arises from the “right facing”
sinus. The right coronary artery will supply the morphologic (systemic) right ventricle but arises from the “left facing” sinus [ 145 ] .
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© Springer International Publishing 2016
M.J. Budoff, J.S. Shinbane (eds.), Cardiac CT Imaging: Diagnosis of Cardiovascular Disease,
DOI 10.1007/978-3-319-28219-0_24
CCTA Cardiac Electrophysiology
Applications: Substrate Identification,
Virtual Procedural Planning,
and Procedural Facilitation
Jerold S. Shinbane , Leslie A. Saxon , Rahul N. Doshi ,
Philip M. Chang , and Matthew J. Budoff
Abstract
Cardiovascular computed tomographic angiography (CCTA) is a valuable adjunctive tool in
the diagnosis and treatment of electrophysiologic disease. This modality provides visualization of anatomy important to arrhythmia substrate identifi cation, as well as electrophysiology procedural planning, facilitation, and follow-up. Atrial fi brillation ablation requires a
detailed understanding of an individual patient’s presence and degree of atrial myopathy
and 3-D characterization of the relationships between the left atrium, the surrounding cardiac and extracardiac thoracic structures. Electroanatomic mapping with CCTA image integration has revolutionized catheter–based therapies by allowing for electrical mapping and
ablation to occur on a 3-D map of the patient’s individual atrial anatomy. CCTA imaging
has assumed an important role in decisions and planning of atrial appendage occlusion
device procedures. Ventricular arrhythmias are associated with a spectrum of cardiovascular structural abnormalities which can be identifi ed by CCTA. Cardiomyopathic substrates
associated with ventricular arrhythmias can be identifi ed, although fi brosis imaging is more
evolved with cardiovascular magnetic resonance imaging. CCTA can visualize the coronary
venous system providing roadmaps for lead placement when obstacles to standard
approaches are identifi ed. The role of CCTA will expand due to continued development of
novel cardiac electrophysiology applications for arrhythmogenic substrate identifi cation
and procedural facilitation.
Keywords
Ablation • Atrial Fibrillation • Anomalous Coronary Arteries • Cardiac Electrophysiology
• Cardiomyopathy • Cardiovascular Magnetic Resonance Imaging • Computed Tomography
• Congenital Heart Disease • Electrophysiology • Ventricular Tachycardia
J. S. Shinbane , MD, FACC, FHRS, FSCCT (*)
Division of Cardiovascular Medicine, Department
of Internal Medicine , Keck School of Medicine of the University
of Southern California , 1520 San Pablo Suite 300 , Los Angeles ,
CA 90033 , USA
e-mail: shinbane@usc.edu
2 4
L. A. Saxon , MD, FACC, FHRS • R. N. Doshi , MD, FACC, FHRS
P. M. Chang , MD
Department of Medicine , Division of Cardiovascular Medicine,
Department of Internal Medicine, Keck School of Medicine of the
University of Southern California , Los Angeles , CA , USA
M. J. Budoff , MD
David Geffen School of Medicine at UCLA , Los Angeles
Biomedical Research Institute , Torrance , CA , USA
Electronic supplementary material The online version of this
chapter (doi: 10.1007/978-3-319-28219-0_24 ) contains supplementary
material, which is available to authorized users.
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Introduction
Cardiovascular computed tomographic angiography (CCTA)
can comprehensively assess cardiovascular structure and
function relevant to the assessment, treatment, and follow-up
of patients with electrophysiologically-related disease processes. CCTA provides 3-D visualization of cardiac chambers, coronary vessels, and thoracic vasculature, including
structures particularly important to cardiac electrophysiology such as the coronary veins, pulmonary veins, and left
atrium. This comprehensive technology is extremely useful
for the identifi cation and characterization of cardiovascular
substrates relevant to cardiac electrophysiology, and has
great relevance to treatment of arrhythmias through preprocedure planning, procedural facilitation, and procedural
follow-up.
Technical Issues Related to Image
Acquisition in Patients with Arrhythmias
There are a variety of approaches for imaging patients with
arrhythmia issues based on indication for study, rate and
rhythm at the time of study, presence of a pacemaker or ICD
and programmed lower rate and mode, CT scanner, imaging
and injection protocol, and editing software. Particular
attention to details of patient preparation and image acquisition and processing is important to optimizing CCTA studies
in patients who may have irregular rhythms due to premature
atrial contractions, premature ventricular contractions, or
other arrhythmias including atrial fi brillation and atrial fl utter. Medications to control heart rate and rhythm as well as
pacemaker reprogramming to regularize rhythms may be
useful. Acquisition protocols which compensate for irregular
beats through deletion of short R-R intervals and analysis of
mid-diastolic phases of the R-R interval with an absolute
rather than relative time from the preceding R wave can
improve image quality [ 1 ].
The indication for the CCTA study is important to decisions regarding the correct fi eld of view and target structures
requiring maximum contrast opacifi cation. The contrast circulation time and the injection protocol are important factors to
take into account to achieve maximal opacifi cation of the
region or regions of interest. Assessment of right ventricular
function as well as coronary venous anatomy requires protocols providing adequate contrast enhancement to these structures, while still opacifying other cardiac structures and
coronary arterial anatomy. Multiphase injection protocols
with varying amounts of contrast, diluted contrast, and/or
saline can be administered at variable speeds of delivery to
optimally opacify structures for various electrophysiology
indications (Fig. 24.1 ).
ab
Fig. 24.1 Axial views demonstrating a contrast injection timing protocol for contrast enhancement of the right ventricle and left ventricle,
useful for assessment of right ventricular pathology ( a ), and a protocol
for contrast enhancement of the left ventricle, useful for coronary artery
assessment without streak artifacts in the superior vena cava ( b )
J.S. Shinbane et al.
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