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4 Pediatric Cardiac CTA
https://t.me/med1917
4.14.1 Case 4.30
A 12-year-old male complains of chest pain during baseball practice. His pediatrician refers him
to a pediatric cardiologist and an echocardiogram
shows absence of the normal left coronary artery
origin and its anomalous origin from the right
sinus of Valsalva. The proximal left coronary
appears narrow. Due to the suspected coronary
artery, anomaly CTA of the coronary arteries is
requested (Figs. 4.107, 4.108, 4.109, and 4.110).
103
Fig. 4.108 The left main coronary artery (arrow) is nar-
row as it courses over the AAo and has an elliptical shape
which is an indication that the vessel is intramural, within
the wall of the AAo rather than merely coursing over the
aorta. This course is considered malignant and is more
often symptomatic and the cause of sudden cardiac death
Fig. 4.107 An axial oblique MIP image of the AAo at the
level of the sinuses of Valsalva shows a single coronary
artery origin from the anterior, right sinus, and the proximal
left (arrow) and right (open arrow) coronary arteries. The
proximal left main coronary artery arises at an extremely
acute angle relative to the AAo and the proximal segment is
severely narrowed as it courses between the RVOT and AAo
Fig. 4.109 The common origin of the right and left coronary arteries from the right sinus (ellipse) is shown in a 3D
surface rendered image. The left coronary artery branches
to the left circumflex which courses under the left atria
appendage (LAA) and the left anterior descending (LAD).
The LAD is a short vessel (black arrow); does not reach
the LV apex as is usually expected
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Fig. 4.110 In this “hollow” 3D view of the common
coronary artery origin (bracket), the acute angulation and
proximal stenosis of the left coronary artery is seen (white
arrow). The short LAD (black arrow) is again seen
D.M.E. Bardo
4.14.2 Case 4.31
A 9-year-old girl experienced a syncopal event
while running. An echocardiogram performed in
the emergency room showed normal cardiac
function and left coronary artery origin. The origin of the right coronary artery was not seen. Two
weeks later after a second syncopal event, her
pediatrician ordered a coronary artery CTA after
consultation with a pediatric cardiologist
(Figs. 4.111, 4.112, and 4.113).
Fig. 4.111 A low dose coronary artery CTA was performed. A curved multiplanar reconstruction of the right
coronary artery (RCA) shows its entire course. The artery
arises from the left sinus of Valsalva (white arrow) and
shows a markedly narrow proximal segment which
courses at an acute angle in relation to the ascending
aorta. The RCA courses to the crux of the heart ends
branching to a very small posterior descending artery
(PDA) (black arrow) and a short posterolateral branch
4 Pediatric Cardiac CTA
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105
Fig. 4.112 In a 3D surface rendering, the anomalous
RCA origin is seen arising from a common origin with the
left (black arrow). The proximal intramural segment is
narrow as it courses over the AAo (white arrow)
Fig. 4.113 Looking at the inferior surface of the heart in
a 3D surface rendering the distal branches of the epicardial coronary arteries show the very short PDA segment
(black arrow) and prominent obtuse marginal vessels
extending to supply the inferior wall of the LV (white
arrows)
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D.M.E. Bardo
4.15 Kawasaki Disease
Kawasaki disease is an idiopathic systemic vasculitis which affects young children almost
exclusively. The classic presentation includes
fever, lip and tongue swelling (strawberry
tongue), injected conjunctiva, polymorphous
rash, desquamation of extremities, and cervical
lymphadenopathy.
Kawasaki disease is recognized as a cause of
acquired heart disease in children. The most serious complication of Kawasaki disease is development of coronary artery aneurysms which are
diagnosed in up to 25% of patients who do not
receive treatment and in approximately 15% of
those treated for Kawasaki disease. Subsequent
coronary artery occlusion and myocardial infarction are known to occur in the acute phase of the
disease as well as in the remote time frame.
Though coronary artery aneurysms typically do
not progress coronary artery diameter does not
return to normal. Therefore, long-term clinical
and imaging follow-up is required to monitor
coronary artery aneurysm diameter, development
of coronary artery stenosis and development of
coronary artery aneurysm calcification.
4.15.3 Case 4.32 Presentation
See Figs. 4.114, 4.115, 4.116, and 4.117.
Fig. 4.114 An axial oblique image of the ascending aorta
(AAo) at the level of the sinuses of Valsalva shows the
origin of the left coronary artery (black arrow) and aneurysm of the proximal left anterior descending coronary
artery (LAD) (dotted ellipse) and the normal caliber LAD
distal to the aneurysm
4.15.1 Pearls (•) and Pitfalls (#)
• Coronary artery CTA and MRI are more sensitive than echocardiography for diagnosis and
follow-up imaging of coronary artery aneurysms,
especially in older children and adults, in whom
acoustic windows may be limited.
#
Coronary artery imaging with MRI will
not reveal extent of coronary artery aneurysm
calcification.
4.15.2 Clinical Presentation
A 4-year-old girl presented with fever and tongue
swelling. Her condition deteriorated and echo
showed very poor cardiac function. Coronary
artery CTA was requested to assess coronary
artery patency and potential aneurysm formation.
Fig. 4.115 In a coronal oblique plane, the ascending
aorta (AAo) is shown at the base of the heart. The origin
of the right coronary artery (white arrow) is very small
caliber and the proximal segment of the vessel (bracket) is
not filled with contrast material, indicating occlusion of
the vessel
4 Pediatric Cardiac CTA
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Fig. 4.116 In a basal short axis view of the heart, the
myocardium of the left ventricle (LV) shows heterogeneous attenuation. The lateral wall (white arrows) is normal. The anterior and septal walls (black arrows) are
lower attenuation than the lateral wall due to poor perfusion and infarction
Fig. 4.117 A 3D surface rendered view of the heart
shows the aneurysm of the proximal left anterior descending artery (LAD) (dotted ellipse) and the normal caliber
LAD distal to the aneurysm
107
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. Accessed 23 Nov 2014
Cardiac CTA of Congenital
ab
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Coronary and Other Anomalies
Claudio Smuclovisky
5
5.1 Case 5.1
5.1.1 History
A 48-year-old female presented with a history of
an abnormal myocardial perfusion stress test
result in the anterior wall.
Fig. 5.1 (a) Volume
rendered. LCX left
circumflex coronary
artery, RCA right
coronary artery. (b)
Globe 3D map. The left
circumflex coronary
artery arises from the
right sinus of Valsalva.
LAD left anterior
descending artery, CRX
left circumflex artery,
RCA right coronary
artery
5.1.2 Findings
Congenital anomalous origin of the left circumflex coronary artery is seen arising from the right
sinus of Valsalva. The left circumflex coronary
artery courses posteriorly between the aortic
annulus and the left atrium and continues into the
left atrial–ventricular sulcus (Fig. 5.1).
C. Smuclovisky, MD, FACC, FSCCT
Department of Radiology, Holy Cross Hospital,
South Florida Medical Imaging Cardiovascular
Institute, Fort Lauderdale, FL, USA
e-mail: smuclovisky@gmail.com
© Springer International Publishing AG 2018
C. Smuclovisky (ed.), Coronary Artery CTA, https://doi.org/10.1007/978-3-319-66988-5_5
109
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C. Smuclovisky
5.1.3 Diagnosis
Congenital anomalous origin of the left circumflex
coronary artery from the right sinus of Valsalva.
5.1.4 Discussion
Coronary artery anomalies are observed in 0.3–
1.3% of patients undergoing diagnostic coronary
angiography. It has been reported in approximately 1% of routine autopsy examinations and
in 4–15% of young people who experience sudden death. Since the left circumflex coronary
artery has a posterior course, along the root of the
aorta and left atrium, it is considered a benign
anomaly.
5.1.5 Pearls and Pitfalls
This case represents the most frequent congenital
coronary anomaly.
5 Cardiac CTA of Congenital Coronary and Other Anomalies
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111
5.2 Case 5.2
5.2.1 History
A 45-year-old male presented with atypical chest
pain and normal nuclear perfusion stress test.
5.2.2 Findings
Congenital anomalous high origin of the right
coronary artery (RCA) is seen from the left sinus
of Valsalva (Fig. 5.2a–d). The artery has a narrow
ostium with acute angulation. The RCA is dominant and has an interaterial course between the
aorta and the pulmonary artery.
5.2.3 Diagnosis
The diagnosis is congenital anomalous origin of
the right coronary artery from the left sinus of
Valsalva, with an interarterial course.
5.2.4 Discussion
As discussed in Case 5.5, this type of anomaly
is significant and considered potentially lethal.
Treatment of congenital coronary anomalies
of origin and course in adults is controversial,
particularly without a previously documented
cardiac event or ischemia on a nuclear stress
test. The patient decided against surgical correction and was placed on beta blockers.
5.2.5 Pearls and Pitfalls
Surgical repair would not be considered if the
RCA was nondominant.
Fig. 5.2 (a) Axial: High
origin of the RCA from
the left sinus of Valsalva
(arrow). Congenitally
narrowed ostium with
acute angulation. (b)
cMPR: Interarterial
course of the RCA and
ostium (arrowhead). AO
aorta, POFT pulmonary
outflow tract, RV right
ventricle, LV left
ventricle. (c, d) 2D
composite and volume
rendered cranial view:
Ostium of the RCA
(arrowhead)
a
c
d
b
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C. Smuclovisky
5.3 Case 5.3
5.3.1 History
A 57-year-old male was worked up for CAD and
with a history of congenital anomalous origin of the
LAD identified on a recent coronary angiogram.
5.3.2 Findings
The left anterior descending coronary artery
(LAD) arises in a common trunk with the RCA
from the right sinus of Valsalva (Fig. 5.3a, b).
The LAD courses anteriorly in the plane of the
pulmonary outflow tract and right ventricle.
5.3.3 Diagnosis
Congenital anomalous origin of the LAD from
the right sinus of Valsalva with an anterior
(precardiac) course and clinically considered
benign was seen. The CTA was acquired with
prospective gated axial technique (PGA).
5.3.4 Discussion
The anomalous LAD from the right sinus of
Valsalva may take a septal, anterior (precardiac), posterior, or interarterial course. Of
these, the interarterial course of the LAD
(between the pulmonary artery and aorta) is
the most frequently associated with sudden
death.
5.3.5 Pearls and Pitfalls
Cardiac CTA is currently the best noninvasive
study for the evaluation of congenital coronary anomalies. One can easily evaluate the
anomalous origin, course, and termination of
the artery. To the contrary, this can be very
challenging with coronary angiography and
may necessitate placement of a second catheter in the pulmonary artery. PGA is an excellent low-radiation technique for the evaluation
of these patients.
Fig. 5.3 (a, b) VR. Axial MIP: LAD originating from the right sinus of Valsalva with an anterior (precardiac) course.
(c) Right coronary angiogram performed prior to the CTA
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