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5 Cardiac CTA of Congenital Coronary and Other Anomalies
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5.4 Case 5.4
5.4.1 History
A 43-year-old male presents with atypical chest
pain. The patient had a previous coronary angiogram, but it was not possible to determine the
course of the left main coronary artery.
5.4.2 Findings
Congenital anomalous origin of the left main
coronary artery from the right sinus of Valsalva is
seen. The left main coronary artery courses posteriorly along the aortic annulus and left atrium
(Fig. 5.4).
5.4.3 Diagnosis
The diagnosis is congenital anomalous origin of
the left main coronary artery from the right sinus
of Valsalva with a posterior course.
5.4.4 Discussion
The left main coronary artery has a posterior
course, which is considered a benign anomaly.
5.4.5 Pearls and Pitfalls
The cardiac CTA has been established as an excellent noninvasive test to identify and classify congenital coronary anomalies, which commonly are
difficult to evaluate with coronary angiography.
Fig. 5.4 (a) Globe 3D
map. The left main
coronary artery
originates from the right
sinus of Valsalva. LAD
left anterior descending
artery, CRX left
circumflex artery. (b)
Volume rendering. LM
left main artery, RCA
right coronary artery
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5.5 Case 5.5
5.5.1 History
A 17-year-old boy collapsed during a football
game, arriving at the hospital in cardiogenic shock.
A coronary angiogram was performed, with failure to cannulate the left main coronary artery. A
cardiac CTA was requested for further evaluation.
5.5.2 Findings
An anomalous origin of the left main coronary
artery (Fig. 5.5a–d) from the right coronary sinus
of Valsalva was seen. The left main had an abnormal ostium, an acute angulation, and an interarterial course. An additional aggravating factor was
that the coronary circulation was left dominant.
a
The left ventricular ejection fraction was estimated at 15%, which was consistent with a
stunned myocardium.
5.5.3 Diagnosis
The diagnosis is interarterial course of a congenital anomalous origin of the left main coronary artery arising from the right sinus of
Valsalva.
5.5.4 Discussion
This type of congenital coronary anomaly is the
most frequently reported to be associated with
sudden death. Coronary anomalies are classified
according to their origin, course, and termination.
c
Fig. 5.5 (a, b) Axial
and volume rendering
(VR): Left main arising
from the right sinus of
Valsalva with an
interarterial course. AO
aorta, POFT pulmonary
outflow tract. (c) cMPR:
left main—abnormal
ostium (arrowhead) and
interarterial course.
(d) 2D composite:
Anomalous left main
coronary artery from the
right sinus of Valsalva.
Left dominant coronary
anatomy: LM left main,
LAD left anterior
descending, LCX left
circumflex, PDA
posterior descending
artery, RCA right
coronary artery
b
d
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Depending on anomaly, they are considered
either benign or potentially lethal, as in this case.
The combination of an interarterial course,
abnormal ostium (often referred to as fish mouth
appearance), and acute angulation is potentially
lethal. The increase in pulmonary arterial pressure during workload causes torque on the left
main coronary artery, resulting in severely diminished arterial flow. The patient survived the acute
event and subsequently underwent successful
coronary reimplantation surgery.
5.5.5 Pearls and Pitfalls
Cardiac CTA (CCTA) has been demonstrated to
be a reliable, accurate noninvasive test in the
assessment of congenital coronary anomalies
and is the preferred diagnostic test. Its use in
newborns and infants is limited due to an accelerated heart rate and the concern about
radiation.
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5.6 Case 5.6
5.6.1 History
A 46-year-old male presented with progressive
shortness of breath.
5.6.2 Findings
There is congenital anomalous origin of the left
main coronary artery from the pulmonary artery
(Fig. 5.6a). The coronary arteries are hypertrophied,
with extensive epicardial and intramyocardial collaterals (Fig. 5.6b). Mild cardiomegaly is seen.
5.6.3 Diagnosis
The diagnosis is anomalous origin of the left
main coronary artery from the pulmonary artery
(ALCAPA) and also known as Bland–White–
Garland syndrome.
5.6.4 Discussion
ALCAPA is one of the most serious congenital
coronary artery anomalies. Approximately 90%
of untreated infants die in the first year of life
and only a few patients survive to adulthood.
Cardiac CT can easily confirm the diagnosis of
ALCAPA and demonstrates collateral circulation between the RCA and LCA and a coronary
steal into the pulmonary artery. There is a
chronic ischemic cardiomyopathy. Note that
the RCA is markedly hypertrophied and is providing retrograde flow into the territory of the
left main coronary artery.
5.6.5 Pearls and Pitfalls
Due to the incidence of high morbidity/mortality,
it is important to properly identify and classify
this congenital coronary anomaly.
Fig. 5.6 (a) Axial
maximum intensity
projection. The left main
coronary artery
originates from the
pulmonary artery
(arrows). There is
marked hypertrophy of
the right coronary artery
(double arrows).
(b) Axial slice through
the heart demonstrates
markedly enlarged septal
perforators (arrows) and
right coronary artery
(double arrows)
(Courtesy of Dr. William
Bugni, Tampa, FL.)
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5.7 Case 5.7
5.7.1 History
A 71-year-old male presented with shortness of
breath.
5.7.2 Findings
Congenital anomalous origin of the right coronary artery from the pulmonary artery is seen
(Fig. 5.7a). The coronary arteries are hypertrophied, with extensive epicardial and intramyocardial collaterals (Fig. 5.7b).
5.7.3 Diagnosis
The diagnosis is potentially lethal congenital
anomalous origin of the right coronary artery
arising from the pulmonary artery.
5.7.4 Discussion
A chronic ischemic cardiomyopathy exists in this
patient. Note that the density in the RCA is higher
than that in the adjacent pulmonary artery, which
is from shunting and retrograde flow from the
coronary collaterals. The septal perforators are
hypertrophied as demonstrated in Fig. 5.7b.
5.7.5 Pearls and Pitfalls
The presence of a congenital coronary anomaly
should be considered in the presence of enlarged
coronary arteries without atheromatous disease.
It is remarkable that this patient has been clinically stable during his lifetime with this
anomaly.
Fig. 5.7 (a) Axial
oblique maximum
intensity projection. The
right coronary artery
originates from the
pulmonary artery
(arrow). (b) Axial slice
through the heart
demonstrates enlarged
septal perforators
(arrows)
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5.8 Case 5.8
5.8.1 History
An 84-year-old female presented with intermittent shortness of breath and a history of an abnormal stress test result.
5.8.2 Findings
There is congenital anomalous origin of the left
main coronary artery from the posterior noncoronary sinus (Fig. 5.8a–d). There is multi-vessel
nonobstructive disease.
5.8.3 Diagnosis
The diagnosis is anomalous origin of the left
main coronary artery from the noncoronary sinus
of Valsalva.
5.8.4 Discussion
Anomalous origin of the left main coronary
artery from the posterior noncoronary sinus of
Valsalva is extremely rare and has been previously noted in only a few reports. Most of these
cases were diagnosed incidentally in asymptomatic patients. Due to the extreme rarity of this
anomaly, the natural history and risk of adverse
events related to this anomaly are unknown.
It was considered a benign anomaly in this
case, based on the patient’s advanced age and no
history of a prior cardiac event. Additional lowrisk indicators are the origin of the left main is
posterior; the ostium has a normal diameter; and
the vessel does not possess an acute angulation.
5.8.5 Pearls and Pitfalls
In order to diagnose and determine clinical relevance of a congenital coronary anomaly, it is
important to carefully observe the origin, course,
dominance, and morphology of the vessels.
Fig. 5.8 (a) 2D map.
Anomalous origin of the
left main from the
posterior noncoronary
sinus of Valsalva
(arrow). (b) 3D volume
rendered vessel tree.
Left main ostium
(arrow). (c, d) Axial and
coronal oblique views of
the ostium of the left
main (arrow)
a
b
cd
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5.9 Case 5.9
5.9.1 History
A 38-year-old male presented with shortness of
breath.
5.9.4 Discussion
The incidence of this anomaly is approximately
0.002% of all patients with congenital heart disease. Patients may present with symptoms of
congestive heart failure, myocardial ischemia,
and sudden death.
5.9.2 Findings
5.9.5 Pearls and Pitfalls
There is an arterial venous malformation, with a
fistula between the LAD and the great cardiac
vein. There is dilatation of the left main coronary
artery and proximal LAD and massive dilatation
of the great cardiac vein (Fig. 5.9).
A dilated vessel in the heart is the major clue
of the presence of an AV fistula. Secondary
signs are dilated cardiac chambers, same contrast density of the arteries and veins, small
caliber thoracic aorta, and dilated pulmonary
artery trunk.
5.9.3 Diagnosis
The diagnosis was coronary AV malformation.
abc
Fig. 5.9 (a) Axial. The left main coronary artery and great cardiac vein are dilated. LM left main coronary artery, GCV
great cardiac vein. (b) Coronal. Dilated LM, left main coronary artery. (c) Volume rendered. AV fistula (arrow)
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5.10 Case 5.10
5.10.1 History
A 57-year-old asymptomatic male presented with
dyslipidemia.
5.10.2 Findings
There is mild disease with calcified plaque in the
left main coronary artery. The mid-LAD has an
intramyocardial course (Fig. 5.10a–c).
5.10.3 Diagnosis
The diagnosis is intramyocardial course of the
mid-segment of the left anterior descending coronary artery (myocardial bridge), with mild CAD.
5.10.4 Discussion
The main coronary arteries are located in the
epicardial surface of the heart. Occasionally, a
coronary artery may have an intramyocardial
course. Myocardial bridging is a clinically
uncommon congenital anomaly characterized
by tunneling of the coronary artery within the
myocardial tissue, usually seen surrounding
the left anterior descending artery. Myocardial
bridging is associated with altered intracoronary hemodynamics during systole and diastole, determined by the severity and the
location of the bridging within the coronary
artery. Patients with myocardial bridging may
present with angina in the absence of other
coronary risk factors.
5.10.5 Pearls and Pitfalls
It is important not to confuse an intramyocardial course with an obstructed and/or occluded
artery. Typically, the intramyocardial segment
has a smaller caliber than the proximal segment. With obstructive coronary artery disease
in which surgical revascularization is warranted, a long myocardial bridge may preclude
grafting of the LAD.
Fig. 5.10 (a) 3D.
Myocardial bridge left
anterior coronary artery
(arrow). (b) Axial.
Intramyocardial course
of the left anterior
coronary artery
(arrows). (c) cMPR:
Intramyocardial course
of the left anterior
coronary artery (arrows)
a
c
b
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5.11 Case 5.11
5.11.1 History
A 45-year-old asymptomatic female presented
for a follow-up study.
5.11.2 Findings
Aneurysmal dilatation of the right sinus of
Valsalva is seen (Fig. 5.11).
5.11.3 Diagnosis
Sinus of Valsalva aneurysm is the diagnosis.
5.11.4 Discussion
Aneurysm of a sinus of Valsalva is a rare congenital cardiac defect that can rupture, causing
heart failure or other catastrophic cardiac
events. The incidence is approximately 0.1–
3.5% of all congenital cardiac anomalies. If the
aneurysm remains unruptured, it occasionally
causes obstruction of coronary flow, resulting
from compression of normal structures. Under
the strain of aortic pressure, the sinus gradually weakens and dilates, causing the formation of an aneurysm. Lack of supporting tissue
(e.g., ventricular septal defect) may contribute
to instability and progressive distortion of the
aortic sinus, often with associated aortic
insufficiency.
5.11.5 Pearls and Pitfalls
Aneurysms of the sinus of Valsalva most often
involve the right sinus (67–85%), followed by the
noncoronary sinus, while an aneurysm of the left
sinus is less common. The CTA is an excellent
noninvasive study for the diagnosis and follow up of these patients.
Fig. 5.11 (a) Axial.
Right sinus of Valsalva
aneurysm (arrow).
(b) Volume rendering.
Right sinus of Valsalva
aneurysm (arrows)
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5.12 Case 5.12
5.12.1 History
A 77-year-old male presented with a history of
atypical chest pain.
5.12.2 Findings
There is a small focal defect in the fossa ovale,
with adjacent redundancy of the atrial septum
(Fig. 5.12a, b).
5.12.3 Diagnosis
The diagnosis is patent foramen ovale (PFO).
5.12.4 Discussion
Most patients with isolated PFO are asymptomatic. Patients may have a history of stroke or transient ischemic event of unknown etiology. PFO is
an anatomic interatrial communication with potential for right-to-left shunt. PFO is a flap like opening between the atrial septa primum and secundum
at the location of the fossa ovalis that persists after
age 1. In utero, the foramen ovale serves as a physiologic conduit for right-to-left shunting. Once the
pulmonary circulation is established after birth,
left atrial pressure increases, allowing functional
closure of the foramen ovale. This is followed by
anatomical closure of the septum primum and septum secundum by age 1.
PFOs are detected in 10–15% of the population by contrast transthoracic echocardiography.
Autopsy studies show a 27% prevalence of
probe-patent foramen ovale. The vast majority of
patients with a PFO experience no symptoms
throughout life. Morbidity, although rare, is predominantly due to paradoxical embolism.
Cerebrovascular ischemic events can be attributed to paradoxical embolism through a patent
foramen ovale. This usually occurs in patients
without other risk factors, although deep venous
thrombosis and hypercoagulable states may significantly increase this risk. Migraine headaches,
especially with aura, have been found to be associated with the presence of a PFO. Up to 50% of
patients with migraine headaches can be found to
have a PFO, compared with a 15–30% prevalence
in the normal population. The reason for this correlation has not been established. PFOs may also
be associated with an atrial septal aneurysm and
other cardiac congenital anomalies.
5.12.5 Pearls and Pitfalls
A small defect in the fossa ovalis, with a redundant atrial septum, may indicate the presence of a
PFO.
Fig. 5.12 (a) Axial.
Patent foramen ovale
(arrow). (b) Axial.
Redundant atrial
septum (arrow)
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