AJR 2005; 185:326-329
© American Roentgen Ray Society
Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Adulthood on CT and MRI
Arati Khanna1,
Drew A. Torigian2,
Victor A. Ferrari3,
Robert J. Bross4 and
Mark A. Rosen2
1 Department of Radiology, Drexel University College of Medicine, Philadelphia,
PA 19102.
2 Department of Radiology, Hospital of the University of Pennsylvania, 3400
Spruce St., Philadelphia, PA 19104-4283.
3 Department of Medicine, Hospital of the University of Pennsylvania,
Philadelphia, PA 19102.
4 Department of Family Practice, Virtua Memorial Hospital of Burlington County,
Mt. Holly, NJ 08060.
Received July 15, 2004;
accepted after revision September 27, 2004.
Address correspondence to D. A. Torigian
(Drew.Torigian{at}uphs.upenn.edu).
Introduction
We report the clinical, CT, and MRI findings of an intermittently
symptomatic elderly woman with the rare congenital syndrome of anomalous
origin of the left coronary artery from the pulmonary artery (ALCAPA) with
marked intercoronary collateral arteries and retrograde filling of the left
coronary arterial system. To our knowledge, this finding as seen on MRI has
not been reported in the literature.
Case Report
A 74-year-old woman with a history of hypertension, borderline
hypercholesterolemia, hypothyroidism, asthma, and a remote history of near
syncopal episodes presented with cough and chronic intermittent nonexertional
chest discomfort that was atypical for angina. No shortness of breath or
diaphoresis was reported. Physical examination was significant for mild
hypertension but was otherwise unremarkable.
Chest radiography showed mild bibasilar atelectasis or scarring. Stress
echocardiography/ECG showed mild left atrial enlargement, normal left and
right ventricular size, borderline left ventricular hypertrophy, normal
biventricular systolic function, mild left ventricular diastolic dysfunction,
mild mitral valve regurgitation, and trace to mild tricuspid regurgitation. In
addition, a right bundle branch block, nonsustained supraventricular
tachycardia during exercise, a hypertensive blood pressure response to
exercise, transient 1-mm horizontal downsloping ST depression just meeting
borderline criteria in the V4–V6, II, III, and aVF leads during exercise
recovery, and moderate–severe hypokinesis of the distal septum during
exercise recovery were noted, although the patient did not experience chest
pain during the study.
Subsequent cardiac catheterization revealed an ejection fraction of 55%,
normal left ventriculography, a patent, large-caliber right coronary artery
(RCA); anomalous origin of the left main coronary artery (LMCA) from the right
coronary sinus, a large-caliber left anterior descending artery (LAD) with
functional occlusion in its proximal portion and reconstitution by left-left
collaterals, and a large-caliber patent left circumflex artery (LCx).
Subsequent contrast-enhanced CT of the chest was performed, showing an
enlarged LMCA that originated from the posterior aspect of the main pulmonary
artery (PA) along with a markedly dilated and tortuous LAD
(Fig. 1A) and an enlarged
tortuous RCA that originated orthotopically from the right coronary sinus of
the aortic root (Fig. 1B), in
keeping with ALCAPA. The remainder of the coronary arterial system appeared
markedly dilated and tortuous (Fig.
1B). Confirmatory cardiac MRI revealed similar anatomic findings
(Figs. 1C,
1D,
1E,
1F,
1G,
1H). In addition, dynamic
gadolinium-enhanced images showed late enhancement of ALCAPA relative to the
PA (Figs. 1E and
1F). Myocardial contractility
and thickness of the ventricles appeared normal.
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Fig. 1A —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Axial enhanced CT shows
dilated left main coronary artery (LMCA) (black arrow) originating
from posterior main pulmonary artery (P) leading to dilated and tortuous left
anterior descending (LAD) artery (white arrow).
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Fig. 1B —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Axial enhanced CT at level
of aortic root shows enlarged tortuous right coronary artery (RCA) and
intercoronary collateral arteries (arrows) along epicardial surface
of heart.
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Fig. 1C —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Confirmatory cardiac MRI
shows anomalous origin of left coronary artery from pulmonary artery (ALCAPA).
Axial thin-section and oblique coronal maximal-intensity-projection (MIP)
postgadolinium fat-suppressed T1-weighted gradient-echo images (TR/TE,
150/1.6/flip angle [FA] 90° and TR/TE, 3.8/0.9/FA 20°, respectively)
show dilated LMCA (black arrow, C) originating anomalously
from posterior main P, leading to dilated and tortuous LAD
(arrows).
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Fig. 1D —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Confirmatory cardiac MRI
shows anomalous origin of left coronary artery from pulmonary artery (ALCAPA).
Axial thin-section and oblique coronal maximal-intensity-projection (MIP)
postgadolinium fat-suppressed T1-weighted gradient-echo images (TR/TE,
150/1.6/flip angle [FA] 90° and TR/TE, 3.8/0.9/FA 20°, respectively)
show dilated LMCA (black arrow, C) originating anomalously
from posterior main P, leading to dilated and tortuous LAD
(arrows).
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Fig. 1E —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Oblique sagittal
postgadolinium fat-suppressed T1-weighted gradient-echo MIP images (TR/TE,
3.8/0.9/FA 20°) during early and late phases of enhancement reveal early
lack of enhancement of ALCAPA (arrows) during enhancement of P with
subsequent late enhancement of ALCAPA, implying retrograde flow of blood
within ALCAPA from RCA.
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Fig. 1F —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Oblique sagittal
postgadolinium fat-suppressed T1-weighted gradient-echo MIP images (TR/TE,
3.8/0.9/FA 20°) during early and late phases of enhancement reveal early
lack of enhancement of ALCAPA (arrows) during enhancement of P with
subsequent late enhancement of ALCAPA, implying retrograde flow of blood
within ALCAPA from RCA.
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Fig. 1G —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Oblique coronal
postgadolinium fat-suppressed T1-weighted gradient-echo MIP image (TR/TE,
3.8/0.9/FA 20°) shows orthotopic dilated and tortuous RCA
(arrow).
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Fig. 1H —74-year-old woman with cough and chronic intermittent
nonexertional chest discomfort atypical for angina. Axial T1-weighted cine
gradient-echo image (TR/TE, 36/3.3/FA 30°) through ventricles shows
high-signal-intensity flow in enlarged intercoronary collateral arteries
(arrow).
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Conservative management rather than surgical intervention was implemented,
and at clinical follow-up 3 years after initial diagnosis at imaging, the
patient did not have new cardiac-related symptomatology.
Discussion
In 1885, Brooks [1] was the
first to show that coronary arteries may anomalously originate from the PA.
ALCAPA, otherwise known as Bland-White-Garland syndrome, is a rare congenital
defect that accounts for 0.25–0.5% of all congenital heart defects
[2]. It most often presents as
an isolated defect, but in 5% of cases it may be associated with other cardiac
anomalies, including atrial septal defect, ventricular septal defect, and
aortic coarctation [3].
Symptoms usually occur in infants after they are 1–2 months old
because of left-to-right shunting from the higher pressure left coronary
arterial system to the lower pressure pulmonary arterial system. This most
often results in death due to circulatory insufficiency from left ventricular
dysfunction or mitral valve incompetence, myocardial infarction, or
life-threatening cardiac dysrhythmias. Before they are 1 month old, however,
physiologic pulmonary arterial hypertension tends to preserve antegrade blood
flow within the left coronary artery (LCA), accounting for the usual lack of
symptomatology in this age group
[4]. Without treatment,
approximately 90% of infants die within the first year of life
[5]. Rarely, however, they
survive into adulthood with clinical presentations varying from symptomatic
chronic mitral insufficiency or global ischemic cardiomyopathy to little or no
symptomatology. Furthermore, the risk for sudden cardiac death due to ischemic
malignant ventricular dysrhythmias exists even in asymptomatic adult patients
[5,
6]. Factors that may lead to
survival beyond infancy include the development of abundant intercoronary
collateral arteries, an alteration in hemodynamics that encourages antegrade
blood flow into the left coronary arterial tree, and a reduction in the area
of the left ventricular myocardium supplied by the LCA
[5].
In general, once the diagnosis of ALCAPA is established, early surgical
repair is undertaken to prevent potential future complications. Surgical
repair procedures that can be implemented include aortocoronary saphenous vein
or internal mammary artery grafting, aortic root reimplantation with or
without a pulmonary flap, or intrapulmonary baffling depending on the anatomic
location of the ostium of ALCAPA
[6]. In the present case,
surgical intervention was not performed and the patient has remained
asymptomatic for the last 3 years since initial diagnosis with conservative
management.
Conventional angiography can detect and depict the course of anomalous
coronary arteries, although this sometimes can be difficult to perform or
interpret, as in the present case. For example, the LMCA that was reported to
arise from the right coronary sinus on the conventional angiographic study
likely represented an enlarged tortuous collateral arterial branch of the RCA.
MRI is highly accurate in the detection of and delineation of the proximal
course of anomalous coronary arteries, and CT may also accurately depict the
origin and proximal course of anomalous coronary arteries
[7–10].
On CT and MRI, previously described imaging findings of ALCAPA in adults
include direct visualization of the origin of the LMCA from the posterior
aspect of the PA, dilatation of the RCA, and visualization of dilated
intercoronary collateral arteries along the external surface of the heart or
within the interventricular septum
[7,
11]. Other differential
diagnostic considerations for dilatation of the coronary arteries include
Kawasaki's disease, coronary arterial fistulas, atherosclerosis, vasculitis
(polyarteritis nodosa or Takayasu's arteritis), scleroderma, Ehlers-Danlos
syndrome, hereditary hemorrhagic telangiectasia, trauma, and hyperlipidemia
[12,
13]. In the present case,
anomalous origin of a dilated and tortuous LMCA from the posterior aspect of
the main PA, along with dilatation and tortuosity of the RCA and external
intercoronary collateral arteries, were well shown on enhanced CT and MRI and
were diagnostic for ALCAPA. In addition, dynamically enhanced MR images showed
a lack of early enhancement of ALCAPA followed by late enhancement, in keeping
with retrograde flow of blood from the RCA to ALCAPA via intercoronary
collateral arteries, which to our knowledge has not been reported on MRI.
Furthermore, the age of clinical presentation of ALCAPA in the present case is
to our knowledge the oldest reported age in adults.
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Introduction
Case Report
