EAJM 2010; 42: 100-2
A Case of Anomalous Left Coronary Artery Arising From the
Pulmonary Artery in Adulthood: Multidetector Computed
Tomography Coronary Angiography Findings
Erişkinde Pulmoner Arterden Kaynaklanan Anomalili Sol Koroner Arter: Bir Olgunun Çok
Kesitli Bilgisayarlı Tomografi Koroner Anjiografi Bulguları
Veysel Akgun, Bilal Battal, Bulent Karaman, Fatih Ors, Mutlu Saglam, Mustafa Tasar
Gulhane Military Medical School, Department of Radiology, Etlik, Ankara, Turkey
Abstract
Anomalous left coronary artery arising from the pulmonary artery (ALCAPA) is a
rare but very serious congenital coronary artery anomaly. Multidetector computed
tomography (MDCT) coronary angiography has recently become the gold stand-
ard for depicting anatomical variations and anomalies of the coronary arteries
because the origin and course of anomalous arteries can be demonstrated very
accurately by this technique. In this report, we present a case of 22-year-old female
who was admitted to our emergency department with cardiac arrest. In the course
of diagnosis, MDCT coronary angiography revealed a left coronary artery arising
from the pulmonary artery as well as marked dilatation of the coronary arteries.
Key Words: ALCAPA, Anomalous left coronary artery, MDCT coronary angiog-
raphy, Pulmonary artery
Introduction
Anomalous origin of the left main coronary artery (LCA) from the
pulmonary artery (PA) is a rare malformation also known as Bland-
White-Garland syndrome. Very few individuals with anomalous left
coronary artery arising from the pulmonary artery (ALCAPA) survive
until adulthood without surgical repair; furthermore, approximately
90% die suddenly at a mean age of 35 years [1]. In the most common
form of this disease, the LCA arises from the PA, and the right coronary
artery (RCA) arises normally from the aorta. This configuration creates
a collateral circulation between the RCA and LCA and a coronary “steal”
phenomenon into the PA [2]. In this article, we present clinical and
MDCT coronary angiography findings of a 22-year-old female patient
with ALCAPA with no history of chest pain.
Case Report
A 22-year-old female with no history of chest pain was admitted
to our emergency department with sudden cardiac arrest. Following
the reinstitution of the cardiopulmonary circulation and medical
management, a transthoracic echocardiographic examination was
performed, revealing an obvious dilatation of the coronary arteries.
The patient was referred to the radiology department on suspicion of
Received: May 25, 2010 / Accepted: May 25, 2010
Summary of this manuscript presented in: 29. National Radiology Congress,1-5 November 2008, Antalya/Turkey
Correspondence to: Bilal Battal, Gulhane Military Medical School, Department of Radiology, 06018, Etlik, Ankara, Turkey
Gsm: +90 533 433 06 67 Fax: +90 312 326 05 51 e-mail: bilbat_23@yahoo.com
doi:10.5152/eajm.2010.28
Case Report
Özet
Sol koroner arterin ana pulmoner arterden köken alması, nadir ancak tehli-
keli konjenital koroner arter anomalilerinden birisidir. Çok kesitli bilgisayarlı
tomografi (ÇKBT) koroner anjiyografi ile anomalili arterlerin kökeni ve seyri
mükemmel olarak ortaya konulabildiği için, özellikle koroner arterlerin ana-
tomik varyasyonları ve anomalilerini göstermede bu teknik son zamanlarda
altın standart haline gelmiştir. Bu yazıda, acil servisimize kardiyak arrest ne-
deniyle kabul edilen 22 yaşında bir kadın vakayı sunuyoruz. Tanı sürecinde,
ÇKBT koroner anjiyografi, ana pulmoner arterden çıkan bir sol koroner arter
ile koroner arterlerde belirgin genişlemeyi göstermiştir.
Anahtar Kelimeler: ALCAPA, Anomalili sol koroner arter, ÇKBT koroner anji-
yografi, Pulmoner arter
coronary artery anomaly and scanned with a 16-detector CT scanner
(Philips Medical Systems MX 8000 IDT Multislice CT System-V 2.5). The
informed consent of the patient’s parents was obtained before radio-
logical examination. Scan parameters were 120 kV, 340 mAs, 420 msec
rotation time with a slice thickness of 1 mm and increments of 0.5 mm,
using a detector collimation of 16x0.75 mm (pitch: 0.2). The heart rate
was 67 beats per minute during acquisition. One hundred milliliters of
non-ionic, iodinated, low-osmolar contrast medium was injected thro-
ugh the antecubital vein at a rate of 5 ml/sec. Subsequently, a bolus of
40 ml saline solution was administered at a rate of 2.5 ml/sec to avoid
possible contrast artifacts at the right atrial entrance. An automatic
bolus tracking method was used to optimize visualization.
MDCT coronary angiography images demonstrated a LCA origi-
nating from the inferior side of the main PA. MDCT showed enormo-
us sinus vessels originating from a normally situated right coronary
artery with collateral circulation to the LCA. The lumen of the LCA
was fully enhanced, and a little enhancement was noticed in the
lumen of the PA, showing retrograde blood flow toward the PA. The
RCA arose from the aorta. All coronary arteries and their branches
were ectatic and showed mildly tortuous courses. Collateral circu-
lation between the RCA and LCA as well as both a coronary “steal”
phenomenon (from RCA to LCA) and a pulmonary “steal” phenome-
non (from LCA to main PA) were readily demonstrated (Figure 1a-c).
EAJM 2010; 42: 100-2
A B
Figure 1. Curved maximum-intensity-projection (MIP) (A), 3D cardiac (B), and volume rendered cardiac (C) MDCT images show abnormal LCA (black arrowhead) originating
from the pulmonary artery (PA) receiving collaterals from a normally originating, dilated and tortuous right coronary artery (white arrowheads). There is minor enhancement in
the lumen of the pulmonary artery, indicating retrograde blood flow toward the PA. (A = aorta), (white arrows = LAD).
The patient died from massive cerebral and cerebellar infarc-
tion 12 hours after the MDCT coronary angiography examination.
Therefore, surgical correction could not be performed.
Discussion
Coronary artery anomalies are common in the general popu-
lation. They may accompany other congenital heart diseases and
can cause myocardial ischemia, a decrease in life expectancy, or
sudden death. Most patients with coronary artery anomalies are
asymptomatic [1]. ALCAPA is one of the most serious congenital
coronary artery anomalies. It has an estimated prevalence of one in
300,000 live births [3]. Most of the affected patients show symptoms
in infancy and early childhood. Approximately 90% of untreated
infants die in the first year of life, and only a few patients survive until
adulthood [3].
Abnormal embryonic development may lead to positional ano-
malies of the coronary arteries. The coronary artery buds appear on
about the 12th day of life after the division of the truncus arteriosus,
which leads to the separation of the aorta and pulmonary artery [4].
Coronary artery anomalies can arise secondary to either malrotation
of the spiral septum dividing the truncus or malpositioning of the
coronary buds themselves. Soloff described the four possible ano-
malous coronary artery connections to the pulmonary artery [5]. The
most common is anomalous left coronary artery from the pulmonary
artery (ALCAPA), followed by anomalous right coronary artery from
the pulmonary artery (ARCAPA), origin of both coronaries from the
pulmonary trunk, and origin of an accessory coronary artery from
the pulmonary trunk. The relatively high incidence of ALCAPA is exp-
lained by the proximity of the left coronary bud to the pulmonary
artery sinus.
Conventional angiography is a traditional technique that has
been used for many decades to diagnose coronary artery anoma-
lies [2]. However, MDCT has recently become accepted as the main
technique for evaluation of coronary anomalies [6, 7]. Several case
reports describing MDCT coronary angiography findings in ALCAPA
have been published previously [1, 7]. In our experience, unlike
Akgun et al. ALCAPA: MDCT Angiography Findings 101
C
conventional angiography, MDCT coronary angiography allows the
accurate and noninvasive depiction of coronary artery anomalies.
MDCT has been determined to be superior to conventional angiog-
raphy for delineating the origin, course, and termination of abnormal
coronary arteries [6, 7]. Familiarity with the CT appearances of vari-
ous coronary artery anomalies and an understanding of the clinical
significance of these anomalies are essential for making a correct
diagnosis and planning treatment.
Most infants with ALCAPA die within the first year of life without
treatment, and survival beyond infancy is dependent upon adequate
collateral circulation from the RCA or another source. These lesions
are rarely tolerated well in the neonate, as the pulmonary vascular
resistance is still high, leading to high-pressure antegrade flow down
the LCA [1]. In our case, the existence of collateral vasculature betwe-
en the LCA and RCA, which caused the coronary steal phenomenon
and supplied normally oxygenated blood to the RCA’s territory, was
the mechanism of survival until adulthood.
Treatment of ALCAPA consists of surgical re-creation of dual
coronary perfusion. In the infantile type of ALCAPA, reimplantation
of the anomalous LCA directly into the aorta or creation of an intra-
pulmonary conduit from the left coronary ostia to the aorta, known
as the Takeuchi procedure, can be implemented. In the adult type of
ALCAPA, ligation of the LCA from the pulmonary artery combined
with coronary artery bypass grafting can be performed [3]. Our case
was asymptomatic, so this serious coronary anomaly remained undi-
agnosed until the patient had a heart attack. Magnetic resonance
imaging (MRI) was performed following MDCT. There were extensive
ischemic areas in both the cerebrum and the cerebellum on MRI.
Because the patient died 12 hours after MDCT coronary angiog-
raphy, no surgical intervention could be performed.
In conclusion, ALCAPA is very rare in adults without a previous
clinical history, as in the presented case. However, a radiologist
encountering ectatic coronary arteries in an MDCT coronary angiog-
raphy scan should always consider ALCAPA as a possible diagnosis.
Determining the origins, courses and terminations of the coronary
arteries is very important in the diagnosis of this syndrome.
102 Akgun et al. ALCAPA: MDCT Angiography Findings
Conflict interest statement: The authors declare that they have
no conflict of interest to the publication of this article.
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