EAJM 2010; 42: 100-2 Case Report

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 Özet
Anomalous left coronary artery arising from the pulmonary artery (ALCAPA) is a Sol koroner arterin ana pulmoner arterden köken alması, nadir ancak tehli-
rare but very serious congenital coronary artery anomaly. Multidetector computed keli konjenital koroner arter anomalilerinden birisidir. Çok kesitli bilgisayarlı
tomography (MDCT) coronary angiography has recently become the gold stand- tomografi (ÇKBT) koroner anjiyografi ile anomalili arterlerin kökeni ve seyri
ard for depicting anatomical variations and anomalies of the coronary arteries mükemmel olarak ortaya konulabildiği için, özellikle koroner arterlerin ana-
because the origin and course of anomalous arteries can be demonstrated very tomik varyasyonları ve anomalilerini göstermede bu teknik son zamanlarda
accurately by this technique. In this report, we present a case of 22-year-old female altın standart haline gelmiştir. Bu yazıda, acil servisimize kardiyak arrest ne-
who was admitted to our emergency department with cardiac arrest. In the course deniyle kabul edilen 22 yaşında bir kadın vakayı sunuyoruz. Tanı sürecinde,
of diagnosis, MDCT coronary angiography revealed a left coronary artery arising ÇKBT koroner anjiyografi, ana pulmoner arterden çıkan bir sol koroner arter
from the pulmonary artery as well as marked dilatation of the coronary arteries. ile koroner arterlerde belirgin genişlemeyi göstermiştir.
Key Words: ALCAPA, Anomalous left coronary artery, MDCT coronary angiog- Anahtar Kelimeler: ALCAPA, Anomalili sol koroner arter, ÇKBT koroner anji-
raphy, Pulmonary artery yografi, Pulmoner arter

Introduction coronary artery anomaly and scanned with a 16-detector CT scanner

(Philips Medical Systems MX 8000 IDT Multislice CT System-V 2.5). The
Anomalous origin of the left main coronary artery (LCA) from the informed consent of the patient’s parents was obtained before radio-
pulmonary artery (PA) is a rare malformation also known as Bland- logical examination. Scan parameters were 120 kV, 340 mAs, 420 msec
White-Garland syndrome. Very few individuals with anomalous left rotation time with a slice thickness of 1 mm and increments of 0.5 mm,
coronary artery arising from the pulmonary artery (ALCAPA) survive using a detector collimation of 16x0.75 mm (pitch: 0.2). The heart rate
until adulthood without surgical repair; furthermore, approximately was 67 beats per minute during acquisition. One hundred milliliters of
90% die suddenly at a mean age of 35 years [1]. In the most common non-ionic, iodinated, low-osmolar contrast medium was injected thro-
form of this disease, the LCA arises from the PA, and the right coronary ugh the antecubital vein at a rate of 5 ml/sec. Subsequently, a bolus of
artery (RCA) arises normally from the aorta. This configuration creates 40 ml saline solution was administered at a rate of 2.5 ml/sec to avoid
a collateral circulation between the RCA and LCA and a coronary “steal” possible contrast artifacts at the right atrial entrance. An automatic
phenomenon into the PA [2]. In this article, we present clinical and bolus tracking method was used to optimize visualization.
MDCT coronary angiography findings of a 22-year-old female patient MDCT coronary angiography images demonstrated a LCA origi-
with ALCAPA with no history of chest pain. nating from the inferior side of the main PA. MDCT showed enormo-
us sinus vessels originating from a normally situated right coronary
Case Report artery with collateral circulation to the LCA. The lumen of the LCA
was fully enhanced, and a little enhancement was noticed in the
A 22-year-old female with no history of chest pain was admitted lumen of the PA, showing retrograde blood flow toward the PA. The
to our emergency department with sudden cardiac arrest. Following RCA arose from the aorta. All coronary arteries and their branches
the reinstitution of the cardiopulmonary circulation and medical were ectatic and showed mildly tortuous courses. Collateral circu-
management, a transthoracic echocardiographic examination was lation between the RCA and LCA as well as both a coronary “steal”
performed, revealing an obvious dilatation of the coronary arteries. phenomenon (from RCA to LCA) and a pulmonary “steal” phenome-
The patient was referred to the radiology department on suspicion of non (from LCA to main PA) were readily demonstrated (Figure 1a-c).

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: [email protected]
doi:10.5152/eajm.2010.28
EAJM 2010; 42: 100-2 Akgun et al. ALCAPA: MDCT Angiography Findings 101

A B C

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- conventional angiography, MDCT coronary angiography allows the
tion 12 hours after the MDCT coronary angiography examination. accurate and noninvasive depiction of coronary artery anomalies.
Therefore, surgical correction could not be performed. MDCT has been determined to be superior to conventional angiog-
raphy for delineating the origin, course, and termination of abnormal
Discussion coronary arteries [6, 7]. Familiarity with the CT appearances of vari-
ous coronary artery anomalies and an understanding of the clinical
Coronary artery anomalies are common in the general popu- significance of these anomalies are essential for making a correct
lation. They may accompany other congenital heart diseases and diagnosis and planning treatment.
can cause myocardial ischemia, a decrease in life expectancy, or Most infants with ALCAPA die within the first year of life without
sudden death. Most patients with coronary artery anomalies are treatment, and survival beyond infancy is dependent upon adequate
asymptomatic [1]. ALCAPA is one of the most serious congenital collateral circulation from the RCA or another source. These lesions
coronary artery anomalies. It has an estimated prevalence of one in are rarely tolerated well in the neonate, as the pulmonary vascular
300,000 live births [3]. Most of the affected patients show symptoms resistance is still high, leading to high-pressure antegrade flow down
in infancy and early childhood. Approximately 90% of untreated the LCA [1]. In our case, the existence of collateral vasculature betwe-
infants die in the first year of life, and only a few patients survive until en the LCA and RCA, which caused the coronary steal phenomenon
adulthood [3]. and supplied normally oxygenated blood to the RCA’s territory, was
Abnormal embryonic development may lead to positional ano- the mechanism of survival until adulthood.
malies of the coronary arteries. The coronary artery buds appear on Treatment of ALCAPA consists of surgical re-creation of dual
about the 12th day of life after the division of the truncus arteriosus, coronary perfusion. In the infantile type of ALCAPA, reimplantation
which leads to the separation of the aorta and pulmonary artery [4]. of the anomalous LCA directly into the aorta or creation of an intra-
Coronary artery anomalies can arise secondary to either malrotation pulmonary conduit from the left coronary ostia to the aorta, known
of the spiral septum dividing the truncus or malpositioning of the as the Takeuchi procedure, can be implemented. In the adult type of
coronary buds themselves. Soloff described the four possible ano- ALCAPA, ligation of the LCA from the pulmonary artery combined
malous coronary artery connections to the pulmonary artery [5]. The with coronary artery bypass grafting can be performed [3]. Our case
most common is anomalous left coronary artery from the pulmonary was asymptomatic, so this serious coronary anomaly remained undi-
artery (ALCAPA), followed by anomalous right coronary artery from
agnosed until the patient had a heart attack. Magnetic resonance
the pulmonary artery (ARCAPA), origin of both coronaries from the
imaging (MRI) was performed following MDCT. There were extensive
pulmonary trunk, and origin of an accessory coronary artery from
ischemic areas in both the cerebrum and the cerebellum on MRI.
the pulmonary trunk. The relatively high incidence of ALCAPA is exp-
Because the patient died 12 hours after MDCT coronary angiog-
lained by the proximity of the left coronary bud to the pulmonary
raphy, no surgical intervention could be performed.
artery sinus.
In conclusion, ALCAPA is very rare in adults without a previous
Conventional angiography is a traditional technique that has
clinical history, as in the presented case. However, a radiologist
been used for many decades to diagnose coronary artery anoma-
encountering ectatic coronary arteries in an MDCT coronary angiog-
lies [2]. However, MDCT has recently become accepted as the main
raphy scan should always consider ALCAPA as a possible diagnosis.
technique for evaluation of coronary anomalies [6, 7]. Several case
Determining the origins, courses and terminations of the coronary
reports describing MDCT coronary angiography findings in ALCAPA
arteries is very important in the diagnosis of this syndrome.
have been published previously [1, 7]. In our experience, unlike
102 Akgun et al. ALCAPA: MDCT Angiography Findings EAJM 2010; 42: 100-2

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