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Transcript
Anomalous coronary
arteries
Tarek Bayyoud
Gillian Lieberman, M.D.
June 17, 2008
Our patient
Patient M.S. is 28 yr old, male
– Chest pain 6/10
– Radiating down his left arm
– Refused any kind of exercise (including
cardiac stress tests)
– Nausea
– History of prior cardiac surgery
– Family history of CAD
Causes of chest pain
Non-central
Central
Pleural
Infection
Malignancy
Pneumothorax
Pulmonary infarction
Connective tissue diseases
Chest wall
Tracheal
Infection
Irritant dusts
Cardiac
Massive pulmonary
thromboembolism
Acute myocardial ischemia
Esophageal
Esophagitis
Malignancy
Rupture
Persistent cough
Great vessels
Muscle sprain
Aortic dissection
Bornholm’s disease
Mediastinal
Tietze’s syndrome
Lung cancer
Thymoma
Rib fracture
Lymphadenopathy
Intercostal nerve compression
Metastases
Thoracic shingles
Mediastinitis
Our patient: axial chest CT with contrast and mediastinal window
Courtesy of Dr. Faisal Khosa; Beth Israel Deaconess Medical Center (BIDMC, PACS)
Prior median sternotomy defect (Æ)
Pulmonary artery (Æ)
Aberrant air (Æ)
Ascending aorta (Æ)
Aberrant right coronary artery (Æ)
Left coronary artery (Æ)
Our patient:
On the previous chest CT image the aberrant right coronary artery
lies between the aortic root and pulmonary artery. This is classified
as a type B course. During exercise these vessels dilate
compressing the aberrant coronary artery and causing the
symptoms our patient experiences.
Furthermore, there is an acute angle formed by the anomalous
coronary artery and the cardiac wall. This may lead to a stenosis
and slit-like ostium aggravating his condition.
In contrast, the left coronary artery has a normal origin and course.
The prior sternotomy defect with adjacent aberrant air constitutes
either simply residual air post-OP or a possible mediastinitis.
Our patient: CT reconstruction, volume rendered 3D image
Aberrant right coronary artery coursing bet. aortic root and pulmonary
arterial area (pulmonary artery not visible as subtracted)
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Our patient: axial chest CT with contrast and mediastinal
window
No right internal mammary artery seen (Æ)
Left internal mammary artery present (Æ)
Surgical clips of previous CABG (Æ)
Courtesy of
Dr. Faisal Khosa;
BIDMC, PACS
Our patient:
The previous slide shows no right internal mammary artery as it was
used for a coronary artery bypass graft.
The left internal mammary artery is found in place. The chest CT
demonstrates the sternotomy with its wire.
The aberrant right coronary artery of type B course predisposes
our patient significantly to sudden death. An anomalous coronary
artery is found in 4-15% of young people who faced sudden death.
Corrective surgery with repositioning of the aberrant vessel
was suggested.
Our patient refused any surgical procedure.
Agenda
Anomalous coronary artery discussion
Normal anatomy
Normal variants
Anomalous coronary arteries
Clinical presentation
Specific associations
Menu of tests
Anomalous coronary arteries
These anomalies occur in less than 1% of the
general population;
They are frequently associated with other major
congenital defects (like tetralogy of Fallot and
transposition of great arteries);
Strongly associated with sudden death,
myocardial ischemia, CHF and endocarditis;
Complicating cardiac surgery or interventions.
Normal anatomy
of coronary
arteries
Views of the
sternocostal and
diaphragmatic
surfaces
Frank H. Netter, M.D.
© Novartis
Normal anatomy of coronary
arteries
Coronary arteries originate from left and right aortic sinuses
Lt. coronary a.:
– LAD (lt. anterior descending a.) gives off diagonal
(superficial) and septal perforator (deep) branches
reaching the anterior 2/3 of the interventricular septum
– Ramus intermedius coronary artery (variation)
– LCX (lt. circumflex a.) gives obtuse marginal branches
Rt. Coronary a. (RCA):
– Conus artery
– Acute marginal branch
– Posterior descending & posterolateral coronary artery
(PDCA and PLCA, respectively)
Normal coronary a. angiography
3D reconstruction of normal
coronary a.
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Right coronary artery (Æ)
Left anterior descending artery (Æ)
Circumflex artery (Æ)
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Notice that the right and circumflex
coronaries form a kind of mirror image
which helps to differentiate the LCX from the
LAD.
Arterial dominance
The RCA is in approximately 90% the dominant
artery;
The crux of the heart is usually supplied by the
atrioventricular node artery from the RCA;
The dominant coronary artery gives the posterior
descending coronary artery (which supplies the
post.1/3 of the interventricular septum by septal
perforator branches);
If both RCA and LCX give rise to the PDCA the
system is co-dominant.
Normal variants
Separated LAD and LCX (absent left main
coronary artery);
Several conal arteries;
Minor variations in the location of the ostia
in the aortic sinuses are common.
Anomalous coronary arteries
Classification:
– Number
Duplicated LAD, RCA
– Structure
Stenosis, atresia, hypoplasia (often associated
with an absent PDCA)
– Origin
From pulmonary trunk, ventricle, nearby artery (like
bronchial, internal mammary, subclavian,
innominate and right carotid)
Anomalous coronary arteries
– Course (single coronary artery): types A-D
Anterior to the right ventricular outflow tract
Between the aorta and the pulmonary trunk
Through the supraventricular crest
Dorsal to the aorta
– Termination
Fistula formation (most fistulas drain into the right
heart; the development of an Eisenmenger’s
syndrome has not been reported)
Clinical Presentation
Non-specific
Mostly asymptomatic
No sex predominance
No definitive inheritance pattern
Age of presentation: early infancy, young adult
life
Infant:
– Episodic crying, tachypnea, wheeze, refusal to eat,
failure to thrive
Young adult:
– Palpitation, angina, refusal to exercise, fatigue, fever
Specific associations
Sudden death:
– Type B course
– Structural abnormalities
Endocarditis:
– Fistulas (the receiving chamber usually is infected
at the point of entrance of the aberrant vessel)
Heart failure:
– Left main coronary artery from pulmonary trunk
(typically seen in early infancy)
Menu of tests
Advantages
Disadvantages
Echocardiography
non-invasive, no
ionizing radiation,
widely available,
inexpensive
Poor coronary
artery imaging
quality
CT angiography
Enables 3D
reconstruction,
high quality
Ionizing radiation;
Nephrotoxic
contrast media
MRA
Preferable in
young patients
Gadolinium rarely induces
nephrogenic systemic
sclerosis (low kinetic
stability Gd agents may be
preferable);
Inferior to CT in
characterizing the distal
part of the coronary
arteries
Coronary
angiography
Gold standard
Invasive procedure
More examples…
Companion patient 1: Axial chest CT with contrast
and mediastinal window
Aberrant left coronary
artery (Æ)
and single origin of
both coronary
arteries (Æ)
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Companion patient 2: CT reconstruction,
volume rendered 3D image
Right
Single left coronary
artery.
The circulation is
left dominant.
Left
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Companion patient 2:
Sagittal chest CT with
contrast and mediastinal
window
The left circumflex is a
prominent vessel which gives
off the posterior descending
coronary artery.
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Companion patient 3: CT reconstructions, volume rendered 3D images
Left Left
Right
Right
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
This patient has a single right coronary artery which supplies the whole heart.
Companion patient 4:
Axial chest CTs with contrast and
mediastinal window
Post aortic valve (not seen) and
aortic root replacement (Æ)
Reimplanted left coronary artery
(Æ )
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Treatment
Only definitive Rx is surgery
Take home point
Think of coronary artery anomalies in
young adult patients presenting with
angina.
Thank you
http://www.thewellingtoncardiacservices.com/the-heart-cardiovascular-system.asp
References
Jamshid Shirani, MD, FACC, FAHA; Isolated coronary artery anomalies; eMedicine
article: Mar 13, 2008; http://www.emedicine.com/med/TOPIC445.HTM
What are the coronary arteries?, Cleveland Clinic online;
http://www.clevelandclinic.org/heartcenter/pub/guide/disease/cad/cad_arteries.htm
Heart and blood vessel conditions, Cleveland Clinic online;
http://www.clevelandclinic.org/heartcenter/pub/guide/disease/default.asp?firstCat=3&
secondCat=246
Graham Douglas, Fiona Nicol, Colin Robertson; Macleod’s Clinical Examinaton, 11th
edition, 2005; Chapters: The cardiovascular system, The respiratory system
http://www.circ.ahajournals.org/cgi/content/full/92/11/3158
http://www.healthsystem.virginia.edu/UVAHealth/peds_cardiac/aca.cfm
Acknowledgements
Gillian Lieberman, M.D.
Faisal Khosa, M.D.