Post on 28-Jul-2018
Anomalous coronary Anomalous coronary arteriesarteries
TarekTarek BayyoudBayyoudGillian Lieberman, M.D.Gillian Lieberman, M.D.
June 17, 2008June 17, 2008
Our patientOur patient
Patient M.S. is 28 yr old, malePatient M.S. is 28 yr old, male–– Chest pain 6/10Chest pain 6/10–– Radiating down his left armRadiating down his left arm–– Refused any kind of exercise (including Refused any kind of exercise (including
cardiac stress tests)cardiac stress tests)–– NauseaNausea–– History of prior cardiac surgeryHistory of prior cardiac surgery–– Family history of CADFamily history of CAD
NonNon--centralcentralPleuralPleural
InfectionInfectionMalignancyMalignancyPneumothoraxPneumothoraxPulmonary infarctionPulmonary infarctionConnective tissue diseasesConnective tissue diseases
Chest wallChest wallMalignancyMalignancyPersistent coughPersistent coughMuscle sprainMuscle sprainBornholmBornholm’’s diseases diseaseTietzeTietze’’ss syndromesyndromeRib fractureRib fractureIntercostalIntercostal nerve compressionnerve compressionThoracic shinglesThoracic shingles
CentralCentralTracheal
InfectionIrritant dusts
CardiacMassive pulmonary
thromboembolismAcute myocardial ischemia
EsophagealEsophagitisRupture
Great vesselsAortic dissection
MediastinalLung cancer ThymomaLymphadenopathyMetastasesMediastinitis
Causes of chest pain
Our patient: axial chest CT with contrast and mediastinal window
Prior median sternotomy defect ( )Pulmonary artery ( )Aberrant air ( )
Ascending aorta ( )Aberrant right coronary artery ( )Left coronary artery ( )
Courtesy of Dr. Faisal Khosa; Beth Israel Deaconess Medical Center (BIDMC, PACS)
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 pulmonaryarterial area (pulmonary artery not visible as subtracted)
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
No right internal mammary artery seen ( )Left internal mammary artery present ( )Surgical clips of previous CABG ( )
Our patient: axial chest CT with contrast and mediastinalwindow
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Our patient:
The previous slide shows no right internal mammary artery as it wasused for a coronary artery bypass graft.The left internal mammary artery is found in place. The chest CTdemonstrates the sternotomy with its wire.
The aberrant right coronary artery of type B course predisposes our patient significantly to sudden death. An anomalous coronaryartery 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.
AgendaAgenda
Anomalous coronary artery discussionAnomalous coronary artery discussionNormal anatomyNormal anatomyNormal variantsNormal variantsAnomalous coronary arteriesAnomalous coronary arteriesClinical presentationClinical presentationSpecific associationsSpecific associationsMenu of testsMenu of tests
Anomalous coronary arteriesAnomalous coronary arteriesThese anomalies occur in less than 1% of the These anomalies occur in less than 1% of the general population;general population;They are frequently associated with other major They are frequently associated with other major congenital defects (like congenital defects (like tetralogytetralogy of of FallotFallot and and transposition of great arteries);transposition of great arteries);Strongly associated with sudden death, Strongly associated with sudden death, myocardial ischemia, CHF and myocardial ischemia, CHF and endocarditisendocarditis;;Complicating cardiac surgery or interventions.Complicating cardiac surgery or interventions.
Normal anatomyof coronaryarteries
Views of the sternocostal and diaphragmatic surfaces
Frank H. Netter, M.D.© Novartis
Normal anatomy of coronary Normal anatomy of coronary arteriesarteries
Coronary arteries originate from left and right aortic sinusesCoronary arteries originate from left and right aortic sinusesLt. coronary a.:Lt. coronary a.:–– LAD (lt. anterior descending a.) gives off diagonal LAD (lt. anterior descending a.) gives off diagonal
(superficial) and (superficial) and septalseptal perforator (deep) branches perforator (deep) branches reaching the anterior 2/3 of the reaching the anterior 2/3 of the interventricularinterventricular septumseptum
–– RamusRamus intermediusintermedius coronary artery (variation)coronary artery (variation)–– LCX (lt. circumflex a.) gives obtuse marginal branchesLCX (lt. circumflex a.) gives obtuse marginal branches
Rt. Coronary a. (RCA):Rt. Coronary a. (RCA):–– ConusConus arteryartery–– Acute marginal branchAcute marginal branch–– Posterior descendingPosterior descending & & posterolateralposterolateral coronary arterycoronary artery
(PDCA and PLCA, respectively)(PDCA and PLCA, respectively)
Normal coronary a. angiographyNormal coronary a. angiography3D reconstruction of normal coronary a.
Right coronary artery ( )Left anterior descending artery ( )Circumflex artery ( )
Courtesy of Dr. Faisal Khosa; BIDMC, PACS Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Notice that the right and circumflex coronaries form a kind of mirror imagewhich helps to differentiate the LCX from theLAD.
Arterial dominanceArterial dominance
The RCA is in approximately 90% the dominant The RCA is in approximately 90% the dominant artery;artery;The crux of the heart is usually supplied by the The crux of the heart is usually supplied by the atrioventricularatrioventricular node artery from the RCA;node artery from the RCA;The dominant coronary artery gives the The dominant coronary artery gives the posterior posterior descending coronary artery descending coronary artery (which supplies the (which supplies the post.1/3 of the post.1/3 of the interventricularinterventricular septum by septum by septalseptal perforator branches);perforator branches);If both RCA and LCX give rise to the PDCA the If both RCA and LCX give rise to the PDCA the system is cosystem is co--dominant.dominant.
Normal variantsNormal variants
Separated LAD and LCX (absent left main Separated LAD and LCX (absent left main coronary artery);coronary artery);
Several Several conalconal arteries;arteries;
Minor variations in the location of the Minor variations in the location of the ostiaostia in the aortic sinuses are common.in the aortic sinuses are common.
Anomalous coronary arteriesAnomalous coronary arteriesClassification:Classification:
–– NumberNumber
Duplicated LAD, RCADuplicated LAD, RCA
–– StructureStructure
StenosisStenosis, , atresiaatresia, , hypoplasiahypoplasia (often associated (often associated with an absent PDCA)with an absent PDCA)
–– OriginOrigin
From pulmonary trunk, ventricle, nearby artery (like From pulmonary trunk, ventricle, nearby artery (like bronchial, internal mammary, bronchial, internal mammary, subclaviansubclavian, , innominateinnominate and right carotid)and right carotid)
Anomalous coronary arteriesAnomalous coronary arteries–– CourseCourse (single coronary artery): types A(single coronary artery): types A--DD
AAnterior to the right ventricular outflow tractnterior to the right ventricular outflow tract
BBetween the aorta and the pulmonary trunketween the aorta and the pulmonary trunk
Through the Through the supraventricularsupraventricular ccrestrest
DDorsal to the aortaorsal to the aorta
–– TerminationTermination
Fistula formation (most fistulas drain into the right Fistula formation (most fistulas drain into the right heart; the development of an heart; the development of an EisenmengerEisenmenger’’ss syndrome has not been reported)syndrome has not been reported)
Clinical PresentationClinical Presentation
Mostly asymptomaticMostly asymptomaticNo sex predominanceNo sex predominanceNo definitive inheritance patternNo definitive inheritance patternAge of presentation: early infancy, Age of presentation: early infancy, young adult young adult lifelifeInfant: Infant: –– Episodic crying, Episodic crying, tachypneatachypnea, wheeze, refusal to eat, , wheeze, refusal to eat,
failure to thrivefailure to thriveYoung adult:Young adult:–– Palpitation, Palpitation, anginaangina, , refusal to exerciserefusal to exercise, fatigue, fever, fatigue, fever
Non-specific
Specific associationsSpecific associationsSudden death: Sudden death: –– Type B courseType B course–– Structural abnormalitiesStructural abnormalities
EndocarditisEndocarditis::–– Fistulas (the receiving chamber usually is infected Fistulas (the receiving chamber usually is infected
at the point of entrance of the aberrant vessel)at the point of entrance of the aberrant vessel)
Heart failure:Heart failure:–– Left main coronary artery from pulmonary trunk Left main coronary artery from pulmonary trunk
(typically seen in early infancy)(typically seen in early infancy)
Menu of testsMenu of testsAdvantages Disadvantages
EchocardiographyEchocardiography non-invasive, no ionizing radiation, widely available, inexpensive
Poor coronary Poor coronary artery imaging artery imaging qualityquality
CT angiographyCT angiography Enables 3D reconstruction, high quality
Ionizing radiation;Nephrotoxic contrast media
MRAMRA Preferable in young patients
Gadolinium rarely induces Gadolinium rarely induces nephrogenicnephrogenic systemic systemic sclerosis sclerosis (low kinetic (low kinetic stability stability GdGd agents may be agents may be preferable);preferable);Inferior to CT in Inferior to CT in characterizing the distal characterizing the distal part of the coronary part of the coronary arteriesarteries
Coronary Coronary angiographyangiography
Gold standard Invasive procedure
More examplesMore examples……
Companion patient 1: Axial chest CT with contrast and mediastinal window
Aberrant left coronaryartery ( )and single origin of both coronaryarteries ( )
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
RightRight
LeftLeft
Companion patient 2: CT reconstruction, volume rendered 3D image
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Single left coronaryartery.
The circulation is left dominant.
Companion patient 2:Sagittal chest CT withcontrast and mediastinalwindow
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
The left circumflex is aprominent vessel which gives off the posterior descendingcoronary artery.
LeftLeftRightRight
LeftLeft RightRight
Companion patient 3: CT reconstructions, volume rendered 3D images
This patient has a single right coronary artery which supplies the whole heart.
Courtesy of Dr. Faisal Khosa; BIDMC, PACS Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Companion patient 4:Axial chest CTs with contrast andmediastinal window
Post aortic valve (not seen) andaortic root replacement ( )
Reimplanted left coronary artery( ) Courtesy of Dr. Faisal Khosa; BIDMC, PACS
Courtesy of Dr. Faisal Khosa; BIDMC, PACS
TreatmentTreatment
Only definitive Rx is surgeryOnly definitive Rx is surgery
Take home pointTake home point
Think of coronary artery anomalies in Think of coronary artery anomalies in young adult patients presenting with young adult patients presenting with angina.angina.
Thank youThank youhttp://www.thewellingtoncardiacservices.com/the-heart-cardiovascular-system.asp
ReferencesReferencesJamshidJamshid ShiraniShirani, MD, FACC, FAHA; Isolated coronary artery anomalies; , MD, FACC, FAHA; Isolated coronary artery anomalies; eMedicineeMedicine article: Mar 13, 2008; article: Mar 13, 2008; http://www.emedicine.com/med/TOPIC445.HTMhttp://www.emedicine.com/med/TOPIC445.HTMWhat are the coronary arteries?, Cleveland Clinic online; What are the coronary arteries?, Cleveland Clinic online; http://www.clevelandclinic.org/heartcenter/pub/guide/disease/cadhttp://www.clevelandclinic.org/heartcenter/pub/guide/disease/cad/cad_arteries.htm/cad_arteries.htmHeart and blood vessel conditions, Cleveland Clinic online; Heart and blood vessel conditions, Cleveland Clinic online; http://www.clevelandclinic.org/heartcenter/pub/guide/disease/defhttp://www.clevelandclinic.org/heartcenter/pub/guide/disease/default.asp?firstCatault.asp?firstCat=3&=3& secondCat=246secondCat=246Graham Douglas, Fiona Graham Douglas, Fiona NicolNicol, Colin Robertson; Macleod, Colin Robertson; Macleod’’s Clinical s Clinical ExaminatonExaminaton, 11, 11thth
edition, 2005; Chapters: The cardiovascular system, The respiratedition, 2005; Chapters: The cardiovascular system, The respiratory systemory systemhttp://www.circ.ahajournals.org/cgi/content/full/92/11/3158http://www.circ.ahajournals.org/cgi/content/full/92/11/3158http://www.healthsystem.virginia.edu/UVAHealth/peds_cardiac/aca.http://www.healthsystem.virginia.edu/UVAHealth/peds_cardiac/aca.cfmcfm
AcknowledgementsAcknowledgements
Gillian Lieberman, M.D.Gillian Lieberman, M.D.Faisal Faisal KhosaKhosa, M.D., M.D.