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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.