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 DR RAJESH KF CORONARY ARTERY ANOMALIES.  CAAs has a global incidence of 5.64%  Incidence of CAA related sudden death is 0.6%

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Presentation on theme: " DR RAJESH KF CORONARY ARTERY ANOMALIES.  CAAs has a global incidence of 5.64%  Incidence of CAA related sudden death is 0.6%"— Presentation transcript:


2  CAAs has a global incidence of 5.64%  Incidence of CAA related sudden death is 0.6%

3 FEATURERANGE No. of ostia2 to 4 LocationRight and left anterior sinuses(upper midsection) Proximal orientation45° to 90° off the aortic wall Proximal common stemOnly left (LAD and Cx) Proximal courseDirect from ostium to destination Mid-courseExtramural (subepicardial) BranchesAdequate for the dependent myocardium Essential territoriesRCA (RV free wall), LAD (anteroseptal),OM (LV free wall) TerminationCapillary bed Normal Features of Coronary Anatomy



6 Anomalies of origination and course Absent left main trunk (split origination of LCA)  LAD and Cx originates from LCS without common trunk  Occurs in about 1%  More frequent with BAV  No clinical consequences  Coronary ostia are smaller  JL catheter for selective cannulation of LAD and amplatz left catheter for LCX

7 Anomalies of origination and course Anomalous location of coronary ostium within aortic root or near proper aortic sinus of Valsalva a. High b. Low c. Commissural  High origin of LCA engaged with Amplatz left,MP catheter  High origin of RCA engaged with Amplatz catheter

8 Anomalies of origination and course Anomalous location of coronary ostium outside normal aortic sinuses a. Right posterior aortic sinus b. LV,RV,PA,AA,Aortic arch, Descending thoracic aorta, Innominate artery,Right carotid artery,IMA, Bronchial artery,Subclavian artery c.Pulmonary artery

9 Ectopic Coronary Ostium in NCS  Benign anomaly  Most commonly involves LCA ostium  Difficult cannulation-unexpected location,tangential or slitlike nature  Nonselective angiography-longer than usual LMCA  RAO and straight lateral projections are most useful  For selective catheterization Amplatz or Multipurpose curved catheters offer best chance of success

10 Ectopic Coronary Ostium Arising Outside the Aortic Root(Ascending Aorta)  Usually involves anterior/left surface of aorta  Frequently have slit like orifices and tangential proximal course along aortic wall  RCA is most frequently ectopic artery  Predisposed to more atherosclerosis  Association with Congenital aortic valve anomalies

11 Ectopic Coronary from Pulmonary artery (1) LCA from posterior facing sinus (2) Cx from posterior facing sinus (3) LAD from posterior facing sinus (4) RCA from anterior right facing sinus (5) Ectopic location (outside facing sinuses) (a) From anterior left sinus (b) From pulmonary trunk (c) From pulmonary branch Smith A., Arnold R., Anderson R.H., Wilkinson J.L., Qureshi S.A., McKay R. Anomalous origin of the left coronary artery from the pulmonary trunk. J Thorac Cardiovasc Surg 1989;98

12 ALCAPA  LCA arises from PA usually from left posterior facing sinus  Fetus-both coronary arteries receive forward flow  Early after birth - Anterolateral infarct and slight retrograde flow from LCA to PA  15% of patients-myocardial blood flow can sustain myocardial function at rest or even during exercise  Adult-Enlarged RCA and collaterals and significant retrograde flow into PA

13 Clinical features  Paroxysmal attacks of acute discomfort precipitated by feeding  CHF at 2 to 3 month  Physical examination-CHF,MR  Abnormal Q waves in leads I, aVL, and precordial leads V4 to V6  Older children and adults -may be asymptomatic or have dyspnea, syncope, or exertional angina  Sudden death after exertion

14 Echo  Abnormal origin of LCA  Flow passes from RCA into PA  Enlarged RCA  RWMA and mitral regurgitation Aortic root angiography  Dilated RCA  large collaterals-filling of LCA and passage of contrast material to MPA

15 Treatment  Ligation of LCA at its origin  Direct reimplantation of origin of LCA into aorta (with a button of PA around the origin)  Ligation of origin of LCA and reconstitution of flow through it with subclavian arterial or SVG  Takeuchi procedure- aortopulmonary window is created and a tunnel fashioned that directs blood from aorta to LCA

16 Anomalous Origination of RCA, LAD or Cx Artery From PA  Benign condition  Typically recognized by atypical angina, systolic heart murmur, abnormal stress test or angiography  In absence of major clinical manifestations not an indication for surgery

17 Coronary ostium at improper sinus (ACAOS) a. RCA from left anterior sinus b. LAD from right anterior sinus c. Cx from right anterior sinus d. LCA from right anterior sinus ARCA~ 6 times more prevalent than ALCA ALCA has a higher risk of SCD Anomalies of origination and course

18 Sudden cardiac death associated with four risk factors Slit-like coronary orifice Acute angle of take-off from aorta Presence of aortic intramural coronary arteries Inter-arterial course between aorta and PA


20  Echo  Difficult by angiography  MRI or CT is more sensitive

21 Abnormal crossing pathways Angelini et al  Retrocardiac  Path is behind mitral and tricuspid valves in posterior AV groove Retrocardiac

22  Retroaortic  Most common  Specifically involve origination of Cx artery from right sinus of Valsalva  Incidence in general population range from 0.1 to 0.9%  No clinical consequences

23  Preaortic  Between the aorta and PA  Osial abnormalities are usual  intramural course within the aortic root and adherent to it for about 1.5 cm  Rarely systolic compression lead to SCD

24  Intraseptal(supracristal)  Located in upper anterior IVS(Derived from conal septum)  Recogonised by systolic phasic narrowing during CAG  Septal perforators

25  Prepulmonary (precardiac)  Common in TOF

26 RCA from left anterior sinus, with anomalous course(ARCA) (1) Posterior AV groove or retrocardiac (2) Retroaortic (3) Between aorta PA (Most common, 30% mortality) (4) Intraseptal (5) Anterior to pulmonary outflow (6) Posteroanterior interventricular groove (wraparound)

27 LCA from right anterior sinus, with anomalous course (1) Between aorta and PA (2) Anterior to PA (3) Retroaortic (4) Intraseptal (5) Posterior AV groove (6) Postero-anterior interventricular groove

28 Angiographic appearace RAO

29 LAD from right anterior sinus, with anomalous course (1) Anterior to PA (2) Intraseptal (3) Between aorta and PA (4) Posteroanterior interventricular groove (wraparound)

30 Cx that arises from right anterior sinus, with anomalous course (1) Posterior AV groove (2) Retroaortic Angiography  Long LMCA segment  Small cx branch  RAO view shows dot of retro aortic course

31 Current recommendations Symptomatic patients  Unroofing  Reimplantation Asymptomatic patient  Demonstrable ischemia: repair  No demonstrable ischemia  ALCA: repair  ARCA: observe

32 Anomalies of origination and course Single coronary artery  Incidence 0.024%  Single ostium with absence of an ostium in opposite sinus  No other coronary artery from an ectopic site  40% associated with cardiac malformations(TGA, TOF, TA, coronary-cameral fistulas, and BAV)

33  Less tangential origin or ostial ridge pathology than ectopic CA with independent ostia  Coronary blood flow is not affected  Incidence of atherosclerotic disease not increased  Ostial obstruction (large guiding catheter or directional atherectomy device) -poorly tolerated  Angioplasty of common trunk is absolutely contraindicated

34 Angiographic classification Lipton et al

35 Coronary artery atresia  Characterized by ostial dimple in left or right aortic sinus terminates in a cordlike fibrotic structure without a patent lumen  Proximal occluded segments have larger diameter  Collaterals  Stress testing demonstrate myocardial ischemia  Seen in PA with intact IVS

36 Coronary artery isolation  Variant of ostial atresia  Juxtaposition of abnormal aortic cusp with aortic sinus wall lead to obliteration of underlying coronary ostium

37 Coronary hypoplasia  Angiographic appearance -small diameter(usually <1 mm) with respect to apparent area of dependent myocardium  Myocardial fibrosis and segmental hypokinasia are frequent  Local reversible ischemia during stress testing with myocardial nuclear scintigraphy  Terminology is incorrect when dependent myocardial bed is actually served by alternative sources (unusual coronary patterns) or coronary spasm or diffuse disease Anomalies of intrinsic anatomy

38 Absent coronary artery  Misnomer  On angiographic grounds, the most frequent reason are  Coronary ectopia (misdiagnosed)  Coronary occlusion with lack of demonstrable collateral filling  Alternative coronary artery tree pattern not recognized on angiography Anomalies of intrinsic anatomy

39 Coronary ectasia or aneurysm  Localized dilations in a normal sized coronary artery  Coronary segment diameter >50% with respect to normal  Doppler coronary wire blood flow velocity - significant reduction in peak flow velocity  Streamlining and slow runoff contrast media in angiography  Acquired stenosis  Mural thrombosis with distal embolization  Aneurysmal rupture Anomalies of intrinsic anatomy

40 Intramural coronary artery (muscular bridge)  More than 1% of incidence  Most commonly associated with ventricular hypertrophy  Coronary artery segment of variable length covered by myocardial fibers  Angiographic recognition of systolic narrowing  Phasic narrowing of a coronary aftery may also occur in ventricular aneurysms or pericardial fibrous bands  Intracoronary nitroglycerin in a 100- to 300- /microg bolus facilitates

41  U sign- Artery's accentuated descent from its subepicaardial location  Most commonly involve proximal LAD  Systolic stenosis is unlikely to cause coronary flow reduction  Rare reports of spasm, thrombus and atherosclerotic change

42 Anomalies of intrinsic anatomy  Subendocardial coronary course  Coronary crossing  Anomalous origination of posterior descending artery from anterior descending branch or a septal penetrating branch

43 Anomalies of intrinsic anatomy Split RCA a. Proximal+distal PDAs that both arise from RCA b. Proximal PDA that arises from RCA, distal PDA arises from LAD c. Parallel PDAs (arising from RCA, Cx) or “codominant”

44 Anomalies of intrinsic anatomy Split LAD Duplication of LAD < 1 % Population a. LAD+first large septal branch b. LAD, double (parallel LADs) c. LAD+large diagonal d. Highly dominant RCA+LAD

45 Ectopic origination of first septal branch a. RCA b. Right sinus c. Diagonal d. Ramus e. Cx Anomalies of intrinsic anatomy

46 Anomalies of coronary termination Coronary fistulas  A sizable communication between a coronary artery and a cardiac cavity or any segment of systemic or pulmonary circulation

47  Fistulas from RCA, LCA, or infundibular artery to RV,RA,CS, SVC, PA, PV,LA, LV, Multiple(right+left ventricles)  Originate from left coronary artery system (50-60%), right coronary artery system (30-40%), or both (2-5%)  Most fistulas (90%) drain into right heart Anomalies of coronary termination

48  Functional coronary fistula  Definite signs of fistulous flow-feeding vessel diameter >50% expected diameter  Angiographically prompt visualization of receiving structure with step up in concentration of injected substance  Evidence of volume overload in affected cardiac chambers  Evidence of steal involving myocardial nutrient blood flow

49  Use large-lumened catheters with side holes (NIH or Gensini ) or guide catheter kept in position by a inch guidewire  Mechanical injector  Diagnose coronary fistula  Identify receiving chamber or vessel  Complete visualization of nutrient myocardial branches  Absence of nutrient coronary branches arising from a fistulous tract should suggest alternative diagnosis-RSOV

50 Complications  Aneurysm formation  Intimal ulceration  Medial degeneration  Intimal rupture  Atherosclerotic deposition  Calcification  Side branch (nutrient) obstruction  Mural thrombosis  Coronary rupture

51 Treatment  Amount of dilation of fistulous vessel is more relevant than fistulous flow, symptoms and signs of myocardial ischemia  Catheter based or surgical intervention at an early age(gianturco coils, double umbrella,gifca vascular closure device)  Large fistulas-risk of rupture and mural clotting  Reversibility of ectasia is consistently reported in pediatric age  Optimal time for correction -fifth to fifteenth year of life

52 VARIATION (%) Coronary anomalies (total)110 (5.64) Split RCA24(1.23) Ectopic RCA (right sinus)22 (1.13) Ectopic RCA (left sinus)18 (0.92) Fistulas17(0.87) Absent left main coronary artery13 (0.67) Circumflex arising from right sinus13 (0.67) LCA arising from right sinus3 (0.15) Low origination of RCA2 (0.1) Other anomalies3(0.27) Incidence of Coronary Anomalies and Patterns Angelini P et al

53 FUNCTIONAL CLASSIFICATION  Myocardial ischemia, primary (fixed /episodic) Ostial atresia Ostial stenosis ALCAPA Coronary fistula (rare) Muscular bridge (rare )  Myocardial ischemia, secondary (episodic) ACAOS-Tangential origin, intramural course Myocardial bridge - spasm and/or clot Coronary ectasia - mural clot Coronary fistula - mural clot

54  Increased risk of fixed coronary atherosclerotic disease Coronary fistula ALCAPA Coronary ectasia Muscular bridge (proximal )  Secondary aortic valve disease Coronary aneurysm (ostial) Coronary fistula ALCAPA

55  Increased risk of bacterial endocarditis- Coronary fistula  Ischemic cardiomyopathy (hibernation) ALCAPA,LCA atresia,rarely in cases of LCA origin from RCS  Volume overload Coronary fistula,ALCAPA

56  Misdiagnosis “ Missing” coronary artery “Hypoplastic” coronary artery  Unusual technical difficulties during coronary angiography or angioplasty Ectopic ostia (tangential) Split left coronary artery Coronary fistula

57 Clinical Management: ACC/AHA Guidelines J. Am. Coll. Cardiol. 2008;52;e1-e121

58 Coronary Artery Patterns with Congenital Heart Defects

59 D-TGA  Important to identify before switch Sx  Facing sinuses -Sinuses adjacent to PA  Nomenclature of facing sinuses depends on relationship of great vessels  VSD or side-by-side GVs more associated with coronary anomalies  Almost all coronaries arise from facing sinuses  In 60% coronary arteries come from appropriate sinuses and branch normally  Seen often with aorta anterior and to right of PA

60 D-TGA  LCX arising from RCA and coursing posterior to PA - 20% of patients  Usually with side-by-side great vessels  Completely inverted-RCA arising from a left anterior sinus and LCA arising from right posterior sinus  Partially inverted- LCX arising from right posterior sinus and LAD arising with RCA from left anterior sinus  Various single coronary anomalies  Intramural coursing

61 TOF  40% -abnormally long, large conus artery supplies a significant mass of myocardium  4% - LAD from RCA and passes across RVOT  Occasionally single coronary artery  Major arteries cross RVOT-Surgery with transannular incision more difficult  Can be detected by echocardiography  If anatomy is uncertain-aortic root angiography or selective coronary arteriography before Sx

62  Aorta is anterior and to left of PA  Coronary arteries come from facing sinuses  Anterior sinus is usually noncoronary sinus  LCA supplies LV but arises in right facing sinus  Passes in front of PA and divides into LAD and Cx, passing in front of RAA in AV groove.  RCA arises from left facing sinus and runs in AV groove in front of LAA to terminate as PDA  Most common variant is SCA coming from right facing sinus. L-TGA

63  Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adults, 7th Edition  Myung k Park 5 th edition  Hurst,s 13 th edition  Morton J Kern 5 th edition  Coronary Artery Anomalies: A Comprehensive Approach, edited by P. Angelini.Lippincott Williams & Wilkins, Philadelphia 1999  Coronary Anomalies: Incidence, Pathophysiology, and Clinical Relevance Paolo Angelini, MD; José Antonio Velasco, MD; Scott Flamm, MD Circulation May 21, 2002  Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adults, 7th Edition  Myung k Park 5 th edition  Hurst,s 13 th edition  Morton J Kern 5 th edition  Coronary Artery Anomalies: A Comprehensive Approach, edited by P. Angelini.Lippincott Williams & Wilkins, Philadelphia 1999  Coronary Anomalies: Incidence, Pathophysiology, and Clinical Relevance Paolo Angelini, MD; José Antonio Velasco, MD; Scott Flamm, MD Circulation May 21, 2002 Referances

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