1) Coronary artery anomalies (CAAs) are defined as morphological features found in less than 1% of the population. They can be classified based on origin, course, termination, or hemodynamic significance. 2) Some important CAAs include anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), anomalous origin of the coronary artery from the opposite sinus (ACAOS), and coronary artery fistulas. 3) ALCAPA causes retrograde flow of blood from the right coronary artery to the pulmonary artery. ACAOS, especially when the ectopic artery takes an interarterial course, can cause ischemia. Coronary artery fistulas can lead to complications like aneurysm formation

1. CORONARY ANOMALY
Dr Siva Subramaniyan
PGIMER &Dr.RML Hospital
New Delhi

2. DEFINITION
• Defining what anatomy of the coronary arteries is normal can be challenging.
• Some normal features can be defined in numerical terms (for example, the
number of coronary ostia), while in some other cases a more qualitative
description is required.
• Angelini and coworkers proposed to classify “normal” as every feature with > 1%
of frequency in an unselected general population
Angelini. Coronary Artery Anomalies. Circulation March 13, 2007

3. • According to this approach, a CAA can be defined as a coronary pattern or feature
that is encountered in less than 1% of the general population.
coronary feature in two groups:
(1) normal coronary anatomy, defined as any morphological characteristics seen in
> 1% of unselected sample. This group also includes normal anatomical
variants, defined as alternative and relatively unusual morphological feature
observed in > 1% of the population; and
(2) anomalous coronary anatomy, defined as morphological features found in < 1%
of the population

4. IMPORTANCE
• Difficulty during cannulation
• Needs to know before going to any thoracic surgery
• Some anomaly can cause ischemia and prone for atherosclerosis
• More importantly some can cause SCD

6. NORMAL CORONARY
Villa ADM et al . Coronary artery anomalies. June 28, 2016|Volume 8|Issue 6|

7. LCA
• In the normal coronary anatomy, the right coronary artery originates from the
right coronary sinus, and the left coronary artery trunk originates from the left
coronary sinus.
• It posteriorly crosses the pulmonary trunk, bifurcating into anterior descending
and circumflex arteries.
• In approximately 37% of the individuals, there is a trifurcation of the left
coronary trunk into anterior descending, circumflex and diagonal coronary
arteries or intermediate branch, which irrigates the free lateral wall of the left
ventricle

8. • The anterior descending artery travels in the interventricular groove and gives off
diagonal branches towards the anterolateral wall of the left ventricle.
• The circumflex artery travels in the left atrioventricular groove and varies both in
size and extent, depending upon the coronary dominance. It gives off one to
three marginal branches supplying the free wall of the left ventricle

9. RCA
• The right coronary artery travels down the right atrioventricular groove. In 50% of
individuals, its first branch is the conus branch that supplies the right ventricle
outflow tract.
• the second branch is the sinoatrial node branch that supplies the sinoatrial node
and the right atrium.
• In 38% of cases, such a branch originates from the left coronary artery, and in 3%,
from both arteries

10. • Also, there are branches towards the free wall of the right ventricle, and the
branch located in the junction between medial and distal thirds of the right
coronary artery is named obtuse marginal artery.
• In approximately 85% of the individuals, the right coronary artery crosses the crux
cordis and gives the
- posterior descending branch (right dominant coronary supply),
- in 7% to 8% the circumflex artery gives PDA (left dominant coronary
supply), and
- in 7% to 8% of the cases the posterior interventricular septum is irrigated
by branches of the right coronary and circumflex arteries (codominance)

11. INCIDENCE

12. CLASSIFICATION
Coronary anomalies may be classified according Angelini et al. as
follows:
1) anomalies of origination and course;
2) intrinsic anomalies;
3) termination anomalies.

14. Angelini. Coronary Artery Anomalies. Circulation March 13, 2007

16. J.M. Pe´rez-Pomares et al. Congenital coronary artery anomalies (2016) 109, 204–216

17. functional classification

19. • Another classification divides coronary anomalies into hemodynamically
significant and non-hemodynamically significant.
• Anomalies classified as hemodynamically significant include:
1) anomalies of origination with interarterial course
2) anomalous origin in the pulmonary artery;
3) atresias;
4) congenital fistulas
Villa ADM et al . Coronary artery anomalies. June 28, 2016|Volume 8|Issue 6|

20. classification based on ischemia

21. 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, left dominance
• No clinical consequences
• Coronary ostia are smaller
J.M. Pe´rez-Pomares et al. Congenital coronary artery anomalies (2016) 109, 204–216

22. CANNULATION
• Inject more dye so that Lcx also can be visualized
• If LAD is cannulated it is bulled back into aorta and clockwise torque is
given to cannulate Lcx
• Use JL4 for LAD and longer curve for Lcx
• JL catheter for selective cannulation of LAD and amplatz left catheter
for LCX

24. Anomalous location of coronary ostium within
aortic root or near proper aortic sinus of Valsalva
a. High
b. Low
c. Commissural
• It is defined as origin of a coronary artery or left coronary trunk more than 1 cm
above the sinotubular junction.
• It usually does not present with clinical problem, however the preoperative
identification of this anomaly is important in case of ascending aorta surgery and
may cause difficulties in catheterization during angiography.

25. • Most frequently, it occurs in the right coronary artery, sometimes in
association with a bicuspid aortic valve

26. CANNULATION
• High origin of LCA engaged with Amplatz left, MP catheter
• High origin of RCA engaged with Amplatz catheter

27. ALCAPA
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
J.M. Pe´rez-Pomares et al. Congenital coronary artery anomalies (2016) 109, 204–216

28. 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

29. Echo
• Abnormal origin of LCA
• Flow passes from RCA into PA
• Enlarged RCA
• RWMA and mitral regurgitation

30. Aortic root angiography
• Dilated RCA
• Absence of left coronary osteum
• large collaterals-filling of LAD
and Lcx
• Later MPA and LMCA filled by
LAD and LCX branch

32. 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

33. ACC/AHA 2008 Guidelines for Adults With CHD. December 2, 2008:e143–263

34. other artery
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

35. ACAOS

36. Anomalous CA ostium location at improper
aortic sinus–wrong sinus
• RCA to left sinus
• LCA to right sinus
• LCX to RCA/ or sinus
• LAD to RCA/or sinus
• RCA or LCA to posterior sinus
with anomalous course: interaterial, prepulmonic, intraseptal,
retroaortic, posterior atrioventricular groove or retrocardiac, postero-
anterior interventricular groove
J.M. Pe´rez-Pomares et al. Congenital coronary artery anomalies (2016) 109, 204–216

37. Abnormal crossing pathways
Angelini et al
• Retrocardiac
• Path is behind mitral and
tricuspid valves in
posterior AV groove
Retrocardiac

38. • 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

39. • Intraseptal(supracristal)
• Located in upper
anterior IVS(Derived
from conal septum)
• Recogonised by systolic
phasic narrowing during
CAG

40. • Prepulmonary
(precardiac)
• Common in TOF

41. INTRAMUARAL

42. ACAOS
• Anomalous origination of coronary artery from the opposite sinus (ACAOS) is an
uncommon coronary anomaly. Its incidence is reported to be around 1.07% .
• It comprises anomaly of right coronary artery originated from left sinus (right
ACAOS) and its opposite or left ACAOS.

43. Right coronary originating from the left coronary sinus
• Right coronary artery originating from the left coronary sinus or as a branch of a
single coronary artery is found in 0.03% to 0.17% of the individuals submitted to
angiography.
• The most common proximal pathway of the right coronary in such cases is
interarterial, and can be associated with sudden cardiac death in up to 30% of
patients

44. • Both right and left ACAOS have significant clinical consequence if the ectopic
artery has an interarterial course or intramural intussusception
• A constant relationship is observed between left ACAOS and sudden death or
ischemia during extreme exercise .
• Right ACAOS with an interarterial course is a type of ACAOS which poses high risk
for myocardial ischemia or sudden death as well

45. The clinical picture of ACAOS can be divided into two spectra:
• the first is sudden death in the young and after strenuous physical activity or
sport and
• the second is atypical clinical picture .
• Most of ACAOS patients are asymptomatic. Atypical chest discomfort is the most
prevalent symptom urging patients to refer to the health facility and to perform
the coronary angiography to detect ACAOS

46. SUDDEN DEATH
• Hard activity causes dilatation of aortic root and pulmonary trunk which
compresses slit-like ostium or particular segment of ectopic coronary artery. This
occurs especially in individuals with sufficient aortic distensibility, such as in
young people or sportsmen.

47. Anomalies of origination and course
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

48. Left coronary trunk originating from the right
coronary sinus
• Left coronary trunk originating from the right coronary sinus or as a branch of a
single coronary artery occurs in 0.09% to 0.11% of the individuals submitted to
angiography .
• Proximal interarterial course occurs in 75% of such patients

49. Anterior descending or circumflex arteries
originating from the right coronary sinus
• circumflex artery is the one that most commonly presents anomalous origin,
occurring in 0.32% to 0.67% of the population. Retroaortic pathway is its most
common course, and there is no association with sudden death.
• The anterior descending artery with anomalous origin rarely occurs in individuals
with a normal cardiac anatomy. It is generally associated with Fallot’s tetralogy,
complex transposition and double right ventricular output tract

50. INTRAMURAL VS INTRA SEPTAL

52. ACC/AHA 2008 Guidelines for Adults With CHD. December 2, 2008:e143–263

53. Surgical and Catheterization-Based
Intervention
• Both surgical revascularization (eg, marsupialization, coronary bypass, or
coronary reimplantation) and limited cases of transcatheter stenting have been
reported to have short-term stability, without long-term follow-up

54. Intramural course (or myocardial bridge)
Intramural coronary artery (muscular bridge)
• More than 1-5% of incidence
• Most commonly associated with ventricular hypertrophy(HOCM)
• 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
J.M. Pe´rez-Pomares et al. Congenital coronary artery anomalies (2016) 109, 204–216

55. • 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

57. • By intravascular ultrasound clinically significant myocardial bridges
are characterized by
- phasic systolic vessel compression,
- persistent reduction in diastolic lumen,
- increased blood flow velocities,
- retrograde systolic flow, and
- decreased coronary flow reserve.
management- surgical de-bridging and even stent implantation have
been successfully carried out in symptomatic cases

58. CORONARY FISTULAS
• A sizable communication between a coronary artery and a cardiac
cavity or any segment of systemic or pulmonary circulation
J.M. Pe´rez-Pomares et al. Congenital coronary artery anomalies (2016) 109, 204–216

59. • 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
• The haemodynamic consequences of CA fistulae depend mainly on the resistance
(which is determined by fistula size, tortuosity, and length) and on the site of
drainage.

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

63. SURGERY
• Surgical fistula closure can be successful if CAVF is well defined and clear surgical
access is believed to be technically achievable.
• Recurrence may be a problem if anatomic definition is suboptimal, and surgery
may be difficult to perform owing to poorly visualized, typically distal fistulous
connections.
• Surgical closure of audible CAVF with appropriate anatomy is recommended in all
large CAVFs and in small to moderate CAVFs in the presence of symptoms of
myocardial ischemia, threatening arrhythmia, unexplained ventricular
dysfunction, or left atrial hypertension

64. Catheterization-Based Intervention
• Numerous reports of transcatheter closure with coils or detachable devices
describe near or complete CAVF occlusion in attempted closure procedures .
• Criteria for transcatheter closure of CAVF are similar to those used for surgical
closure of CAVF.
• Transcatheter closure of CAVF should be performed only in centers with
particular expertise in such intervention.

65. ACC/AHA 2008 Guidelines for Adults With CHD. December 2, 2008:e143–263

66. anomaly in congenital heart disease

67. 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

68. D-TGA

69. 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

70. CONCLUSION
• Coronary anomaly common, but rarely causes symptoms
• Most of them detected during angiography and will have
difficulty in cannulation
• Atypical chest pain or TMT positive in athletes needs attention
and evaluation
• It can cause SCD