This is a ppt i presented during my residency at GB Pant hospital, 2019-2022. I have included many angiogram videos but unfortunately they are not playable on slideshare. I hope this will cardio residents and consultants during their day to day practice. Dr. Ghazi Muheeb Senior resident (DM), GB Pant Hospital and MAMC, New Delhi.

1. ANOMALOUS CORONARY
ARTERIES
CHALLENGES IN ACCESS AND
INTERVENTION
Dr. Ghazi Muheeb
Dr. Girish MP

2. How do we define a Normal Coronary Artery?
“..nature and name of a specific coronary artery are assigned, not according
to the site of origin, proximal course or branches, but according to the
dependent territory..”
P. Angelini.Coronary artery anomalies : An entity in search for identity.Circulation. 2007;115:1296-1305.
“Any form observed in >1% of an unselected general population is normal.”
Becker AE,Anderson RH. Coronary artery anomalies. In: BeckerAE, Anderson RH, eds. Pathology of
Congenital Heart Disease. London: Butterworths; 1981: 369–378.

3. FEATURES OF A NORMAL CORONARY ARTERY
P. Angelini.Coronary artery anomalies : An entity in search for identity.Circulation. 2007;115:1296-1305.

4. Normal Anatomy
• Proposed Definition of the Essential Features of the
3 (Elementary) Coronary Arteries-
Paolo angelini, tex heart inst j 2002;29:271-8)

5. Definition of Anomalous Coronary Artery
Constellation of conditions, where there is a variation in one or
both the coronary arteries in the
• Origin
• course
• Termination
• intrinsic anatomy
And occurring in less than 1 percent of an unselected population
Angelini P. Normal and anomalous coronary arteries: definitions and classification. Am Heart J.1989;117(2)

6. Incidence
Yamanaka O, Hobbs RE. Coronary artery
anomalies in 126,595 patients undergoing coronary
arteriography. Cathet Cardiovasc Diagn 1990;21:28
1.3 % in in 126,595 Patients
Undergoing Coronary
Arteriography at the
Cleveland Clinic Foundation
from 1960 to 1988.
P.Angelini.Coronary artery anomalies : An entity in search
for identity. Circulation. 2007;115:1296-1305.
Study by P. angelini in 1950
patients found 5.6% overall
incidence.

7. WHAT SHOULDWE KNOW?
CAN WE HAVE A PRACTICAL CLASSIFICATION ?
WHOWILL BECOME SYMPTOMATIC ?
INTERVENTION INWHOM ?
SPECIAL CONSIDERATION DURING INTERVENTION ?

8. Classification of coronary artery anomalies based on anatomical features

9. ACAOS are further characterized by 1 of 5 course subtypes as
• interarterial,
• subpulmonic (intraconal or intraseptal),
• pre-pulmonic,
• retroaortic, or retrocardiac (Central Illustration).
• Additionally, ACAOS may have an early intramural segment (within the
aortic wall), as seen in the majority of inter-arterial cases.
• Among course subtypes, the potential for SCD has been largely attributed to
an interarterial course between the aorta and pulmonary artery

11. WHO WILL BECOME SYMPTOMATIC ?
TYPE OF ISCHEMIA CORONARY ANOMALY
Absence of ischemia Majority of anomalies (split RCA, ectopic RCA from
right cusp)
Episodic ischemia Anomalous origin of a coronary artery from the
opposite sinus (ACAOS); coronary artery fistulas
Obligatory ischemia Anomalous left coronary artery from the pulmonary
artery (ALCAPA)
Modified from Angelini P (ed): Coronary Artery Anomalies: A Comprehensive Approach. Philadelphia, Lippincott Williams &
Wilkins, 1999, p 42.

12. Anomalous origin of a coronary artery from the opposite sinus (ACAOS)
a. RCA from left anterior sinus 0.92%
b. LAD from right anterior sinus
c. LCx from right anterior sinus 0.67%
d. LCA from right anterior sinus 0.15%
• ARCA~ 6 times more prevalent than ALCA
• ALCA has a higher risk of SCD
Paolo AngeliniCoronary Artery Anomalies ,An Entity in Search of an Identity; Circulation 2007;115:1296-
1305

13. Maron BJ, Thompson PD, Ackerman MJ, et al. Recommendations and considerations related to preparticipation screening for cardiovascular
abnormalities in competitive athletes: 2007 update. Circulation 115, 164 3–1655 ( 2007).
CLINICAL PRESENTATION
SUDDEN CARDIAC DEATH INYOUNG ADULTS

14. CLINICAL PRESENTATION
< 40 years
SUDDEN DEATH
PRECEEDED BY SEVERE
EXERTION
MALES
> 40 years
DYSPNEA, PALPITATIONS,
ANGINA PECTORIS,
DIZZINESS, SYNCOPE
FEMALES
NEW ONSET
HYPERTENSION
RobertsWC, Shirani J.The four subtypes of anomalous origin of the left main
coronary artery from the right aortic sinus (or from the right coronary
artery). Am J Cardiol. 1992;70:119 –121.

15. PATHOPHYSIOLOGY OF ISCHEMIA
IN CORONARY ANOMALIES
• Slit-like coronary orifice
• Acute angle of take-off from aorta
• Inter-arterial course between aorta and PA
• Spasm secondary to endothelial damage
P.Angelini. Coronary artery anomalies : An entity in search for identity. Circulation.
2007;115:1296-1305.

18. • OSTIAL IDENTIFICATION
• OPTIMAL GUIDE CATHETER : CO-AXIAL AND GOOD SUPPORT
• ADDITIONAL SUPPORT : BUDDYWIRE, BALLOON ANCHORAGE
• HARDWARETRACKING
ANOMALOUS RIGHT CORONARY ARTERY (ARCA) (0.92%):
CHALLENGES

19. OSTIAL IDENTIFICATION
Catheter Selection for Coronary Angiography and Intervention in Anomalous Right Coronary Arteries.
Sarkar K, Sharma SK.J Interven Cardiol 2009;22:234–239

20. LAO 30–35◦ projection

21. Distribution of coronary catheters for selective coronary angiography and PCI of anomalous RCA based on the site of origin in theaortic sinuses of Valsalva. RCA = right
coronary artery; VL = Voda left; AL = Amplatz left; FL = forward take-off JL, FCL = femoral curve left; JR = Judkins right.

22. Common guiding catheter shapes used for ARCA-LSOV. FCL curve, Q curve (FCL for large aortic root size),
Voda (VL), forward takeoff Judkins (FL), and standard Judkins (JL) catheters are represented.

23. Indian Experience, N=17
TYPE A
TYPE B
TYPE C
Kalaichelvan Uthayakumaran, et al. Coronary intervention in anomalous origin of the right coronary artery (ARCA) from the left sinus of valsalva (LSOV): A
single center experience. Indianheartjournal66(2014)430e434

25. Abnormal origin of right coronary artery and use ofTiger catheter through femoral
route
Goutam Datta a,*, Durga Prasad Rai. indianheartjournal68(2016)102–105
• N = 5120 patients
Judkin catheter -Type 1.
Amplatz catheter -Type 2, and
MP catheter was useful in Type 3 origin.
For Type 4, no specific catheter is useful
JL in Type A, FCL (femoral curved left) 3,3.5 for Type B,
Voda left for Type C, and AL-1,2 for Type D.
Judkin catheter was useful in normal and low take-off
origin.
AL catheters are helpful in high take-off cases. The have
used EBU or AL catheter in few cases.

26. Tiger catheter from femoral route in these cases.
Advantage of this catheter is that it can take different
shapes by push, pull, torque, and rotation.
Its tip is also very soft and there is less chance of
dissection.

27. HOWTO IMPROVE SUPPORT ?
• TWO WIRES
• BALLOON ANCHORAGE
• UNDERSIZED GUIDE CATHETER
• STEAM DEFORMATION AND UNDERSIZING
• SPECIAL CATHETERS

28. TwoWires
Stabilizing a guide with the “buddy” wire technique
• When working with an unstable guide, a second angioplasty wire can be
advanced parallel to the first one.
• It straightens the tortuous vessel and provides better support for device
tracking.
• A second wire in a side branch can be very useful in “anchoring” the guide (e.g.
second wire in the LCX when dilating an LAD lesion).
• This provides for better “backup” and allows retraction of the guide when
necessary, without loss of position.

29. CASE : 45 M IWSTEMI
( ARCAOS 0.92% )

31. UNDERSIZING OF JL 3
Primary percutaneous coronary intervention of an anomalous right coronary artery arising from the left coronary cusp using
an undersized Judkins catheter: A case report J Chris Matchison MD, David M Shavelle.Int J Angiol 2007;16(1):33-35.
CASE : 60/M, IWSTEMI

32. CASE : 54/M, IWSTEMI
• Radial Route
• Undersized JL 3
• TWO 0.014 PTCA wires
in RCA
J Clin Case Rep 2014, 4:11

33. STEAM-DEFORMED JUDKINS-LEFT GUIDING CATHETER
Steam-deformed Judkins-left guiding catheter for anomalous right coronary artery.Toshiki Kuno. SAGE Open Medical Case Reports 3:
2050313X15608339.
Case:
88/F, NSTEMI

34. Case: 71/F, IWMI
• MULTIPLE FAILED ATTEMPTS WITH JL, EBU,AL
• IKARI-LTHROUGH FEMORAL ROUTE
• ADDITIONAL SUPPORT FROM CONTRALATERAL
WALL AND LEFT SINUS
SPECIAL CATHETERS
Porwal, S.C., Vishwanath, H., Tasgaonkar, R., Sitapara, T. and Thakkar, A. (2014) Percutaneous Coronary Intervention in Chronic Total Occlusion of Anomalous
Right Coronary Artery. International Journal of Clinical Medicine, 5, 567-571

36. CASE : 50/M, AWSTEMI (RCA from mid LAD)

38. ANOMALOUS LM FROM R SINUS (0.15%)
Subtypes:
1. Inter-arterial : SCD 50%
2. Anterior free-wall course
3. Retro-aortic course
4. Septal course-most common
Yamanaka O, Hob bs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet
Cardiovasc Diagn 1990;21:28

39. Dot And Eye Sign
• Each course can be described in a simple method, coined by Serota H et
al1, the “dot and eye” method

40. RAO PROJECTION : BEST SEPERATES PA FROM AORTA
INTER-ARTERIAL
RETRO-AORTIC
ANTERIOR
SEPTAL
DOT EYE
Kimbiris D., et al. Circulation 58:606-15, 1978.

41. “DOT”

42. EYE IN RAO

43. “..anomalous coronaries are more likely to be encountered as
the culprit infarct-related artery in the hemodynamically
unstable patient.”
“A key determinant to proceed with percutaneous or surgical
revascularization depends on demonstration of the origin and
course of the anomalous coronary and the hemodynamic and
general clinical status of the patient.”
“Application of the “dot and eye” method can be useful to
choose the most appropriate revascularization strategy.”
Primary Angioplasty of an Anomalous Left Main Coronary Artery: Diagnostic andTechnical
Considerations. Linzieri M. Catheterization andCardiovascular Interventions 58:185–188 (2003).

44. SURGICAL OPTIONS FOR L-ACAOS
• DIRECT RE-IMPLANTATION
• UNROOFING

45. Coronary Artery Anomalies, Grossman & Bairn's Cardiac Catheterization, Angiography, and Intervention, 8th edition, chapter
16, pg. no. 347.

46. 2018 ACHD Guideline: Executive Summary

47. 2018 ACHD Guideline: Executive Summary

48. ANOMALOUS LCX FROM R SINUS : 0.67%
• Type I: Separate ostia for right coronary artery (RCA) and
LCX.
• Type II:Common ostia in the right sinus.
• Type III: LCX arising as a branch of the proximal RCA.
Uncommon variants of left circumflex coronary artery (LCX): evaluation with 256-slice dual source CT coronary angiography. Rissam HK et al. BMJ Case
Reports 2015; doi:10.1136/bcr-2015-210495
Anomalous origin of LCX from right coronary sinus (arrows in B) with retroaortic course (arrowheads in A)

49. CASE : 48/F, IWSTEMI
(ANOMALOUS LCX FROM R SINUS : 0.67%)
AR
JR

51. SPLIT RCA (1.23%)
• Split RCA is defined as an RCA that features a
split posterior descending branch
• Anterior subdivision leading to the distal portion
of the PDA and supplies distal portion of posterior
septum, inferior wall of LV and anterior free wall
of RV
• Posterior subdivision maintains a course in the AV
groove and forms the uppermost portion of the
PDA, supplying the proximal (basal) posterior
interventricular septum
• The length of each of the 2 posterior descending
branches varies from patient to patient
Angelini P, Villason S, Chan AV Jr, Diez JG. Normal and anomalous coronary arteries in humans. In: Angelini P, editor. Coronary artery
anomalies: a comprehensive approach. Baltimore: Lippincott Williams & Wilkins; 1999. p. 27-79.

52. Case: 54/M, NSTEMI

53. Surender Deora, Sanjay Shah, Tejas Patel. Double or split right coronary artery: Still a diagnostic dilemma for this rare coronary anomaly.
http://dx.doi.org/10.1016/j.ijcard.2013.11.058

55. Coronary OstialVariations

56. High take-off RCA (0.202%)
Defined as RCA that arises at least 1 cm above the sino-tubular junction (Vlodaver et al., 1975; Loukas et al., 2009b)

57. When the right coronary ostium is very high, the
left Amplatz guide may be used to engage the
right ostium.

58. Shepherd’s Crook RCA (4.9%)
• Shepherd’s crook deformity of the RCA is a dramatic upturn with a near-180° switchback
turn.
• It is a haemodynamically non-significant course anomaly
Shriki JE, Shinbane JS, Rashid MA et al. Identifying, characterizing, and classifying congenital anomalies of the coronary arteries.
RadioGraphics, 2012; 32: 453–468.

59. Case: 45/M, NSTEMI
Amplatz L-2 guide catheter was used to
hook RCA which gives the good coaxial
support.
Other Catheters
• SCR
• Arani
• IMA
• HS
Alok Kumar Singh. A case of non-ST elevation myocardial infarction presenting with Shepherd’s crook right coronary artery with tortuosity: Issues in
management. Heart India / Volume 6 / Issue 1 / January-March 2018

60. Dual LAD (0.13%)
• Dual LAD consists of a short LAD that ends high in the AIVG and a long LAD that most
commonly originates as an early branch of the LAD proper (types 1–3) and rarely originates
anomalously from the right coronary artery (type 4).
• Proposed originally by Spindola in 1983
• He classified it into 4 types
• In recent years the dual LAD has increased from 4 to 6 types.

62. Case: 54/M, NSTEMI (TypeVI LAD)
RCA: MPC f/b PTCA to RCA and PDA
Long LAD: JR4 f/b POBA
Short LAD: JL4 f/b PTCA to short LAD crossing over to D1
Lee et al. A case report of type VI dual left anterior descending
coronary artery anomaly presenting with non-ST-segment elevation
myocardial infarction. BMC Cardiovascular Disorders 2012, 12:101

63. Absent LMCA
• N = 407
• The LMCA was absent in 9.6%
• The LAD and LCx arteries originated directly from
the LCS with a single ostium in 8.6% (35/407)
• and separate ostia in 1% (4/407) of the angiograms
N. O. Ajayi, L. Lazarus, E. A. Vanker and K. S. Satyapal. Absent Left Main Coronary Artery with Variation in the Origin of its
Branches in a South African Population. Anat. Histol. Embryol. 44 (2015) 81–85

65. Case: 64/M, Atypical Chest Pain
Yalcin Hacioglu and Matthew Budoff. Is the Left Anterior Descending Artery Really Absent?— A Decisive Input from Coronary
CT Angiography. Catheterization and Cardiovascular Interventions 76:117–120 (2010

66. Single Coronary Artery (SCA) (0.05%)
• “Single coronary artery” is a misnomer: because all of the normal coronary
arteries are present
• this entity is not defined by the presence of a single coronary “artery” but
by the fact that all these arteries originate from a single ostium.
• This term is clinical jargon used to refer to cases of CAA that feature a
single coronary ostium, which provides flow to the whole coronary tree
without interruption of continuity and without evidence of collateral
vessels.

67. Lipton MJ, Bany WH et al: Isolated single coronary artery: diagnosis, angiographic classification, and clinical
significance. Radiology 13039-47, 1979
Classification of SCA

68. Case: 75/M, CSA
Mohit DG, Girish MP, Subhendu MSK, Sanjay T. Percutaneous coronary intervention of all three coronary arteries originating
from single coronary artery arising from the right sinus. Cardiovasc Interv and Ther (2013) 28:322–326
AR2 AR2

69. Mohit DG, Girish MP, Subhendu MSK, Sanjay T. Percutaneous coronary intervention of all three coronary arteries originating
from single coronary artery arising from the right sinus. Cardiovasc Interv and Ther (2013) 28:322–326
3DRC AR2

70. Mohit DG, Girish MP, Subhendu MSK, Sanjay T. Percutaneous coronary intervention of all three coronary arteries originating
from single coronary artery arising from the right sinus. Cardiovasc Interv and Ther (2013) 28:322–326

71. Case: 58/M, Acute IWMI
Triple anomaly of origin of SCA from ascending aorta, acute take of RCA from
SCA and anomalous inter-arterial course.
Mohit DG, Girish MP, Ankit Bansal and Sanjay Tyagi. Primary percutaneous intervention in anomalous right coronary artery originating
from anomalously arising single coronary trunk. European Heart Journal - Case Reports (2018) 2, 1–2
AL2 EBU

72. Mohit DG, Girish MP, Ankit Bansal and Sanjay Tyagi. Primary percutaneous intervention in anomalous right coronary artery
originating from anomalously arising single coronary trunk. European Heart Journal - Case Reports (2018) 2, 1–2

73. Case: 45/M, Acute AWMI
(SCA from Ascending Aorta)
JL AL2
Mohit DG, Girish MP, Ankit Bansal, Vivek Chaturvedi, Vijay Trehan and Sanjay Tyagi. Primary percutaneous coronary intervention in an
anomalous single coronary trunk arising anomalously from ascending aorta. Cardiovasc Interv and Ther. DOI 10.1007/s12928-015-0343-3

74. Mohit DG, Girish MP, Ankit Bansal, Vivek Chaturvedi, Vijay Trehan and Sanjay Tyagi. Primary percutaneous coronary intervention in an
anomalous single coronary trunk arising anomalously from ascending aorta. Cardiovasc Interv and Ther. DOI 10.1007/s12928-015-0343-3

75. CORONARY OSTIAL STENOSIS OR ATRESIA (COSA)
Congenital Coronary Artery Ostial Disease A Spectrum of AnatomicVariants with Different Pathophysiologies and
Prognoses. P.Angelini. Texas heart journal. 2012.

76. CLINICAL SIGNIFICANCE OF COSA
• Absence of clinical angina (or ischemia at
stress testing)
• Myocardial scarring is very rare.
• Very rarely is a surgical bypass
intervention required
• Collateral vasculature is usually
adequate.
CongenitalCoronary Artery Ostial DiseaseA Spectrum
of AnatomicVariants with Different Pathophysiologies
and Prognoses. P.Angelini.Texas heart journal. 2012.
1 or 2 full diameter
connecting collateral
No transition
No STEP-UP

77. Case: 48/M, USA
Girish MP, Mohit DG, Vivek C, Amit G, Sonali S. Ostial atresia of left circumflex coronary artery arising from non-
coronary sinus: a combination of rare anomalies. Cardiovasc Interv and Ther. DOI 10.1007/s12928-015-0350-4

78. Girish MP, Mohit DG, Vivek C, Amit G, Sonali S. Ostial atresia of left circumflex coronary artery arising from non-
coronary sinus: a combination of rare anomalies. Cardiovasc Interv and Ther. DOI 10.1007/s12928-015-0350-4

79. Simultaneous coronary angiogram of RCA and LCA in LAO – cranial view showing ostial atresia of RCA and
opacification of RCA by intercoronary communication from left circumflex artery running in AV groove.
Case: 62/M, Atypical Chest Pain
Mohit DG, Girish MP, Vijay Trehan, Sanjay Tyagi. Absent right coronary artery: A case of single coronary artery or congenital ostial
atresia? i n d i a n h e a r t j o u r n a l 6 7 ( 2 0 1 5 ) s 1 1 – s 1 3

80. Mohit DG, Girish MP, Vijay Trehan, Sanjay Tyagi. Absent right coronary artery: A case of single coronary artery or congenital ostial
atresia? i n d i a n h e a r t j o u r n a l 6 7 ( 2 0 1 5 ) s 1 1 – s 1 3

81. ALCAPA
• 1 in 300,000
• LV failure
• 90% do not survive infancy
• Sx asap
• Have been diagnosed in patients upto 72 yrs

82. ALCAPA

83. CASE : A 30-year-old woman , previous patch repair of an AVSD at
age 2 yr. Routine follow up echocardiogram she was found to
have continuous low velocity flow along the mid to distal RV
septum with a dilated RCA ostium.
Successful percutaneous treatment of anomalous left coronary artery from pulmonary artery.
N.Collins et al. International Journal of Cardiology 122 (2007) e29–e31

84. Successful percutaneous treatment of anomalous left coronary artery from pulmonary
artery. N.Collins et al. International Journal of Cardiology 122 (2007) e29–e31
JR 3.5

85. CASE : # 2.9 kg term baby, underwent ALCAPA resection
and reimplantaion with PA reconstruction.
# Normalisation of LVEF over next 2 weeks.
# 4 weeks post op, FTT, sev LVSD, sev MR.
4 Fr JR

87. 2018 ACHD Guideline: Executive Summary

88. MISSINGARTERIES

91. TAKE HOME MESSAGE
• TAILOR MADE APPROACH
• INTERVENTION IS POSSIBLE IN A SELECT FEW
• DOT AND EYE
• GUIDE SELECTION AND SUPPORT
• INNOVATIONS DURING INTERVENTION