Coronary Artery Anomalies:Angiographic Recognition of High Risk

Richard E. Browne, MD

John D. Symanski, MD

Case Presentation

• 43-year-old male with longstanding history of palpitations

• Undergoes cardiac CT scan which reveals abnormal coronary calcium score and suspected abnormal origin of left coronary

• Referred for diagnostic catheterization (echo also performed)

Coronary Artery Anomalies

Pete Maravich(June 22, 1947 – January 5, 1988)

Atherosclerotic CAD (2%)

Other congenital diseases (1.5%)

MVP (2%)

Myocardial scarring (3%)

ARVC (3%)

DCM (3%)

Myocarditis (3%)

Aortic valve stenosis (4%)

Tunnelled LAD (5%)Ruptured aortic aneurysm

(5%)Other coronary

abnormalities (6%)

Aberrant coronary arteries (13%)

LQTS (0.5%)

Sarcoidosis (0.5%)

Sickle cell trait (0.5%)

"Normal" heart (2%)HCM (36%)

Unexplained cardiac mass (10%)

Sudden Cardiac Death in Athletes

Coronary Artery AnomaliesDemonstrated by Echocardiography

RAO

RAO

RAO

Myocardial BridgingTunneled LAD

• Autopsy: ~30%, Angiographically: <5%

• Prevalent in HCM patients

• Segment proximal to bridge frequently shows atherosclerotic plaque (tunnel spared)

• Symptomatic patients may be treated with

β-blocker or CCB

• Myotomy, CABG, and stenting in refractory cases

Myocardial BridgingTunneled LAD

Möhlenkamp et al. Circulation 2002;106:2616-22.

Möhlenkamp et al. Circulation 2002;106:2616-22.

High Takeoff Coronary OstiaAbove Sinuses of Valsalva

LAD and CFX Arising from Separate Ostia

Kimbiris D., et al. Circulation 58:606-15, 1978.

Retro-Aortic Left Circumflex

Kimbiris D., et al. Circulation 58:606-15, 1978.

Retro-Aortic Left Circumflex

Absent CircumflexSuper-dominant RCA

Absent Right CoronarySuper-dominant Circumflex

Left Coronary Arising From PABland-White-Garland Syndrome

Blood flows from the RCA via collaterals to the left coronaryartery, and then into the pulmonary artery.

Anomalous Coronary Arteries by Electron Beam Angiography

Circulation. May 27, 2003;107:2630

Anomalous RCATakeoff From Left Coronary Sinus

Kimbiris D., et al. Circulation 58:606-15, 1978.

*The most common, potentially serious coronary anomaly, accounting for 8.1% of serious coronary anomalies (25% incidence of sudden cardiac death).

Anterior Course of RCAArising From Left Coronary Sinus

Anomalous Right Coronary ArteryRCA Arising From Left Coronary Sinus

Coronary Artery AnomaliesMagnetic Resonance Imaging

Left Main Arising from Right Coronary Sinus

Subtypes:1. Anterior free-wall course2. Retro-aortic course3. Septal course4. Inter-arterial- incidence 1:12,500

[Accounts for 60% of anomalous left main from right coronary sinus (2.8% overall coronary anomalies). Recognized association with ischemic symptoms and sudden death >50%]

Left Main from Right SinusAnterior Course

The left main trunk arises ectopically from the right sinus of Valsalva and passes epicardially across the RV outflow tract.

Left Main from Right SinusAnterior Course

Anomalous Left CoronariesRetro-Aortic Circumflex, Anterior LAD

The LAD and circumflex branches arise from the right sinus of Valsalva. TheLAD passes epicardially across the RVOT, and the CFX passes behind the aorta.

Anomalous Left MainRetro-Aortic

The left main trunk originates from the right sinus of Valsalva and passes behind the aorta before dividing into the LAD and CFX branches.

Retro-Aortic Course of Left Main

Kimbiris D., et al. Circulation 58:606-15, 1978.

*Dot-sign posterior to aortic root

Anomalous Left MainInter-Arterial

The left main trunk arises ectopically from the right sinus of Valsalva and passes between the aorta and pulmonary artery.

Inter-arterial Course of Left MainArising From Right Coronary Sinus

Kimbiris D., et al. Circulation 58:606-15, 1978.

*Dot sign anterior to aortic root

Anomalous Coronary Artery

• 15-year-old male soccer player

• Syncope one year prevously (ECG normal).

• Died suddenly while running

• Acute angle take-off of LMCA from right coronary sinus

Left Main from Right SinusSeptal Course

The left main trunk originates from the right sinus of Valsalva and passes intramuscularly to the septum before reaching an epicardial position.

Left Main from Right SinusSeptal Course

Anomalous Left MainAngiographic Features of Subtypes

Kern, MJ. The Cardiac Catheterization Handbook, 1991