This document summarizes a case presentation on a 40-year-old previously healthy woman who was found unresponsive at home. She was brought to the hospital where cardiac biomarkers were markedly elevated, indicating a non-ST-segment myocardial infarction (NSTEMI). Angiography revealed several congenital coronary artery anomalies including coronary artery fistulae and an anomalous left main coronary artery originating from the right sinus of Valsalva with an intra-atrial course. The presentation reviews various congenital coronary artery anomalies that can cause myocardial ischemia or sudden death including anomalous coronary artery origins, coronary artery fistulae, and coronary artery stenosis or atresia.

1. Cardiothoracic / Cardiology
Combined Confernce
Michael G. Katz, MD
Fellow in Cardiovascular Disease
University of Rochester
April 4, 2011

2. 2
Case - 2009
40 year old white woman
Generally healthy
Found unresponsive at home
EMS called by husband
Pt describes being found at home with a “pulseless arrest”
• Details not available
Evaluated at Newark Wayne Hospital

3. 3
Cardiac Biomarkers were markedly elevated
Patient transferred to RGH for management of NSTEMI and cardiac
catheterization

4. Review of Angiography
4

5. 5
Network of fistula:
pLCx  PA (common orifice)
pLAD  PA (common orifice)
Anomolous LM from right
sinus of Valsalva with
intraatrial course (between
aorta and PA)

6. Normal Coronary Anatomy
6

7. 7
Left Coronary
Caudal RAO
LAD
Diag
LM
OM
LCx
Diagonal
Slide courtesy of Dr. Fred S. Ling, MD

8. 8
Left Coronary
LAO Cranial
LAD
Diags
LCx OM
LM
Slide courtesy of Dr. Fred S. Ling, MD

9. 9
Right Coronary
LAO Cranial
RPL
AM or RV branch
PDA
Crux
AM
PDA
Slide courtesy of Dr. Fred S. Ling, MD

10. 10
Right Coronary
RAO
PDA
AM
SAN
AM
PDA
RCA
Slide courtesy of Dr. Fred S. Ling, MD

11. Congenital Anomalies of Coronary Arteries
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12. 12
Overall, infrequent (<1%) findings on
coronary arteriograph

13. Anomalies that cause myocardial ischemia
Coronary artery fistulae
• About half of the patients with a coronary artery fistula are
asymptomatic, but the other half develops HF, infective endocarditis,
mycocardial ischemia, or rupture of an aneurysm.
• Can arise from any of the coronary arteries, and drain into the RV, RA,
pulmonary artery, LV, or superior vena cava.
• Hemodynamic assessment is the best method to identify the left-to-
right shunt resulting from these fistulae
13

14. LCA origin from the pulmonary artery
• generally manifest as HF and myocardial ischemia in the first 4 months of
life. However, about 25% of patients survive to adolescence or adulthood
when they develop mitral regurgitation, angina, or HF.
• Aortography demonstrates a large RCA with the absence of a left coronary
ostium in the left aortic sinus. During the late phase of an aortogram,
patulous LAD and LCx branches are seen filling by collateral circulation from
RCA branches. Retrograde flow from the LAD and LCx opacifies the LMCA
and its origin from the main pulmonary artery. The clinical course of the
patient tends to be more favorable if extensive collateral circulation exists.
14

15. Congenital coronary stenosis or atresia of a coronary artery
• an isolated lesion or in association with other congenital diseases (e.g.,
calcific coronary sclerosis, supravalvular aortic stenosis,
homocystinuria, Friedreich's ataxia, Hurler's syndrome, progeria, and
rubella syndrome).
• When this occurs, the atretic vessel usually fills by means of collateral
circulation from the contralateral side
15

16. Anomalous origin of either coronary artery from the contralateral
sinus
• Associated with sudden death during exercise in young persons.
• Attributed to myocardial ischemia and electrical instability due to a slit-
like, flow-limiting ostium with acute takeoff angles of the aberrant
coronary arteries or by compression between the pulmonary trunk and
aorta.
• Origin of the RCA from the LCA or left aortic sinus with passage
between the aorta and the RV outflow tract is somewhat less
dangerous, but has also been associated with sudden death.
16

17. LM from the right: 4 possibilities
“Dot and eye” technique
More aptly: Dot -or- Eye technique
17
Anterior to PA
Retro-aortic
Intramyocardial (Septal)
Intra-arterial (between Ao and PA)

18. 18
“Eye”

19. 19
“Dot”

20. 20
“Eye”

21. 21
“Dot”

22. Our patient:
22
Dot

23. “Dot and eye”
23

24. 24