1. Coronary Artery Anomalies
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery

2. Coronary Artery Anomalies
Incidence : 1-2% of the population including minor
(major anomalies<1% of CHD)
1. Major anomalies Minor variations
- Coronary arterio-venous fistula High take-off
- Anomalous origin from PA Anomalous circumflex artery origin
- Single ostium or multiple ostia in
Left coronary artery other aortic sinus
Right coronary artery Hypoplastic proximal coronary artery
Both coronary arteries Congenital proximal or distal stenosis Coronary artery from the posterior
aortic sinus
- Ventricular origin of an accessory
coronary artery

3. Coronary Artery Anomalies
Major categories
1. Coronary arterio-venous fistula
2. Anomalous connection of left or right
coronary artery to pulmonary artery
3. Anomalous origin of a main coronary
artery from the aorta

4. Coronary Artery Anomalies
Pathophysiology
The anomalous origin of the left coronary artery on the main pulmonary artery leads to reversal of the left coronary arterial flow. This anomalous circuit results in coronary steal, myocardial ischemia, left ventricular dysfunction, and congestive heart failure.
Coronary arteriovenous fistulae between the left or right coronary arteries and the right heart chambers or the pulmonary artery also create a coronary steal phenomenon and myocardial ischemia.

5. Coronary Arterio-venous Fistula
Definition
Direct communication between a coronary artery and the lumen of any one of the four cardiac chambers or the coronary sinus, or its tributary vein or SVC, pulmonary artery or pulmonary veins close to the heart
History
Krause : 1st description in 1865
Trevor : 1st autopsy report in 1912
Biorck & Crafoord
: 1st report of surgical correction in 1947
Fell : 1st report of correct diagnosis & surgical correction
in 1958
Swan : 1st report of surgical correction by CPB in 1959

6. Coronary A-V Fistula Morphology
1. Coronary artery site : dilated, elongated, serpiginous,
Right coronary A : 50 ~ 55%, Left coronary A : 35%
Both : 5%
2. Site of fistulous connection
Right ventricle : 40% Right atrium : 25%
Pulmonary artery : % Coronary sinus : 7%
SVC : 1% Left atrium : 5%
Left ventricle : 3% Other : rarely
3. Size & multiplicity of the fistula
2 ~ 5mm fibrous margin, single or multiple opening
4. Cardiac chamber : dilation in atrial, venous drain site
5. Bacterial endocarditis : 5%
6. Associated lesions : most occur as isolated lesions

7. Coronary A-V Fistula Natural history
The fistula, if not present at birth, develops early in life. Likely small fistulae remains small and moderate fistulae slowly increase in size, although there may be little change over a 10 to 15 year period.
Onset of dyspnea, heart failure, and angina can occur in young patients with large fistula
The maximum incidence of congestive heart failure occurs in the fifth and sixth decades.
Spontaneous rupture and closure is very rare.

8. Coronary A-V Fistula Clinical features & diagnosis
1. Presentation ; Most present late age in life
2. Symptoms ; Asymptomatic in young age
Mild cardiomegaly
Plethora on chest X-ray
DOE from Lt. to Rt. shunt
Angina (7%)
Myocardial infarction (3%)
Congestive heart failure (12 ~ 15%) in old age
3. Diagnosis ; Continuous murmurs
EKG
Chest radiography
Echocardiography
Cardiac catheterization, and angiography

9. Coronary Artery – RV Fistula

10. Coronary A-V Fistula Technique of operation 1. Closed without CPB
When termination of a major coronary artery
branch into an easily accessible site
When CPB is used
Indications; (1) when artery is dilated & tortuous &
relatively inaccessible as in AV groove (2) when the
fistula is in the course of coronary artery (3) when
an aneurysm requires excision
Methods
a. Closure through arteriotomy & aneurysm
repair
b. Closure through chamber

11. Coronary A-V Fistula Surgical indications & results 1. Survival
◎ Early death
Hospital mortality : rare
Complication : rare
◎ Time related
Late results : excellent
2. Indications
Diagnosis is an indication for operation unless the
shunt is very small(QP/QS<1.3)
3. Controversies
Interventional catheter-delivered occluding devices
and coils have been reported successfully

12. Anomalous Connection of Left Coronary Artery to Pulmonary Artery
1. Definition
The whole of the left main or only the left anterior descending
or circumflex branch connects anomalously to proximal main pulmonary artery, or very rarely to proximal right pulmonary artery.
2. History
Brooks : 1st description in 1886
Bland, White & Garland (BWG Syndrome)
: Described clinical syndrome in 1933
Sabiston : 1st successful operation in 1959
Cooley : Bypass graft in 1966
Neches : Coronary transposition in 1974
Takeuchi : Tunnel repair in 1979

13. ALCAPA Morphology 1. Connection
◎ Left or posterior cusp of pulmonary trunk, rarely right cusp
◎ Uncommonly circumflex branch connection anomalously
◎ Rarely only the LAD connects anomalously
2. Left ventricle
◎ Hypertrophied, dilated
◎ Diffuse fibrosis in subendocardial layer
◎ Secondary subendocardial fibroelastosis
3. Mitral valve incompetence
◎ Extensive fibrosis, calcification in the papillary muscle
(dysfunction)
◎ Endocardial fibroelastosis in the mitral apparatus & LV
(fusion & shortening of chordae tendinae)

14. Anomalous Connection of Left Coronary Artery to Pulmonary Artery
ALCAPA (1)
Anomalous Connection of Left Coronary Artery to Pulmonary Artery

15. ALCAPA (2)

16. ALCAPA Clinical features & diagnosis 1. Infant presentation
Symptoms may be recognized within a week or so of birth (high postnatal
pulmonary artery pressure limits runoff into the pulmonary artery)
EKG : Anterolateral infarct with Q waves & ST segment elevation, LVH
Chest radiology : Cardiomegaly with interstitial pulmonary edema
Echocardiogram
Cineangiography
2. Adult presentation
Collateral circulation is adequate to prevent infarction.
When severe symptoms do not occur in infancy, presentation is often
delayed to beyond 20 years of age.
Some patients remain asymptomatic.

17. ALCAPA Natural history 1. Incidence 2. Survival
rare (0.26%) , 1 per 400 CHD
2. Survival
65% : die due to LV failure during the 1st year of life
lf death does not occur during the 1st year,
the hazard lessens considerably by
Rich interarterial collateral
Restrictive opening of left coronary artery
Marked right coronary dominance
Most patients who survive infancy continue to be a risk of
death from chronic heart failure secondary to ischemic
cardiomyopathy

18. ALCAPA Techniques of operation Construction of a two-coronary system
Tunnel operation (Takeuchi Repair)
Left coronary artery transfer
Subclavian-left coronary artery anastomosis
Other techniques for assisting reimplantation
Extension by autologous flaps of aorta or pulmonary artery
Mobilization with pulmonary button
Coronary artery bypass grafting
Ligation of left coronary artery

19. ALCAPA Goals of surgery Establishing the 2-coronary system with direct
aortic implantation
Situations where direct aortic implantation is
not suitable
1. Anomalous orifice is located in the nonfacing
sinus or in the lateral position on the left
pulmonary sinus
2. The patient is older with well-developed
collateral around the pulmonary sinus

20. ALCAPA
Tunnel Operation

21. Implantation of Anomalous Artery
ALCAPA
Implantation of Anomalous Artery

22. Subclavian-left Coronary Artery Anastomosis
ALCAPA
Subclavian-left Coronary Artery Anastomosis

23. ALCAPA Surgical results 1. Survival Early death : variable
Time-related : good
2. Mode of death : acute cardiac failure
3. Incremental risk factors for premature death
Status of left ventricular myocardium
lmportant mitral incompetence
4. Functional status : generally good late postoperatively
5. Left ventricular function
6. Mitral incompetence
7. Effect of the nature of the postoperative coronary system
8. Conduit patency after the two-vessel repair
9. Right ventricular outflow obstruction after a tunnel repair

24. ALCAPA Indications for operation
The diagnosis in an infant is an indication for urgent operation and this condition in older patients as well.
The mitral valve should be left alone when operation is performed in young patients, but in older patients mitral repair or replacement may be required.

25. ALCAPA Special Situations & Controversies
1. Anomalous connection of the right coronary artery, circumflex coronary artery, or left anterior descending coronary artery to pulmonary trunk
. Even rarer
. Much less lethal
. Operation is probably advisable.
2. Total anomalous connection of the coronary arteries
to the pulmonary trunk
. Very rarely
. Symptoms appear within a few days of birth and
death follows within 2 weeks.
. Urgent operation should be carried out.

26. Anomalous Coronary Artery Connection to Aorta
Definition
A condition in which either the main coronary artery arises from the aorta in a site other than the left coronary sinus or the aorta just above it ; or in which right coronary artery arises in a site other than the right coronary sinus or the sino-tubular junction (apparently clinical events are related to this condition only when the anomalously arising artery passes between aorta & pulmonary trunk)
History
Cheitlin : Description of death of anomalous LCA in 1974
Roberts : Description of anomalous RCA in 1984

27. Anomalous Coronary Artery Connection
Morphology
1. Anomalous connection of left main coronary artery
. Two coronary ostia are side by side in the right sinus mostly.
. Single enlarged ostium in the right sinus near to or overlying
commissure between right and left less commonly
. Coronary artery may pass posteriorly between aorta & PA trunk
2. Anomalous connection of right coronary artery
. More common than anomalous connection of the left main coronary artery
. The anomalously connecting right coronary artery passes forward between the two great arteries.
. Commonly seperated ostium, but uncommonly single ostium
within left sinus or over the commissure

28. Anomalous Coronary Artery Connection
Restriction of coronary flow
Acute angulation at the coronary takeoff
Presence of the ostial ridge, slitlike orifice,
Stretching and compression of the intramural segment by the aortic valve commissure
Most recently, variable lateral luminal compression of the intramural trunk that worsens during systole
RCAs arise from the left sinus or ectopically in the right sinus.

29. Anomalous Coronary Artery Connection
Medical (expectant) treatment
Coronary bypass grafting
Implantation into the correct sinus from outside the aorta
Translocation of the pulmonary artery
The use of primary angioplasty in patients with myocardial infarcts
The use of stents to prevent compression
Transaortic modification of the origin and proximal portion of the ectopic or anomalous RCA

30. Anomalous Coronary Artery Connection
Clinical features & diagnosis
The natural history is controversial.
No characteristic clinical or electrocardiographic
feature
Diagnosed by coronary cineangiography
A search is made for objective evidence of reversible
ischemia (A number of cases have been reported in
which serious sequelae, including sudden death)

31. Anomalous Coronary Artery Connection
Surgical Strategies
Anomalous connection of a coronary artery (ACCAA) to an incorrect sinus of Valsalva is a relatively rare congenital defect
Coronary reimplantation,
Unroofing intramural segment,
Coronary artery bypass grafting.
Reinplantation of pulmonary trunk

32. Anomalous Coronary Artery Connection
Compromised coronary blood flow
Compression of the intramural segment of the coronary artery between the pulmonary artery and aorta
Narrowing of the slit-like orifice to anomalous artery with increased aortic pressure
An acute angle of the takeoff of the coronary artery
Stenosis in intramural segment of the coronary artery, especially at the level of the commissure.

33. Anomalous Coronary Artery Origin
Operative indications & techniques
1. Indication
Indications remain arguable, but reversible ischemia is an
indication for operation.
2. Technique
. Coronary bypass graft operation
internal mammary artery
saphenous vein graft
. Reconstruction of proximal portion of the coronary artery
(lntramural course)
. Division and reimplantation
3. Results
Generally good

34. Anomalous Coronary Artery Connection
Unroofing Procedure

35. Coronary Ostial Stenosis
Developmental etiology
1. Congenital
1) Membranous obstruction
2) Hypoplasia
3) Atresia
2. Inflammatory
1) Takayasu’s : 8.5%
2) Kawasaki : 9%
3) Middle aortic syndrome

36. Coronary Artery Aneurysm
Development & diagnosis
The first pathologic description of a coronary artery aneurysm was by Morgagni in 1761, and the first clinical case of coronary artery aneurysm was reported by Bourgon in 1812.
Coronary artery aneurysm is an uncommon disease, the main causes of coronary artery aneurysm include congenital, atherosclerosis, Kawasaki disease, complication of percutaneous transluminal coronary angioplasty, and endocarditis.
On rare occasions, a coronary artery aneurysm grows large enough to be called giant coronary artery aneurysm, for which a precise definition is still lacking

37. Coronary Artery Aneurysm
Clinical features
Coronary artery aneurysm is defined as coronary dilation that exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times.
Coronary artery aneurysm is an uncommon disease, found in only 0.15% to 4.9% of patients who undergo coronary angiography.
The complications of coronary artery aneurysm include thrombosis, embolization, rupture, and vasospasm
Operative procedures include coronary fistula closure, underwent coronary artery aneurysm resection, embolectomy, and plastic repair of the aortic wall with partial excision of the free aortic wall.