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Coronary Artery Anomalies. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. Coronary Artery Anomalies. Incidence : 1-2% of the population including minor (major anomalies<1% of CHD)

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Coronary Artery Anomalies

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coronary artery anomalies

Coronary Artery Anomalies

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery

coronary artery anomalies2
Coronary Artery Anomalies
  • Incidence : 1-2% of the population including minor

(major anomalies<1% of CHD)

1. Major anomalies 2. 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 coronaryartery - 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

coronary artery anomalies3
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

coronary artery anomalies4
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.
coronary arterio venous fistula
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

coronary a v fistula
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 : 15-20% 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

coronary a v fistula7
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.
coronary a v fistula8
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


Chest radiography


Cardiac catheterization, and angiography

coronary a v fistula10
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


a. Closure through arteriotomy & aneurysm


b. Closure through chamber

coronary a v fistula11
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

anomalous connection of left coronary artery to pulmonary artery
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

  • 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


◎ Endocardial fibroelastosis in the mitral apparatus & LV

(fusion & shortening of chordae tendinae)

alcapa 1

Anomalous Connection of Left Coronary Artery to Pulmonary Artery

  • 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



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.

  • Natural history

1. Incidence

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


  • 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
  • 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

  • Tunnel Operation
  • Implantation of Anomalous Artery
  • Subclavian-left Coronary Artery Anastomosis
  • 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

  • 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.
  • 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.

anomalous coronary artery connection to aorta
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

anomalous coronary artery connection
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

anomalous coronary artery connection28
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.
anomalous coronary artery connection29
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
anomalous coronary artery connection30
Anomalous Coronary Artery Connection
  • Clinical features & diagnosis
  • The natural history is controversial.
  • No characteristic clinical or electrocardiographic


  • 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)

anomalous coronary artery connection31
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
anomalous coronary artery connection32
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.
anomalous coronary artery origin
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

coronary ostial stenosis
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

coronary artery aneurysm
Coronary Artery Aneurysm
  • Development & diagnosis
  • The first pathologic description of a coronary artery aneurysmwas by Morgagni in 1761, and the first clinical case of coronaryartery aneurysm was reported by Bourgon in 1812.
  • Coronaryartery aneurysm is an uncommon disease, the main causes of coronary artery aneurysm include congenital, atherosclerosis,Kawasaki disease, complication of percutaneous transluminalcoronary angioplasty, and endocarditis.
  • On rareoccasions, a coronary artery aneurysm grows large enough tobe called giant coronary artery aneurysm, for which a precisedefinition is still lacking
coronary artery aneurysm37
Coronary Artery Aneurysm
  • Clinical features
  • Coronary artery aneurysm is defined as coronary dilation thatexceeds the diameter of normal adjacent segments or the diameterof the patient's largest coronary vessel by 1.5 times.
  • Coronary artery aneurysm is an uncommon disease, found in only0.15% to 4.9% of patients who undergo coronary angiography.
  • The complications of coronary artery aneurysm include thrombosis,embolization, rupture, and vasospasm
  • Operative procedures include coronaryfistula closure, underwent coronary artery aneurysm resection,embolectomy, and plastic repair of the aortic wall with partialexcision of the free aortic wall.