Download Congential Anomalies of the Coronary Arteries

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)
1. Major anomalies
- Coronary arterio-venous fistula
- Anomalous origin from PA
Left coronary artery
Right coronary artery
Both coronary arteries
2. Minor variations
- High take-off
- Anomalous circumflex artery origin
- Single ostium or multiple ostia in
other aortic sinus
- Hypoplastic proximal coronary artery
- Congenital proximal or distal stenosis
- Coronary artery from the posterior
aortic sinus
- Ventricular origin of an accessory
coronary artery
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 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
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
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 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 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
Coronary Artery – RV Fistula
Coronary A-V Fistula
 Technique of operation
1. Closed without CPB
When termination of a major coronary artery
branch into an easily accessible site
2. 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
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
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
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)
ALCAPA (1)
Anomalous Connection of Left Coronary
Artery to Pulmonary Artery
ALCAPA
(2)
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.
ALCAPA
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
cardiomyopathy
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
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
ALCAPA
Tunnel Operation
ALCAPA
Implantation of Anomalous Artery
ALCAPA
Subclavian-left Coronary Artery Anastomosis
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
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.
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.
Anomalous Coronary Artery
Connection to Aorta
1. 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 sinotubular junction (apparently clinical events are related to this
condition only when the anomalously arising artery passes
between aorta & pulmonary trunk)
2. History
Cheitlin : Description of death of anomalous LCA in 1974
Roberts : Description of anomalous RCA in 1984
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
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
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
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)
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
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
 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
Anomalous Coronary Artery
Connection
Unroofing Procedure
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
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
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.