Coronary Artery Anomalies by bBz3bT3

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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)
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 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
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
• 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
  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
   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
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
                   Chest radiography
                   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
     a. Closure through arteriotomy & aneurysm
     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
  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
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
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 sino-
   tubular 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
 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
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
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
  Anomalous Coronary Artery
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
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
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
• 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
      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.

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