CONGENITAL CORONARY ARTERY ANOMALIES

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• CONGENITAL CORONARY ARTERY ANOMALIES

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CONGENITAL CORONARY ARTERY ANOMALIES

• Isolated congenital coronary artery anomalies
  have been described in approximately 1 of
  patients who undergo coronary angiography and
  0.3 at autopsy.
• The two most common types of isolated congenital
  coronary artery anomalies that are associated
  with myocardial ischemia during childhood
  include
• (1) Anomalous origin of a coronary (AOCA)
  from the opposite sinus of Valsalva.
• (2) Anomalous origin of a coronary from the
  pulmonary artery.

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Anomalous origin of a coronary (AOCA) from the
opposite sinus of Valsalva

• Anatomic Features
• AOCA from the opposite sinus of Valsalva where
  the right coronary artery (RCA) arises from the
  left sinus or the left coronary artery (LCA)
  arises from the right sinus of Valsalva.
•  
• The anomalous coronary artery either courses
  between the great arteries (interarterial),
  courses within the myocardial sulcus between the
  great arteries (intramyocardial), or within the
  anterior wall of the aorta (intramural).

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AOCA (Anatomic Features)

• AOCA from the opposite sinus of Valsalva has been
  associated with myocardial ischemia, ventricular
  arrhythmias, and sudden death, particularly when
  the anomalous coronary courses between the great
  arteries.
• The mechanisms by which myocardial ischemia
  occurs are unclear, but it may be due to
  compromise in coronary flow reserve as a result
  of slitlike ostium of coronary A. or acute
  angulation at its origin from the aorta. Also,
  AOCA has the risk of compression between the
  great arteries.

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Clinical presentation

• -         Asymptomatic.
• -         Symptoms of myocardial ischemia as
  chest pain.
• -         Exercise-induced sudden death.

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Diagnostic findings

• Transthoracic focused two-dimensional
  echocardiography and color Doppler imaging of the
  coronary arteries have become the most important
  noninvasive tools for identification of AOCA.
• In case of AORCA from left sinus, a red
  color Doppler signal will be seen in the
  intramural segment as flow moves toward the right
  sinus from its origin in the left sinus. The
  opposite is seen in case of AOLCA from right
  sinus
• Coronary angiography, ultrafast CT and/or
  MRI may be necessary to delineate coronary
  anatomy when echocardiography is not definitive.
• ECG and chest radiograph are not helpful.

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Surgical therapy

• Indicated for patients with symptoms of
  myocardial ischemia.
• Procedures include
• Coronary bypass graft placement.
• Patch enlargement of the anomalous
  coronary origin.
• Reimplantation of the anomalous CA to the
  appropriate sinus.
• Unroofing of the intramural segment of the
  anomalous CA.

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Anomalous origin of the left coronary artery from
the pulmonary artery (ALCAPA)

• In ALCAPA the blood supply to the left
  ventricular myocardium is severely compromised.
  Soon after birth, as pulmonary arterial pressure
  falls, perfusion pressure to the LCA becomes
  inadequate myocardial ischemia, infarction, and
  fibrosis result.
• In some cases, interarterial collateral
  anastomosis develop between right and left CA and
  blood flow in LCA is reversed, where it empties
  into PA, a condition known as myocardial steal
  syndrome. The LV becomes dilated, and perfomance
  is decreased. Mitral insufficiency is a frequent
  complication.

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Clinical presentation

• The timing of presentation is variable and is
  related to adequacy of collateralization from the
  RCA.
• Symptomatic infants present with clinical
  findings of CHF and echocardiographic features of
  severe dilated cardiomyopathy.
• LV dysfunction with chamber dilatation results
  and lead to tachycardia, tachypnea, hepatomegaly,
  and failure to thrive.
• ALCAPA must be excluded in all children who are
  diagnosed with dilated cardiomyopathy, because it
  is treatable with potential of complete recovery.

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Clinical presentation

• Patients who are diagnosed later in childhood
  often are asymptomatic and usually present with
  heart murmur or cardiomegaly on chest radiograph.
  
• However, ischemic injury still occurs, resulting
  in papillary muscle fibrosis, MVP, MI, and LV
  dilatation. Sudden death usually occurs with
  exercise.

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Diagnostic findings

• Chest radiograph cardiomegaly.
•         ECG as myocardial infarction.
•         Two-dimensional echocardiography is
  diagnostic in most cases. It may provide direct
  visualization of the anomalous coronary insertion
  into PA and RCA dilatation.
•       
• Color Doppler echocadiography can show
  abnormal diastolic flow signals within the
  myocardium and retrograde filling of LCA from the
  RCA.
•        Cardiac cathetarization with coronary
  angiography is the definitive confirmatory test.

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Treatment

• - Medical for CHF (diuretics, digoxin,
  captopril) and for ischemia (nitrates, B-
  blocking agents).
• - Surgical detachement of the anomalous CA and
  anastomosing it to the aorta to establish normal
  myocardial perfusion.

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B) Anomalous origin of the right coronary artery
from the pulmonary artery (ARCAPA)

• Angina and sudden death can occur in adolescence
  or adulthood.
• RCA arises from PA. It is rarely manifested in
  childhood.
• When recognized, it should be repaired by
  reanastomosis of of the RCA to aorta.

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(3) Coronary artery fistula

• A rare anomaly where there is a communication
  between a CA and another vascular structure.
• It usually connects to one of intracardiac
  chambers, systemic veins, or PA.
• Most patints are asymptomatic and present
  because of a continuous murmur along the
  precordium.

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