Anomalous origin of the left coronary artery from the pulmonary artery

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[edit] Discussion of Anomalous origin of the left coronary artery from the pulmonary artery

  • Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is otherwise known as Bland-White-Garland syndrome
  • Rare congenital defect that accounts for 0.25–0.5% of all congenital heart defects.
  • It most often presents as an isolated defect.
  • Before they are 1 month old, however, physiologic pulmonary hypertension tends to preserve antegrade blood flow within the left coronary artery, accounting for the usual lack of symptomatology in this age group
  • Symptoms usually occur in infants after they are 1–2 months old because of left-to-right shunting from the higher pressure left coronary arterial system to the lower pressure pulmonary arterial system.
  • This most often results in death due to circulatory insufficiency from left ventricular dysfunction or mitral valve incompetence, myocardial infarction, or life-threatening cardiac dysrhythmias.
  • Without treatment, approximately 90% of infants die within the first year of life.
  • Factors that may lead to survival beyond infancy include the development of abundant intercoronary collateral arteries, an alteration in hemodynamics that encourages antegrade blood flow into the left coronary arterial tree, and a reduction in the area of the left ventricular myocardium supplied by the LCA.
  • In general, once the diagnosis of ALCAPA is established, early surgical repair is undertaken to prevent potential future complications.

[edit] Imaging Findings for Anomalous origin of the left coronary artery from the pulmonary artery

  • On CT and MRI, previously described imaging findings of ALCAPA in adults include direct visualization of the origin of the LMCA from the posterior aspect of the PA, dilatation of the RCA, and visualization of dilated intercoronary collateral arteries along the external surface of the heart or within the interventricular septum.


[edit] Images

Patient #1

[edit] See Also

[edit] External Links

[edit] References for Anomalous origin of the left coronary artery from the pulmonary artery