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HomePediatrics: Cardiac Disease and Critical Care MedicineSurgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery

Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery


Anomalous left coronary artery from the pulmonary artery (ALCAPA), or Bland-White-Garland syndrome,
 is a rare but very significant lesion that requires prompt recognition and diagnosis. In most patients with this condition, the origin of the left main coronary artery abnormally originates from the posterior or leftward sinus of the pulmonary artery. Less commonly, the anomalous coronary artery arises from the right pulmonary artery. The branching of the anomalous left coronary artery is typically normal, with normal left anterior descending and circumflex coronary arteries. The origin of the right coronary artery is also normal; however, this vessel is usually enlarged and tortuous. With early diagnosis, the prognosis of ALCAPA is excellent after surgical repair.

ALCAPA affects 1 in 300,000 live US births, representing approximately 0.24-0.46% of congenital heart defects.
 No data are available to suggest variance in the frequency of this condition in different countries or between social, economic, or ethnic groups.


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