Anomalous Coronary Artery Repair

Also known as: ALCAPA repair.

What is anomalous coronary artery repair?

Anomalous coronary artery repair is a surgery performed to repair a heart problem known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA).  In this defect, the left coronary artery, which supplies oxygen-rich blood to the heart muscle, is abnormal in form; the left coronary artery arises from the pulmonary artery instead of from the aorta. It is a very rare defect that is present at birth and affects boys and girls equally, but it may not be diagnosed till later in life.

What happens during the procedure?

Anomalous coronary artery repair usually only requires one surgery. The procedure is an open heart surgery and involves disconnecting the anomalous left coronary artery from the pulmonary artery, repairing the damage and connecting the artery to the aorta to restore normal blood flow.  

Is any special preparation needed?

Anomalous coronary artery repair is performed shortly after diagnosis. If the child is very ill, medications may be needed before surgery can occur.

What are the risk factors?

As with all surgeries, there are potential complications. However, anomalous coronary artery repair has become increasingly effective in recent years. The risks related to not surgically treating ALCAPA far outweigh the risks related to the repair. Recovery from the surgical repair of this defect may be difficult and is dependent on the degree of existing heart injury at the time of diagnosis and repair. Poor heart function and irregular heartbeat may occur. Gradual improvement can be expected in most cases.

Anomalous coronary artery repair at Nicklaus Children’s Hospital: Nicklaus Children’s Hospital pediatric cardiologists are well trained in cutting edge procedures for anomalous coronary artery repair.


Reviewed by: Bhavi Patel, DO

This page was last updated on: 6/12/2018 2:57:10 PM

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Jason Katz, MD of Nicklaus Children's Hospital is a pediatric cardiologist with The Heart Program and the Director of the Pediatric Cardiac Fellowship Program.