Anomalous Left Coronary Artery (ALCAPA)

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What is ALCAPA?

Anomalous left coronary artery (ALCAPA) is a congenital (present at birth) heart defect in which the left coronary artery forms abnormally. Instead of connecting to the aorta, as it should, it connects to the pulmonary artery.

In a normal heart, the left coronary artery starts in the aorta, the major blood vessel that takes oxygen-rich blood from the heart to the rest of the body.

In children with ALCAPA, the left coronary artery starts at the pulmonary artery and carries oxygen-poor blood to the left side of the heart. When the heart doesn’t get enough oxygen, it can weaken or die. This is what happens during a heart attack. The damaged heart muscle cannot pump effectively, leading to heart failure.

This very rare syndrome causes dangerously poor cardiac function in infancy. Surgery is needed to correct the defect. Without surgery, most babies don’t survive their first year, but with timely surgery, most babies do well and live a normal life.

Care for ALCAPA

At Boston Children’s Hospital, our Heart Center provides expert screening, evaluation and care for children with ALCAPA. Screening begins soon after your child’s first cardiac surgery and continues as your child grows.

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