Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA)

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a congenital (present at birth) heart defect in which the left coronary artery arises abnormally from the pulmonary artery. Normally, the left and right coronary arteries arise from the aorta and supply blood with oxygen to the heart.

In children with ALCAPA, the left coronary artery arises from the pulmonary artery and carries blood without oxygen to the left side of the heart. When the heart doesn’t get enough oxygen, the heart muscle can weaken or die, similar to having a heart attack. The damaged heart muscle cannot pump effectively, leading to cardiomyopathy and heart failure.

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

Symptoms of anomalous left coronary artery from the pulmonary artery (ALCAPA) in an infant include:

• Crying or sweating during feeding
• Poor feeding
• Rapid breathing
• Sweating
• Symptoms of pain or distress (these are often mistaken for signs of colic)

Symptoms usually appear within the first few months of a baby's life.

If a doctor thinks your child has ALCAPA, he or she may order tests such as:

• Chest X-ray
• Electrocardiogram (ECK or EKG)
• Echocardiogram (cardiac ultrasound)
• Cardiac MRI
• Cardiac catheterization

Surgery is needed to treat anomalous left coronary artery from the pulmonary artery (ALCAPA). Options for repair include detaching the anomalous left coronary artery from the pulmonary artery and moving it over to the aorta directly (translocation) or creating a natural tunnel from its abnormal location to the aorta (Takeuchi repair). If the mitral valve is leaking, it can be repaired at the same time. In some cases, the heart is severely damaged and may require mechanical circulatory/ventricular assist device support, and a heart transplant may be needed.

With timely therapy, most children do very well after surgery and can expect to live a normal life. However, routine and life-long follow-up with a heart-failure specialist will be necessary to ensure recovery of the heart muscle and good function of the mitral valve. Children born with ALCAPA are at an increased risk for heart-rhythm problems later in life.

What medications are used to treat ALCAPA?

Some children need medications prior to surgery in order to build up the strength necessary for the procedure. These medications may include:

• Diuretics: “Water pills”
• Inotropic agents: Medications that make the heart muscle pump harder
• Beta-blockers, ACE inhibitors: Medications that lower the work load on the heart

In addition, some of these medications may be needed after surgery.

At Boston Children’s Hospital, our Benderson Family 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.