In a healthy heart, both coronary arteries arise from the aorta. In one type of heart defect, the left coronary artery comes off the pulmonary artery instead of the aorta. This condition is called anomalous left coronary artery from the pulmonary artery (ALCAPA). Similarly, it is possible for the right coronary artery to arise from the pulmonary artery instead of the aorta.

This is a type of congenital (present from birth) heart disease. Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, surgeons are experienced in treating patients with ALCAPA. The surgery moves the origin of the left coronary artery from the pulmonary artery to the aorta, from which it should originate.

Following ALCAPA reimplantation surgery, most children have a good quality of life and can live without limitations on sports or other activities.

ALCAPA Symptoms

Patients with ALCAPA may show symptoms in infancy. Signs and symptoms may include:

• Sweating with feeds
• Not moving around
• Fast breathing
• Not eating enough
• Skin that looks mottled, like a lacy red or blue pattern
• Not gaining enough weight
• Shortness of breath, particularly after feeds

Older patients may:

• Have fatigue
• Quickly tire with exercise
• Fast breathing
• Abdominal pain
• Cold hands and feet
• Nausea

ALCAPA is a very serious form of congenital heart disease. Without prompt diagnosis and treatment, the heart can suffer damage from a lack of oxygen.

Diagnosing ALCAPA

In infants, low energy, poor feeding/nursing and difficulty breathing may signal an issue with the heart, such as ALCAPA.

The following tests may provide more details to find the diagnosis:

• Cardiac MRI (magnetic resonance imaging): A test that uses radio waves, magnets and a computer to form three-dimensional pictures of the heart. These pictures can show structural issues, such as ALCAPA.
• Chest X-ray: This shows pictures of the heart and lungs, and can show heart issues, extra blood flow or fluid in the lungs.
• Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show the structure and function of the heart including where the coronary arteries originate. Norton Children’s Heart Institute has 28 tele-echo locations in Kentucky and Southern Indiana so many patients don’t have to travel far from home for an echocardiogram.
• Electrocardiogram (ECG or EKG): This is a test that checks the heart’s electrical activity to show damage or irregular rhythms, suggesting an issue with the heart. This may be an important study in diagnosing ALCAPA since the heart can be damaged from the left coronary artery being supplied with oxygen poor blood for a period of time.
• Heart catheterization: This invasive procedure studies the structure, function, and provides direct pressure measurements of the heart chambers. Catheterization can provide the visual angiogram images to also diagnose ALCAPA.

How Does ALCAPA Reimplantation Occur?

This complex surgery requires experience, skill and training. The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute are the leading providers of pediatric heart care in Louisville and Southern Indiana.

Our pediatric cardiothoracic team will review your child’s case and choose a reimplantation surgery strategy tailored to your child.

ALCAPA repair includes:

• Cardiopulmonary bypass
• Separating the anomalous left coronary artery from the pulmonary artery
• Lengthening the coronary artery if necessary, and then stitching the anomalous left coronary artery into the aorta
• Creating a hole from the aorta to the anomalous left coronary artery
• Repairing the pulmonary artery
• Depending on the exact anatomy of the patient’s lesion, surgeons may need to address the anomaly
• Creating a link between the large artery that carries blood to the left arm and upper body (left subclavian artery) and the left coronary artery, allowing oxygen-rich blood from the subclavian artery to reach the left coronary artery and the heart

Norton Children’s Heart Institute has a network of remote diagnostic and treatment services in Kentucky and Southern Indiana.

ALCAPA Reimplantation Surgery Complications and After Care

Many kids will be able to stop taking medicines weeks or months after surgery. Some children will need to continue taking medicines after surgery, including:

• Beta blockers and ACE inhibitors: Medications that help the heart not work as hard
• Inotropic agents: Medications that make the heart pump harder
• Water pills: Medications that help the body get rid of salt and water

Children with ALCAPA will need lifelong care from a cardiologist. Children born with ALCAPA are at an increased risk for heart rhythm issues later in life.

Why Choose Norton Children’s Heart Institute

• Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
• The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
• The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
• More than 17,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
• Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
• The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.