Anomalous Left Coronary Artery From the Pulmonary Artery

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a congenital heart defect in which the left coronary artery arises (branches) abnormally from the pulmonary artery instead of the aorta.

About Anomalous Left Coronary Artery From the Pulmonary Artery

Normally, both the left and right coronary arteries arise (branch) from the aorta (the major blood vessel that takes blood from the heart to the rest of the body) and carry oxygen-rich blood to the muscle of the heart. In children anomalous left coronary artery from the pulmonary artery (ALCAPA), the left coronary artery arises from the pulmonary artery and carries deoxygenated blood to the left side of the heart. As a result, the left-sided heart muscle becomes damaged due to the lack of oxygen and nutrients over time.

Symptoms of Anomalous Left Coronary Artery From the Pulmonary Artery

Symptoms of ALCAPA usually occur within the first few months of life.

Symptoms of ALCAPA may include:

• Chest pain
• Excessive crying, particularly during feeds
• Irregular heartbeats or palpitations
• More sleepiness than normal
• Sweating and skins that appears pale when crying or feeding

Diagnosing Anomalous Left Coronary Artery From the Pulmonary Artery

During physical examinations, your child’s doctor listens to your child’s heart and lungs and may detect a heart murmur, which are extra sounds heard throughout the cardiac cycle due to increased blood flow. If your pediatrician suspects increased blood flow, a recommendation to see a pediatric cardiologist may be made.

Tests performed when diagnosing ALCAPA may include:

• Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make the heart’s structure more visible on an X-ray.
• Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies. A cardiac MRI or CT scan can help determine exactly where the coronary arteries arise.
• Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs.
• Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood.
• Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.

Treating Anomalous Left Coronary Artery From the Pulmonary Artery

Treatment options for children with ALCAPA vary based on how much of the heart muscle is damaged. Some children with ALCAPA have mild heart muscle damage and can easily tolerate an operation during which the coronary artery is moved. In children with severe heart muscle damage, other options, such as mechanical circulatory support and transplantation, must be considered.

Treatment options for ALCAPA may include:

• Surgery: A pediatric heart surgeon must perform surgery to ensure oxygen- and nutrient-rich blood flows through the left coronary to the heart muscle. The type of surgery is dependent upon the location at which the coronary artery originates and the damage sustained to the muscle of the heart.

Care Team Approach

The Texas Center for Pediatric and Congenital Heart Disease, a clinical partnership between Dell Children’s Medical Center and UT Health Austin, takes a multidisciplinary approach to your child’s care. This means your child and your family will benefit from the expertise of multiple specialists across a variety of disciplines. Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way. We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your child’s care with referring physicians and other partners in the community to ensure that we are providing comprehensive, whole-person care.

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