Interrupted Aortic Arch

An interrupted aortic arch is a congenital heart defect in which the aorta (the major blood vessel that takes blood from the heart to the rest of the body) is interrupted, causing the aorta to lack a continuous structure.

About Interrupted Aortic Arch

The aorta is the large blood vessel that arises (branches) from the left ventricle of the heart and carries oxygen-rich blood from the heart to all areas of the body. The aorta starts at the heart and arches up and curves down in a shape that resembles a candy cane. As the aorta begins to curve, there are three major arteries that arise from it to supply the head and arms with blood. These three arteries include the innominate artery, the left carotid artery, and the left subclavian artery. The remainder of the aorta supplies blood to the body and legs. When a diagnosis of an interrupted aortic arch is made, it means that there is a disruption (missing portion) of the aorta. Interruptions are classified based on the location of where the interruption occurs.

The three different types of interruption are:

• Type A (30-40%): The interruption occurs just beyond the left subclavian artery (supplies blood to upper thorax, below the clavicle).
• Type B (50-60%): The interruption occurs between the left carotid artery (supplies blood to head and neck) and the left subclavian artery (supplies blood to upper thorax, below the clavicle).
• Type C (4-5%): The interruption occurs between the innominate artery (supplies blood to right arm, head, and neck) and the left carotid artery (supplies blood to head and neck).

When patients are diagnosed with an interrupted aortic arch, they almost always have a hole between the two pumping chambers of their heart (ventricular septal defect), which allows the mixing of oxygen-rich and oxygen-poor blood and causes the blood to not be as oxygenated as it should be. Some patients with an interrupted aortic arch also experience other medical conditions, such as DiGeorge Syndrome (also referred to as 22Q11 deletion syndrome), which is a chromosomal disorder and can result in the poor development of several body systems. If the diagnosis of an interrupted aortic arch is made, chromosome analysis should be performed.

Symptoms of Interrupted Aortic Arch

Symptoms of interrupted aortic arch typically develop in the first 1 to 2 days of life and require urgent intervention and care.

Symptoms of interrupted aortic arch may include:

• Fast breathing
• Lower oxygen saturations in the legs, causing legs to appear blue or dusky in color (cyanosis)
• Poor feeding
• Sleepiness (more than normal) or lethargy
• Weak or absent pulses in the legs

Diagnosing Interrupted Aortic Arch

Interrupted aortic arch is often diagnosed by ultrasound before the baby is born. 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 in the right atrium, a recommendation to see a pediatric cardiologist may be made. Your doctor may also refer you to a pediatric cardiologist if your child is breathing fast or has weak pulses in the lower extremities, poor exercise tolerance (for babies, this means poor feeding), or an enlarged liver.

Tests performed when diagnosing interrupted aortic arch may include:

• 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.
• 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 Interrupted Aortic Arch

Treatment of interrupted aortic arch requires open-heart surgery to repair the interruption. Typically, a medication referred to as prostaglandin is prescribed as soon as a diagnosis is made. This medication keeps the ductus arteriosus (a blood vessel that connects two major arteries—the aorta and pulmonary artery) open, which provides a different route for the blood to be pumped out to the body while the baby is awaiting surgery.

During surgery, the two ends of the aorta on either side of the interruption will be connected to establish a continuous pathway for blood to flow from the left ventricle to the rest of the body. If the baby also has a ventricular septal defect, this is typically closed by placing a patch over the hole during the same operation.

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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