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Transposition of the great arteries is a rare congenital heart defect involving an abnormal arrangement of the aorta and the pulmonary arteries (the two major arteries carrying blood away from the heart). The aorta rises from the right ventricle instead of the left, and the pulmonary artery rises from the left ventricle instead of the right. This condition is recognized early, often at birth. Transposition of the great arteries should be treated promptly to avoid complications. Even with treatment, patients diagnosed with this condition remain at risk for complications throughout adulthood. Therefore, regular lifelong care by a cardiologist who specializes in congenital heart conditions is important.
Transposition of the great arteries occurs during fetal development. In most cases, the cause is unknown.
Risk factors may include:
If your family has a history of congenital heart defects, you may want to consider consulting with a genetic counselor, who may recommend genetic testing.
The primary symptom is cyanosis (bluish coloration of the skin due to low oxygen levels in the blood). Infants who display this symptom are sometimes referred to as blue babies. In addition to cyanosis (bluish coloration of the skin due to low oxygen levels in the blood), symptoms include shortness of breath, lack of appetite and failure to gain weight.
Ohio State’s Wexner Medical Center offers a team approach to CHD, which means each patient is evaluated by an Adult Congenital Heart Disease (ACHD) specialist, and when appropriate, a cardiac surgeon, an imaging specialist and an interventional cardiologist with training in CHD. This integrated approach means that each patient’s disease is treated individually, with that particular patient’s needs and physical condition, in mind.
Anyone who has ever been diagnosed with and/or treated for transposition of the great arteries should have lifelong care from a cardiologist who specializes in congenital heart defects. The Ohio State University Wexner Medical Center provides this care from birth to adulthood. We partner with Nationwide Children’s Hospital to provide the resources necessary for the care of adult congenital cardiac patients through the Columbus Ohio Adult Congenital Heart (COACH) Disease Program.
The COACH program focuses on:
Due to improvements in technology, some congenital heart defects can be detected before a baby is born. In other cases, severe heart defects are detected shortly after birth.
Tests used to diagnose transposition of the great arteries include:
Electrocardiogram (EKG/ECG) – A test that records the electrical activity of the heart.
Echocardiogram (also called echo) – A test that uses sound waves to assess the function and structure of the heart muscle and valves.
Chest X-ray – A radiograph or picture of the heart and lungs including blood vessels, ribs and bones of the spine.
Cardiac MRI (cardiac magnetic resonance imaging or CMR) – A noninvasive, sophisticated imaging procedure that uses large magnets and a computer to produce detailed images of the structure and function of the heart while it is beating.
Cardiac catheterization – A procedure to check for problems in coronary arteries. A long, thin tube (catheter) is inserted into an artery or vein in the groin, arm or neck, and then threaded to the heart. The physician injects a contrast solution into the artery and takes X-rays to check for blockage and other abnormalities.
Babies born with transposition of the great arteries must have surgery to correct the heart defect.
If surgery is not immediately possible, medications can help to manage the condition until surgery occurs. Prostaglandins, a medication to dilate the blood vessels, may be recommended before a baby is ready for surgery.
A cardiac catheterization procedure can enlarge the connection between the heart’s upper chambers and improve oxygen delivery throughout the body until surgery is possible.
Surgical procedures to treat transposition of the great arteries include:
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