Congenital Heart Disease

Transposition of the Great Arteries

Signs and Symptoms
Diagnosis
Treatment

Treatment

Currently, permanent treatment requires a surgical procedure to switch the arteries to their proper places. This operation, called an arterial switch operation, is done within the first few weeks of life. It is an open-heart procedure that requires a temporary stopping of the baby's heart while a heart-lung machine handles respiration and blood circulation. Any abnormal holes between the ventricles or atria also are closed. As part of the procedure the coronary arteries -- the arteries that supply blood to the heart -- have to be taken off their normal position on the aorta and transplanted into the new "aorta" that now carries oxygenated blood from the left ventricle around the body. Rarely, this may be lead to problems that require further procedures.

Most adults with transposition of the great arteries have been treated with a different operation that created an atrial switch and corrected the path of blood flow. These operations carry the names of the surgeons who first described them, the Senning or the Mustard procedure. The most common complications to arise from these types of operations include heart failure, abnormal heart rhythms and leaks in the "baffle" that diverts the blood flow along the right path. Nevertheless, many adults lead relatively normal lives and women have had successful pregnancies.

Patients who have this condition need follow-up care by a cardiologist who specializes in congenital heart disease for the rest of their lives to monitor any subsequent complications. A woman who has had surgery for transposition of the great arteries should have a thorough evaluation before contemplating pregnancy. All patients with transposition of the great vessels should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis.

Reviewed by health care specialists at UCSF Medical Center.
Last updated February 6, 2008