Congenitally Corrected Transposition of the Great Arteries

Treatment

Treatment depends on how the main pumping chamber (in this case the right ventricle) and its valve tolerate the strain of supporting the high-pressure circulation. The weaker right ventricle is very vulnerable to failure and this may be easily overlooked. Treatment will also depend on whether associated defects are present or if surgery was performed perviously.

Observation and monitoring

For people without symptoms or heart rhythm disturbances, regular observation and monitoring by an adult congenital cardiologist are appropriate. A typical evaluation would include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test. A Holter monitor or event recorder may also be used to check for irregular heart rhythms.

Complete heart block (an abnormally slow rhythm) is treated with the insertion of a pacemaker. Heart blockage is not uncommon in patients with corrected transposition. Careful monitoring of heart rhythm is required regularly in patients with congenitally corrected tranposition of the great arteries.

Medication

Patients with corrected transposition may be treated with medications to improve the pump function and reduce the effects of a leaky valve.

Surgery

Surgery is often required in patients with congenitally corrected transposition of the great arteries. When associated congenital heart lesions are present, surgery is often done at a young age. Valve replacement may be required in patients without associated heart defects.

Pregnancy and family planning

Pregnancy plans should be discussed with your doctor before pregnancy occurs. Close monitoring by your adult congenital cardiologist during pregnancy, labor, delivery and for a period after the pregnancy is recommended.