Congenitally Corrected Transposition of the Great Arteries

Surgery

The standard surgical approach is to treat each condition associated with corrected transposition separately. This approach, however, does not solve the problem of the right ventricle having to do the work that is normally done by a larger, stronger left ventricle — pushing oxygen-rich blood throughout the body. Over time, this extra burden on the right ventricle results in gradual deterioration of function of the right ventricle and can lead to congestive heart failure.

Valve regurgitation

Surgery to replace the tricuspid valve is considered before the pumping function of the right ventricle is impaired. If the ventricular pumping function is reduced, contraction may not return to normal even after a perfect valve replacement. The valve will need to be replaced before severe symptoms develop since symptoms occur late in the course of decreasing heart function. If the function of the right ventricle has become severely depressed, valve replacement surgery may no longer be possible or recommended. Heart transplantation may be considered.

Obstruction at or beneath the pulmonary valve (pulmonary stenosis)

In some cases, pulmonary or subpulmonary stenosis may occur. A conduit (tube) may be placed between the ventricle and pulmonary artery bypassing the obstruction. In other cases, the obstruction can be surgically removed or, if the obstruction is caused by a narrowed pulmonary valve, replacement or enlargement of the valve is considered.

Ventricular septal defect (VSD)

A VSD may occur in patients with corrected transposition. It is usually surgically closed in childhood, occasionally in adulthood.

Double Switch Operation

Today, more patients are being offered a "double switch" operation. At the time the surgeon fixes a defect such as a ventricular septal defect, he or she also reroutes the great arteries so that the ventricles are pumping blood in the direction of a normal heart. This is clearly a much more formidable surgical undertaking, with somewhat greater risk, but if successful, should result in better long-term health of ventricles, especially the over-worked right ventricle.

The best surgical approach to use today in patients with corrected transposition remains controversial, but as surgeons gain greater experience with the "double switch" operation and surgical risk decreases, there is increasing enthusiasm for this approach.