Search

Up until 25 years ago, the mortality rate of untreated transposition of the great arteries was about 90% during the first year of life. The Rashkind balloon atrio-septostomy [5], introduced in 1966, increased effective flow in both circulations and, therefore, diminished cyanosis. This decrease in cyanosis was associated with a significant decrease in the mortality rate. However, the blood exchange at atrial level does not keep up with the increasing flow dimension of children during growth. The Mustard operation, which establishes an atrial switch [4] was developed, and proved to have a low operative mortality rate; it also allows a total separation of pulmonary and systemic circulation. The atrial switch remained, therefore, the operation of choice in the following two decades. Initial enthusiasm for this operation, however, subsided when complications such as obstruction of the venous pathways, arrhythmias, and right-ventricular dysfunction caused a staggering increase in late mortality. The development of the arterial switch operation [1] suggested a new surgical treatment for patients with transposition of the great arteries without the previously stated severe complications.

Twenty-two patients with a ventricular septal defect and aortic incompetence underwent surgical repair. Mean age was 9.6 years (range, 9 to 15 years). Mean follow-up was 32.4 ± 15.8 months. The ventricular septal defect was perimembranous in 19 patients and doubly committed or juxtaarterial in 3. Most showed only a small left-to-right shunt. Mean diameter was 11.9 ± 4.8 mm. Aortic incompetence was mild in 6 patients, moderate in 4, and severe in 12. In 16 patients closure of the ventricular septal defect and aortic valvoplasty were both performed through the aortic root. There were no deaths. All patients retained normal sinus rhythm. No complete heart block was found. In 3 patients secondary aortic valve replacement was required for severe incompetence; in 1 the cause was bacterial endocarditis, in another technical failure, and in a third progressive incompetence over an 8-month period. All patients showed substantial clinical improvement, marked decrease or disappearance of the valvar incompetence, diminution of the left ventricular end-diastolic diameter, and decreased cardiothoracic ratio. We conclude that primary repair is the operation of choice for this combination of lesions. This can avoid or delay considerably valvar replacement. The left-sided approach proves safe and is our preferred technique.