Intermediate-term results of the Senning or Mustard procedures combined with the Rastelli operation for patients with discordant atrioventricular connections associated with discordant ventriculoarterial connections or double outlet right ventricle

BACKGROUND: In patients with discordant atrioventricular and ventriculoarterial connections, anatomic repair restores the morphologically left ventricle to its role in supporting the systemic circulation. In this study, we have evaluated the outcomes in the intermediate term for this complex surgical procedure. METHODS: Between December 1984 and October 2003, 4 patients underwent an atrial switch operation concomitantly with a Rastelli operation, and 2 patients underwent an atrial switch operation and a patch-plasty of the pulmonary outflow tract for anatomic repair at a mean age of 3.3 plus or minus 2.1 years. All patients had intracardiac rerouting, connecting the morphologically left ventricle to the aorta. RESULTS: There were no hospital deaths. In 5 patients, reoperation was needed, either for baffle complications, exchange of the conduit, repair of a residual ventricular septal defect, or relief of obstruction within the left ventricular outflow tract. Death occurred in 1 patient, from cardiac failure 6 months after correction. Mean follow-up time was 6.5 plus or minus 6.4 years, with a range from 6 months to 17 years. At follow-up, 1 patient presented with moderate tricuspid insufficiency, and 1 patient with mild obstruction of the pulmonary venous pathway. The remaining 3 patients showed good left and right ventricular function, and no, or mild tricuspid and mitral insufficiency. CONCLUSIONS: Anatomic repair can be performed with low hospital mortality. Restoration of the morphologically left ventricle into the systemic circulation in patients with discordant atrioventricular and ventriculoarterial connections is a demanding approach, associated with various reoperations over time. Despite this, the approach seems to be an appropriate solution for selected patients, since the majority of the patients show good left and right ventricular function, and no, or mild tricuspid and mitral insufficiency up to 17 years after correction.