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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.
Fenestration is still widely used in right heart bypass operations. Our study was conducted to assess its need in the most recent modification, the completion of a total cavopulmonary connection with an extracardiac tube.
The extracardiac approach was introduced at our institution in January, 1999. Since June of 2000, no patient had a fenestration. If more than 1 risk factor amongst ventricular function being more than moderately impaired, atrioventricular valvar regurgitation more than moderate, mean pulmonary arterial pressure more than 15 millimetres of mercury, mean atrial pressure higher than 12 millimetres of mercury, pulmonary arterial distortion, or other than sinus rhythm was present preoperatively, the patient was considered a “high risk” candidate. Postoperatively elevated pulmonary arterial pressure higher than 16 millimetres of mercury, prolonged effusions and requirement for drainage longer than 7 days, and death were considered endpoints in the statistical analysis.
Our study group included 84 patients who underwent surgery up to August, 2004. A previous bidirectional cavopulmonary anastomosis had been accomplished in 73 patients at a mean age of 27.01 plus or minus 32.60 months, with a median of 11.5 months, without creating an additional source of flow of blood to the lungs.
At the time of the total cavopulmonary connection, the mean age was 66.4 plus or minus 60.1 months, with a median of 37.1 months, and a range from 17.3 to 251.2 months, with 50 patients being younger than 48 months.
We deemed 16 patients to be at “high risk”. These patients were older at the time of bidirectional cavopulmonary anstomosis (p smaller than 0.016), at the time of completion (p smaller than 0.019), and also differed in size at time of completion (p smaller than 0.020). They required a longer time on cardiopulmonary bypass (p smaller than 0.015), and reached higher early postoperative pulmonary arterial pressures after completion (p smaller than 0.025). There were no differences between groups of patients having up to 1 or more risk factors in regard to need for intubation (p smaller than 0.511), pulmonary arterial pressures after extubation (p smaller than 0.817), and duration of chest drainage (p smaller than 0.650). Three patients died, one in the group deemed at high risk. There was no death in the last 38 patients.
We conclude that a total cavopulmonary connection with an extracardiac tube can be performed without fenestration, even if the patients are deemed to be at increased risk. Early staging of patients with functionally univentricular physiology might be one of the keys for these findings.
Background: Creation of an extracardiac cavopulmonary connection has been proposed as a superior alternative to the lateral intracardiac tunnel for the completion of total cavopulmonary connection. Methods and results: We made a retrospective review of our experience with 125 patients undergoing a total cavopulmonary connection between June 1994 and January 2003. Our experience with the extracardiac connection for completion began in 1999. Since 1994, we have constructed an intracardiac tunnel in 50 patients, and an extracardiac connection in 75. Of the total number, 83 had undergone an earlier partial cavopulmonary connection. Additional intracardiac procedures were performed in 43 patients at time of completion, in 25 of those undergoing extracardiac completion, and in 18 of the patients having an intracardiac procedure. The mean size of the tube used for completion was 19 mm. The mean cross-clamp time for placement of the intracardiac tunnel was 77 min, with a median of 80.5 min, and a mean cardiopulmonary bypass time of 139 min, with a median of 131 min. For construction of the extracardiac connection, a mean cross-clamp time in 24 of the 75 patients was 54 min, with a median of 54 min. Mean cardiopulmonary bypass time for all the patients with an extracardiac connection was 100 min, with a median of 88 min. Reoperations were needed in 10 patients, 6 having intracardiac and 4 extracardiac procedures. Of these, 5 were early and 5 late, including one take down. None of the patients died after these interventions. Taken overall, 8 patients died, with 5 early deaths. In the multivariable analysis, cardiopulmonary bypass time of more than 120 min, atrioventricular valvar replacement, and banding of the pulmonary trunk prior to the total cavopulmonary connection, all reached statistical significance for early death, whereas only heterotaxy syndrome remained as the sole risk factor for late death. There was no significant difference in survival between the modifications used. Discussion: Whereas we could not identify any clinical superiority for the extracardiac approach in the short-term, the concept of extracardiac completion has helped to simplify the overall procedure. Longer follow-up will be required to elucidate any potential advantages.
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