Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.

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Objective: To describe a 12 year experience with staged surgical management of the hypoplastic left heart syndrome (HLHS) and to identify the factors that influenced outcome. Methods: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure (median age 4 days, range 0-217 days). Subsequently 203 patients underwent a bidirectional Glenn procedure (stage II) and 81 patients underwent a modified Fontan procedure (stage III). Follow up was complete (median interval 3.7 years, range 32 days to 11.3 years).Results: Early mortality after the Norwood procedure was 29% (n = 95); this decreased from 46% (first year) to 16% (last year; p , 0.05). Between stages, 49 patients died, 27 before stage II and 22 between stages II and III. There were one early and three late deaths after stage III. Actuarial survival (SEM) was 58% (3%) at one year and 50% (3%) at five and 10 years. On multivariable analysis, five factors influenced early mortality after the Norwood procedure (p , 0.05). Pulmonary blood flow supplied by a right ventricle to pulmonary artery (RV-PA) conduit, arch reconstruction with pulmonary homograft patch, and increased operative weight improved early mortality. Increased periods of cardiopulmonary bypass and deep hypothermic circulatory arrest increased early mortality. Similar factors also influenced actuarial survival after the Norwood procedure. Conclusion: This study identified an improvement in outcome after staged surgical management of HLHS, which was primarily attributable to changes in surgical technique. The RV-PA conduit, in particular, was associated with a notable and independent improvement in early and actuarial survival.

Anatomic repair of congenitally corrected transposition of the great arteries can be carried out with low early mortality. Excellent functional status can be achieved, with good midterm survival. Continued surveillance is necessary for patents with valved conduits and to determine the longer-term function of the aortic valve and the morphologically left ventricle in the systemic circulation.

There is significant morbidity after anatomic repair of ccTGA, which is higher in the DS than the RS group. Nevertheless, the majority of patients are free of heart failure at 10 years, including high-risk patients in severe heart failure before repair. Aortic annuloplasty may reduce risk of late aortic insufficiency.

Background-Some patients with a morphological right ventricle (mRV) in the systemic circulation require early intervention because of progressive systemic ventricular dysfunction or atrioventricular valve regurgitation. They may be eligible for anatomic repair (correction of atrioventricular and ventriculoarterial discordance) but require prior training of the morphological left ventricle (mLV). Methods and Results-Forty-one patients with congenitally corrected transposition of the great arteries or a previous atrial switch procedure embarked on a protocol of pulmonary artery (PA) banding with a view to anatomic repair. All had an mRV in the systemic circulation and a subpulmonary mLV that was not conditioned by either volume or pressure load. Two patients were not banded, and 39 were followed up for a median of 4.3 years (range, 25 days to 12.6 years). Sixteen patients achieved anatomic repair, with 3 in the early stages of the training protocol. After 2 years, 12 patients were not suitable for anatomic repair and persisted with palliative banding; 8 were functionally improved; and 4 died, underwent transplantation, or required debanding. PA banding improved functional class but did not improve tricuspid regurgitation in the long term for patients not achieving anatomic repair. mLV function was a critical determinant of survival with a PA band as well as survival after anatomic repair. Patients Ͼ16 years were unlikely to achieve anatomic repair. Conclusion-PA banding is a safe and effective method of training the mLV before anatomic repair. It is also an effective palliative procedure for those who do not attain this goal.

Objective: High concentrations of potassium and lactate in irradiated red cells transfused during cardiopulmonary bypass may have detrimental effects on infants and neonates undergoing cardiac surgery. The effects of receiving washed and unwashed irradiated red cells from the cardiopulmonary circuit on serum potassium and lactate concentrations were compared. Methods: The study population included neonates and infants undergoing heart surgery for complex congenital heart disease. A control group (n = 11) received unwashed irradiated red cells and the study group (n = 11) received irradiated red cells washed in a cell saver (Dideco Electa) using 900 ml of 0.9% saline prior to pump priming. Potassium and lactate concentrations were compared before, during and after bypass. Results: Washing irradiated red cells reduced donor blood [potassium] from > 20 to 0.8 AE 0.1 mmol/l, and [lactate] from 13.7 AE 0.5 to 5.0 AE 0.3 mmol/l ( p < 0.001). The resulting prime had significantly lower [potassium] and [lactate] than the unwashed group (potassium 2.6 AE 0.1 vs 8.1 AE 0.4 mmol/l, p < 0.001; lactate 2.6 AE 0.2 vs 4.6 AE 0.3 mmol/l, p < 0.001). Peak [potassium] in the unwashed group occurred 3 minutes after going on bypass (4.9 AE 0.3 mmol/l) and during rewarming (4.9 AE 0.4 mmol/l). These were significantly higher than the washed group (3.1 AE 0.1, p < 0.001 and 3.0 AE 0.1 mmol/l, p < 0.001). The [potassium] was greater than 6.0 mmol/l for 4 out of these 11 unwashed patients compared with none of the washed group. Immediately postbypass the washed group had significantly lower serum [potassium] (3.2 AE 0.1 vs 4.2 AE 0.2 mmol/l, p = 0.002). There was no significant difference in [lactate] between groups during and after cardiopulmonary bypass. Conclusions: The washing of irradiated red cells reduces potassium and lactate loads and prevents hyperkalaemia during cardiopulmonary bypass. The washing of irradiated red cells should be considered in neonates and infants undergoing cardiac surgery for complex congenital heart disease. #

By using a strategy of unifocalization, intrapericardial pulmonary artery reconstruction, and right ventricle-pulmonary artery conduit, excellent long-term survival can be achieved in this group of patients even in the absence of native intrapericardial pulmonary arteries.