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Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern.

The incidence of trivial or mild AI after the ASO is considerable and a progression over time is evident. However, severe AI and the need for AVR are rare. Patients with VSD or Taussig-Bing anomaly, and those with left ventricular outflow tract obstruction exhibit a higher risk of developing significant aortic insufficiency. Particularly patients who have developed an AI at 1 year after the ASO need to be under close observation.

Background-Survival and functional status of patients with transposition of the great arteries treated by atrial switch are reported to be reasonably good within the first 15 postoperative years. However, in some patients, the function of the systemic right ventricle deteriorates, leading to significant morbidity or even to late mortality. This study seeks to identify risk factors for late death. Methods and Results-Records of 329 patients after the Senning operation and 88 after the Mustard operation at a single center were retrospectively reviewed for demographic, anatomic, and echocardiographic predictors and outcomes. Mean follow-up interval was 19.1Ϯ6.5 years and was 95% complete. Survival 25 years after the Mustard procedure was 75.9Ϯ4.8% and after the Senning procedure was 90.9Ϯ2.3% (Pϭ0.002). Mustard patients died more often of arrhythmia than Senning patients (PϽ0.001) and needed more baffle-related reoperations (PϽ0.0001). Ventricular septal defect closure at the time of the atrial switch operation (hazard rateϭ2.3; 95% confidence interval, 1.1 to 4.7; Pϭ0.025) and the Mustard operation (hazard rateϭ2.0; 95% confidence interval, 1.01 to 3.8; Pϭ0.045) emerged as independent risk factors for late mortality in multivariate analysis. At follow-up, 85.8% of the patients led a normal life with full-time work, and 11.8% were able to do part-time work. Only 2.4% experienced noticeable limitation of activities. Conclusions-Our patient data reveal satisfactory results at long term in this historic collective. Patients who had undergone ventricular septal defect closure at the time of the atrial switch operation and those who had undergone a Mustard operation are at higher risk for late death. Close follow-up, especially of these subgroups, is warranted.

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Repair, as opposed to replacement, is feasible in the vast majority of patients presenting with Ebstein's anomaly with a low early mortality rate. Outcome, in terms of survival and freedom from re-operation in the long term is determined by the clinical state at the time of surgery. Therefore, timely operation is warranted before significant cardiomegaly develops and functional status deteriorates.

Homografts, bovine jugular vein conduits and porcine-valved Dacron conduits exhibit equal durability after implantation in patients below 1 year of age independent of their size. Nonetheless, moderate conduit stenosis or insufficiency develops earlier in patients with a Contegra conduit. Conduit placement in the neonatal period and implantation in a heterotopic position shortens the durability.