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Background: Information on surgical management and outcome in patients who develop symptomatic right ventricular failure after prior Mustard or Senning operations is limited., Methods and Results: From March 1987 to March 1991, 10 patients 3.6-23.5 years old (median, 7.0 years) with transposition of the great arteries and prior Mustard (six patients) or Senning (four patients) repairs (performed at ages 2 months to 5 years; median, 6 months) underwent surgical intervention for symptomatic right ventricular failure. In five of 10 patients, anatomic correction with either an arterial switch operation (three patients) or a pulmonary artery-to-aorta anastomosis and right ventricle-to-pulmonary artery conduit (two patients) was performed. Before anatomic correction in these five patients, four of five patients had a pulmonary artery band to prepare the left ventricle. The interval between preparation and correction ranged from 8 days to 12 months (median, 2 months). One patient died after an arterial switch operation. In the remaining five patients, coexisting left ventricular dysfunction precluded anatomic correction; all five patients survived cardiac transplantation. Survival for the entire group of 10 patients is 90%, and the median postoperative hospital stay was 17 days. During follow-up (12-62 months; median, 27 months), there were no deaths. Neoaortic insufficiency after anatomic correction was common (mild in one patient, moderate in two patients, and severe in one patient who required aortic valve replacement 4 months after surgery). In the transplantation group, one patient developed lymphoma 3 months after transplantation but is currently in remission after reduction of immunosuppression., Conclusions: In patients who develop late right ventricular failure after Mustard or Senning repair, surgical intervention with either anatomic correction or cardiac transplantation can be done with acceptable morbidity and low mortality. Neoaortic valve insufficiency demands close follow-up after anatomic correction.

Between 1971 and 1983, 201 patients received synthetic right heart conduits, predominantly porcine-valved Dacron conduits, at The Children's Hospital, Boston. There were 45 hospital deaths (22%). Follow-up has been achieved in 148 of 156 survivors (95%). Thirty-four conduits have been replaced, all because of conduit obstruction. The actuarial freedom from conduit replacement was 81% at 5 years, 61% at 7 years, and 0% at 10 years for valved conduits. There was no significant difference to 5 years in reoperation rate between patients with Carpentier-Edwards and those with Hancock conduits. Patients older than 18 years at the time of conduit insertion were 92% free of conduit replacement at 5 years. Those with nonvalved conduits were 100% reoperation free at 4 years. The actuarial survival of patients with valved conduits was 91% at 5 years and 83% at 10 years. The poor performance of porcine-valved tightly woven Dacron conduits warrants a change to use of an alternative conduit, particularly in smaller children. Possible alternatives include antibiotic-sterilized homografts and valved or nonvalved high-porosity knitted Dacron conduits appropriately pretreated with collagen impregnation or fibrin glue.

Between 1972 and 1984, 100 consecutive neonates (less than or equal to 30 days old) underwent repair of coarctation of the aorta. Mean (+/- SD) age at operation was 12.0 +/- 8.0 days; mean weight was 3.1 +/- 0.5 kg. Simple coarctation was present in 29 patients (group I), 32 patients had additional ventricular septal defects (group II) and 39 patients had additional complex heart disease (group III). An associated patent ductus arteriosus was present in a total of 81 patients. Subclavian flap angioplasty (SFA) was performed in 70 patients, resection and end-to-end anastomosis (E-E) in 24 patients, and miscellaneous procedures in six patients. All SFA procedures were performed after 1977, and 87.5% of E-Es were done before 1977. The early mortality was 33.3% for patients undergoing E-E and 11.4% for those undergoing SFA. Freedom from reintervention for recoarctation after 5 years was 92.9% for patients who underwent E-E and 75.2% for those who underwent SFA. Actuarial survival at 4 years was 85.5% for group I, 79.9% for group II, and 42.9% for group III. Follow-up was 97.7% for a mean (+/- SD) of 41.9 +/- 37.1 months. It is likely that some of the differences between patients undergoing SFA and E-E with respect to early mortality and freedom from reintervention are due to the different time frames during which the procedures were done. However, neonatal ductal tissue after SFA may contract and fibrose causing recoarctation, while disadvantages related to the circumferential suture line after E-E may have been overemphasized in the past. A prospective randomized trial of these two techniques is warranted.