Abstract

Di-George syndrome. Two patients had interrupted aortic arch. The median age and weight at surgery were 2.9 months (0.70– 33.37 months) and 3.50 kg (2.70–9.80 kg), respectively. Eleven patients (58%) were operated at less than 3 months of age. 12 patients (57%) had tricuspid truncal valve, 3 (20%) had quadricuspid truncal valve and 6 (29%) had bicuspid truncal valve. Nine (47%) patients had truncal valve stenosis and 9 (47%) had truncal valve insufficiency prior to surgery. 15 patient (71.4%) had no regurge post surgical repair. Early and late mortality were 4.7% each. Three patients (14%) required reintervention in the form of conduit balloon dilatation in one and conduit replacement with pulmonary artery plasty in two after two year of the first surgery. All of them are alive and well. Conclusion: TA repair can be done in early infancy with low mortality. Careful follow up is mandatory as some patients may develop stenosis of the RV-PA conduit requiring reintervention.

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