Over a 13-year period, 20 infants and children underwent transsternal approach for repair of coarctation and associated cardiac defects. Fifteen patients (75%) were operated on in the last 6 years. Thirteen patients (group 1) had intracardiac shunts and 7 (group 2), intracardiac obstruction or valvular insufficiency. Group 1 had a mean age of 0.8 +/- 1.9 years versus 4 +/- 3 years for group 2 (p = 0.05). There were 12 patients (92%), 7 months old or less in group 1. Aortic arch hypoplasia was present in 6 patients in group 1. A large patent ductus arteriosus was present in 5 of these 6 patients versus no patent ductus arteriosus in patients without aortic arch hypoplasia (p = 0.006). The mean pulmonary blood now to systemic blood flow ratio in group 1 was 3.8 +/- 2 and the mean right ventricular to left ventricular ratio, 0.8 +/- 0.2. The coarctation repair fell mostly into three types: side patch aortoplasty (8), ductal tissue excision and patch aortoplasty of the concavity of the aortic arch (6), and subclavian aortoplasty (4). There was one early death (5%) which was due to sepsis in a newborn. Another newborn who had subclavian aortoplasty needed a left carotid artery-descending aorta bypass conduit early because of aortic arch hypoplasia. All patients were followed to 12 years (mean follow-up, 4.3 +/- 3.5 years). There were no late deaths. Two patients had recurrent coarctation, 1 after an end-to-end repair and the other because of incomplete arch enlargement after a side patch aortoplasty. We conclude that a transsternal approach, which minimizes incisions and reoperations, can be safely accomplished in infants and children to repair coarctation and associated cardiac defects. Although most coarctation repair techniques could be performed, ductal tissue excision with patch aortoplasty of the concavity of the arch appears simple and provides an excellent option for complete relief of obstruction, unhampered aortic growth, and probably reduced aneurysm formation.