We report a case of interruption of the aortic arch associated with patency of the arterial duct, restrictive ventricular septal defect, obstruction of the left ventricular outflow due to a discrete subaortic shelf and severe pulmonary arterial hypertension. The patient survived to adulthood, when a complete angiographic diagnosis was made. The subaortic obstruction was treated by percutaneous balloon dilation performed through a right brachial arteriotomy. Correction of the interrupted arch was carried out surgically using a woven Dacron graft from the aortic arch to the descending thoracic aorta associated with ligation of the duct. Hemodynamic studies after one year revealed a marked decrease in pulmonary vascular resistance and a sustained reduction in the gradient across the left ventricular outflow tract. The case is of interest in the light of the unusual survival and the success of staged management of this complex situation.

In a neonate born prior to term with a weight of 1825 grams, and diagnosed prenatally as having atrioventricular septal defect and Down's syndrome, we found the aortic arch to be interrupted between the left carotid artery and the left subclavian artery, with the arterial duct being the only route of distal perfusion. Three days later, however, echocardiographic interrogation revealed marked collateral connections between the aortic arch and the descending aorta, the picture then mimicking coarctation rather than interruption of the aortic arch. The rapid development of the collateral arteries was confirmed by magnetic resonance imaging and during cardiac surgery.