Univentricular atrioventricular connection with subaortic stenosis: a staged surgical approach

A staged surgical approach was developed for the management of hearts with univentricular atrioventricular connection (double-inlet left ventricle or tricuspid atresia) and discordant ventriculoarterial connection with anatomical substrate for the development of subaortic stenosis. This consisted of initial palliation with pulmonary artery banding, followed by early elective relief of subaortic obstruction using a proximal pulmonary artery to ascending aorta anastomosis in infancy. Pulmonary blood flow was maintained at this time by creating a bidirectional superior cavopulmonary anastomosis. Over an 18-month period, 5 children, including 4 seen in the first week of life with aortic arch obstruction, were palliated with this approach. All patients survived operation and are asymptomatic with transcutaneous oxygen saturations of 80% to 85%. Completion of cavopulmonary repair is planned at 2 years of age. Although some authors have considered pulmonary artery banding contraindicated in these infants, the current staged approach offers an attractive alternative to the construction of a pulmonary artery to aorta anastomosis in the neonatal period.