Outcomes in patients with ventricular dysfunction undergoing superior cavopulmonary connection (SCPC) are not well known. We reviewed records of patients undergoing SCPC at our center from December 2005 to October 2009 and studied those whose pre-SCPC echocardiograms demonstrated at least moderate systemic ventricular dysfunction. Of the 213 patients undergoing SCPC, 19 (9%) met inclusion criteria. Diagnoses were hypoplastic left heart syndrome (n = 18) and rightward unbalanced atrioventricular canal with pulmonary stenosis (n = 1). In those surviving >2 months after SCPC, ventricular function was assessed by echocardiography 4.9 (range 3.5 to 9.7) months after SCPC and was improved in ten of 17 (59%), unchanged in six of 17 (35%), and worsened in one of 17 (16%) patients. After SCPC, three patients died, and one underwent heart transplant 21.9 months after SCPC. Transplant-free survival was attained by 15 of 19 (79%) patients during follow-up of 33.0 months (range 10.8 to 51.4). Fontan completion was performed on six survivors on reaching an appropriate age. Ventricular dysfunction before SCPC is not uncommon and occurs primarily in patients with a morphologic right ventricle. Ventricular performance improves in the majority of patients after SCPC, and midterm outcomes are comparable with previous reports, suggesting that these patients remain candidates for staged palliation.