BackgroundStenosis of the total cavopulmonary connection (TCPC), in particular the pulmonary arteries, is common in children with single ventricle physiology. Stenting is an efficient interventional strategy to treat stenosis and optimize TCPC hemodynamics. MethodsA retrospective single center study was performed to investigate the prevalence, causes and outcome of TCPC stenosis in children treated with stent implantation guided by three-dimensional rotational angiography (3DRA). ResultsFrom September 2011 to February 15th 2021 48 patients received 73 stents at 67 locations in 59 3DRA stent procedures. Median age and weight were 3.6 years (0.0–17.2) and 14.1 kg (3.4–70.0), respectively. Left pulmonary artery (LPA) stenosis accounted for 75% of the stenosis and is often caused by retroaortic compression. Adverse events occurred in 6 catheterizations (10.2%). Twenty-one patients (43.8%) underwent 27 reinterventions including planned serial redilation (N = 17), extra stent placement (N = 6), surgery (N = 2), unplanned balloon dilation (N = 1) and stent placement (N = 1) for restenosis by intima proliferation. ConclusionTCPC stenoses, especially of the LPA, are common in children with single ventricle physiology and external compression is one of the main causes. 3DRA safely guides stent interventions as intraprocedural knowledge of possible interactions with surrounding structures are evaluated. Besides it is effective in both short and long term and can be performed in small children. Restenosis by intima proliferation is rare, though repeated redilations are necessary to match stent diameter with patient size.