Backgrounds The Damus-Kaye-Stensel (DKS) procedure is effective in management of subaortic stenosis not only in functional single ventricle but also in biventricular repair. We retrospectively reviewed our experience of the DKS procedure. Methods Between 1995 and 2003, 19 patients underwent DKS operation. Median age at the DKS operation was 6.5 months (range, 32days-9.5 years). The DKS operation performed at initial palliation (Group I, n=6) or applied at variable stage after pulmonary artery banding (PAB) (Group II, n=13). If there was the progression of subaortic stenosis after PAB or volume unloading procedure, we performed a DKS procedure at any stage. Coarctation repair was performed in 10 patients. The mean follow-up after the DKS operartion was 3.3 years.Results There were two early mortalities in group I (2/6, 33.3%) and no mortality in group II. In group II, pulmonary vascular resistance (Rp) was decreased to acceptable range (mean Rp: 2.9 wood unit) after PAB, and mean interval between PAB and DKS procedure was 5.7 짹 11 months. In group II, the DKS procedure was done with shunt (n=6), BCPS (n=5) and Fontan repair (n=2) concomitantly. In three patients (initial diagnosis of Taussing-Bing anomaly with coarctation of aorta and subaortic stenosis), ventricular hypertrophy and subaortic stenosis were regressed after DKS procedure, so successful biventricular repair was done(take down of the DKS anastomosis and reuse both aortic and pulmonic valve. Nine of these patients had completed a Fontan procedure and had a good Fontan state. Remaining 5 patients are good candidates for Fontan repair without risk factors. Conclusions This experience demonstrates that initial application of DKS operation beyond neonatal period may be risky. After preliminary short-term PAB, a DKS operation can be done safely. The DKS procedure after short-term period of PAB had no detrimental effect on later Fontan repair. In selected cases, later biventricular repair can be done after regression of subaortic stenosis via a DKS procedure.