Background: Hearts with right atrial isomerism (RAI) are usually combined with complex cardiac anomaly and the surgical management is varied and challenging. We reviewed our experience of patients with RAI. Methods: Between April 1991 and April 2006, 84 patients were diagnosed with right atrial isomerism. Of these, 75 patients underwent surgical treatment. Median age and body weight at initial operation were 5.7months (0-141months) and 6.2kg (2.1-29.5kg), respectively. Thirty-five (46.7%) patients had total anomalous pulmonary venous return (TAPVR). Pulmonary atresia was 23 (30.7%), pulmonary stenosis (PS) was 40 (53.3%), and no PS was 12 (16%). Follow-up was possible in all patients and median follow up duration was 36.8months (0 day- 182months). Results: In-hospital mortality was 13.3% (10/75) and late mortality was 20% (13/65). The most common cause of in-hospital mortality was low cardiac output (n=5). Causes of late death were infection (n=5), pulmonary vein stenosis (n=3), shunt occlusion (n=3), and cerebral infarction (n=1). Biventricular repair was done in two patients with one death. The others were in the single ventricle physiology. Until now 40 patients (53.3%) received Fontan operations. Overall survival estimates were 71% at 5 years, and 53.7% at 10 years. Survival rates in patients without TAPVR were 88% at 5 and 10 years and in patients with TAPVR were 52.7% at 5 years and 20.1% at 10 years. Risk factors for in-hospital mortality in univariate analysis were age less than 1 month (p=0.019), TAPVR (p=0.038), and no PS (p=0.048). Risk factor for late death was only TAPVR (p=0.001). Conclusions: This study showed that the early results of patients with RAI were not so bad, especially, in patients without TAPVR. During the follow up we should keep in mind the problems related to the asplenia, pulmonary vein stenosis, and shunt occlusion.