Objective · To analyze the risk factors for the death of patients with heterotaxy syndrome. Methods · The clinical data of 56 patients with clear diagnosis of heterotaxy syndrome were collected. Clinical characteristics and risk factors for death were analyzed. Results · 47 patients were right isomerism and 9 patients were left isomerism according to anatomical classification. Patients with right isomerism had more severe cardiovascular malformation than those with left isomerism. Of 56 patients, 6 (10.7%) with right isomerism died, in which 5 (83.3%) had moderate to severe atrioventricular valve regurgitation. Results of Mann-Whitney test and Chi-squared test showed that the differences in incidences of moderate to severe atrioventricular valve regurgitation and postoperative low blood oxygen saturation ( ≤80%) were statistically significant (P=0.033 6, P=0.041 7). Univariate Logistic regression analysis showed moderate to severe atrioventricular valve regurgitation was a risk factor for the death of patients with heterotaxy syndrome [OR (95% CI): 11.666 (1.254-108.557), P=0.030 9]. Conclusion · Moderate to severe atrioventricular valve regurgitation is an important risk factor for the death of patients with heterotaxy syndrome.