Abstract:

Objective To analyse the risk factors for early mortality in patients with heterotaxy syndrome undergoing single ventricle series palliation. Methods The clinical data of 61 consecutive patients with heterotaxy syndrome undergoing single ventricle palliation in Shanghai Children's Medical Center from January 2004 to January 2011 were retrospectively reviewed, and the risk factors for early mortality after operation were analysed. Single ventricle series palliation included Blalock shunt, pulmonary artery band, bidirectional cava-pulmonary shunt, modified Fontan and Kawashima procedure. Early mortality was recorded as a death event from the first surgical procedure to 30 d after Fontan operation. Results There were 8 early death events, with the early mortality of 13.1%. Among the 8 cases, one was left atrial isomerism, and the other 7 were right atrial isomerism; 2 occurred after neonatal operation, 1 occurred after bidirectional cavapulmonary shunt, and the other 5 occurred after modified Fontan. Multivariate Cox regression analysis revealed that neonatal operation (hazard ratio, 5.56; P=0.04), obstructed anomalous pulmonary venous drainage (hazard ratio, 18.21; P=0.02) and greater than moderate atrioventricular valve regurgitation (hazard ratio,12.31; P=0.03) were associated with early mortality after operation in heterotaxy syndrome. Conclusion The surgical outcome of left atrial isomerism may be better than that of right atrial isomerism. Neonatal operation, obstructed anomalous pulmonary venous drainage and greater than moderate atrioventricular valve regurgitation are risk factors for early mortality after operation in heterotaxy syndrome.

Key words: heterotaxy syndrome, surgery, risk factor