ObjectiveTo investigate the effect of fenestration on total cavopulmonary connection (TCPC) in the treatment of complex congenital heart disease. MethodsWe retrospectively analyzed the clinical data of 142 patients undergoing TCPC in Fu Wai Hospital between January 2010 and December 2013. The patients were divided into 2 groups depending on with fenestration or not. There were 71 patients including 44 males and 27 females at age of 65.7+24.5 months in the fenestration group. There were also 71 patients with 42 males and 29 females at age of 60.7+20.8 months in the no fenestration group. Perioperative variables were compared between the two groups. ResultsFour patients (2.82%) died postoperatively. The fenestration significantly increased in the patients with atrioventricular valve regurgitation (AVVI). There were no statistical differences in duration of mechanical ventilation, ICU hospitalized time, early mortality and complications between the two groups (P>0.05). But there were statistical differences in the postoperative pleural effusion duration and 24 h capacity requirement (9.1 d versus 13.1 d, 4.19 ml/(kg · h) versus 5.48 ml/(kg · h)) between the two groups. In the patients whose preoperative mPAP was more than 12 mm Hg, postoperative CVP was lower (P=0.046), maintaining the same blood pressure (SBP=80-90 mm Hg) of vasoactive drugs (P=0.019) and 24 h capacity requirement (P=0.041) were lower, pleural effusion duration was shorter (9.8 d versus 17.8 d, P=0.000) in the fenestration children. 113 patients were followed up for 1.1+1.2 years. SpO2 was 92.1%+3.5% in the fenestration children. Spontaneous closure occured in 8.5% of the patients. No severe cyanosis (SpO2<85%), limb embolism, or stroke. ConclusionFenestration should not be a routine in children of TCPC. Patients with fenestration or not can obtained satisfactory early clinical efficacy. Atrial septal fenestration should be considered in high-risk children with mPAP higher than 12 mm Hg or serious AVVI and be corrected at the same time. Fenestration contributes to stable circulation after TCPC surgery. It can shorten the duration of pleural effusion.