Surgical treatment of single ventricle and total anomalous pulmonary venous connection

Abstract

Objective To investigate and summarize the surgical indication and outcome in patients diagnosed with functional single ventricle and total anomalous pulmonary venous connection. Methods A total of 17 patients of functional single ventricle and TAPVC underwent stage Ⅰ surgical palliation between June 2009 and May 2014. For the total study population, 11 males and 6 females were included. Their median age at first intervention was 1.0 year(range, 6 days to 21.0 years), and the median weight was 7.5 kg(ranged, 3.2 kg to 41.0 kg). 13 cases were surgically treated with TAPVC repair in combination with stage Ⅰ palliative procedures, which included B-D Glenn procedure(6 cases), B-D Glenn with atrioventricular valvoplasty(2 cases), B-D Glenn, atrioventricular valvoplasty and pulmonary artery banding(1 case), B-D Glenn with pulmonary artery banding(1 case), pulmonary artery banding(2 cases), modified Blalock-Taussig shunt(2 cases) and patent ductus arteriosus ligation(1 case). There were two cases received TAPVC repair 2 years after B-D Glenn procedure. The remaining two cases were surgical managed through pulmonary artery banding with TAPVC untreated. Results Two of the 17 patients died early postoperatively. For the 12 surviving patients with complete follow-up, median follow-up time was 3.3 years. After initial surgical intervention, two of the 17 cases failed to be followed in 3 months respectively. Conclusion In neonates and small infants diagnosed with functional single ventricle and TAPVC with pulmonary venous obstruction, surgical correction of TAPVC is recommended, and additional intervention of aorto-pulmonary shunting or pulmonary artery banding should be considered. In neonates and small infants who require univentricular palliative procedures, total correction of TAPVC is surgically indicated at the same stage. In patients with unobstructed pulmonary venous return, TAPVC should be properly managed when B-D Glenn shunting is performed. In neonates and small infants with functional single ventricle and TAPVC, early postprocedure mortalities, estimated mid-long term survival rates, and proportions of Fontan circulation eligibility are still pessimistic. Key words: Heart defects, congenital; Total anomalous pulmonary venous connection; Functional single ventricle; Cardiac surgical procedures