Objectives: Surgical outcomes of patients with functional single ventricle have improved, though those for patients whose condition is complicated by extracardiac type total anomalous pulmonary venous connection (TAPVC) remain poor. We retrospectively reviewed our 21 years of surgical experiences with this challenging group.
Methods: From 1990 to 2010, 48 consecutive patients with functional single ventricle complicated by extracardiac TAPVC (26 males, 46 with right atrial isomerism) underwent initial surgical palliation at our centre. The median age and body weight at surgery were 69 days and 3.5 kg, respectively. The type of TAPVC was supracardiac in 31 patients, infracardiac in 14 and mixed type in 3. TAPVC was repaired in 25 patients before bidirectional Glenn (BDG) and 18 at BDG, while it remained in 3 patients. Since 2007, stent implantation for obstructive drainage veins for patients with preoperative pulmonary venous obstruction and sutureless marsupialization for relief of postoperative pulmonary venous stenosis (PVS) have been initiated. The mean follow-up period was 4.2 ± 5.1 years.
Results: The overall survival rates at 1, 3 and 5 years after the initial surgical intervention were 58.3, 41.1 and 31.3%, respectively. Sixteen patients achieved the Fontan operation (33.3%). The freedom from postoperative PVS rates at 1 and 3 years after repair was 68.7 and 63.4%, respectively. Univariate analysis detected that infracardiac TAPVC (P = 0.036), coexisting major aortopulmonary collaterals (P = 0.017), and TAPVC repair before BDG (P = 0.036) all reduced survival, and multivariable analysis indicated the repair of TAPVC before BDG as the only risk factor (P = 0.032). Whereas the occurrence of postoperative PVS did not reduce survival, which had a significant negative impact on achieving the Fontan operation (P = 0.008). The cumulative survival rate did not improve by surgical era.
Conclusions: Surgical outcomes of patients with functional single ventricle undergoing the repair of extracardiac TAPVC in the neonatal period due to obstruction of the venous drainage pathway remain poor. Stent implantation for obstructive drainage veins to delay the timing of surgical correction and sutureless marsupialization as relief of postoperative PVS are expected to improve the late outcomes; however, the effect is still limited.