Mid-Term Results and Risk Factors For 10 Years of Functional Single Ventricle Associated With Total Anomalous Pulmonary Venous Connection

Background: There are few surgical treatment results in elderly patients with functional single ventricle (FSV) and total anomalous pulmonary venous connection (TAPVC). We retrospectively analyzed 10 years of mid-term surgical treatment results and risk factors of these age-specific people. Methods: Between March 2008 and December 2018, 43 consecutive patients with FSV and TAPVC received initial surgical palliation in our center. There were 20 cases of supra cardiac TAPVC, 21 of cardiac type, and two cases of mixed type. Initial surgical palliation procedures involved pulmonary artery banding (PAB) for patients, modified BlalockTaussing shunt (mBTs) for five patients, and bidirectional Glenn (BDG) for 34 patients. TAPVC repair was performed in 12 patients during BDG. Results: The 1-year and 5-year overall survival rates were 69.7% and 62.8%, respectively. In TAPVC repair group and non-TAPVC repair group, the 1-year overall survival rates after initial surgical palliation were 41.7 and 80.5%, respectively, and the 3-year ones were 25% and 77%, respectively. There were significant differences in the type of TAPVC (P < 0.001), preoperative pulmonary venous obstruction (P = 0.001), and overall mortality (P = 0.001) between these two groups. Cox univariate and multivariable analysis indicated concomitant TAPVC repair was the only risk factor for mortality. Conclusions: The mid-term results of surgical treatment of FSV and TAPVC, especially for patients who underwent concomitant TAPVC repair, remain poor. TAPVC repair may be a priority over single-ventricular palliative surgery for patients with FSV and TAPVC.