Abstract
Backgroud : Surgical results of functional single ventricle
(FSV) patients with totally anomalous pulmonary venous connection
(TAPVC), have a poor outcome. We retrospectively analyzed our 10-year
surgical clinical experience and risk factors of mortality of these
patients.
Methods : Between March 2008 and August 2018, 43 consecutive
patients with FSV and TAPVC underwent initial surgical palliation and
TAPVC repair or not. The median body weight and age were 12 (range 5-44)
kg and 32 (range 2-256) months, respectively. Among these cases, there
are 19 cases of supracardiac TAPVC, 22 of intracardiac type, and 2 of
mixed type. 12 patients need to perform TAPVC repair during initial
surgical palliation (supracardiac in 10 and mixed type in 2).
Results : Overall survival at 1 and 5 years were 89.5% and
83.3%, respectively. In TAPVC repair group and non-TAPVC repair group,
overall survival after the initial surgical palliation were 58.3 and
87.1% at 1 year, 40.0% and 87.1% at 3 years, respectively. Cox
univariate analysis detected that preoperative pulmonary vein
obstruction (PVO) (p =0.047) and concomitant TAPVC repair
(p =0.007) were risk factors for mortality, and multivariable
analysis indicated concomitant TAPVC repair as the only factor
(p =0.033).
Conclusions : The mid-term results of surgical results of FSV
associated with TAPVC, especially for patients who need to concomitant
TAPVC repair, remain poor. Preoperative PVO is identified as risk factor
that increases mortality of these patients.
KEYWORDS : Single ventricle, Total anomalous pulmonary venous
connection, Pulmonary vein obstruction