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Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by insufficient left ventricle growth and diastolic dysfunctions. We hypothesized that reduced blood flow into the left heart in the uterus could also affect left atrium (LA) growth, leading to anomalies after birth. We compared the age-related changes in LA parameters detected using two-dimensional speckle-tracking echocardiography (2DSTE) in patients with TAPVC. Method: The LA datasets of 28 neonates with isolated TAPVC were analyzed before surgery and during follow-ups and compared with those of age-matched healthy controls (January 2009–2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) functions. LA pressure was determined by performing catheterization. Results: Compared to the control group, the TAPVC group had significantly smaller LA maximum size preoperatively and normal and larger time-dependent increase in the LA maximum and minimum size, respectively. All 2DSTE-determined LA functions showed significant reductions at all time points in the TAPVC group compared to those in the control group (median εR, εCD, and εCT; before surgery: 17.0 vs. 26.0, 12.9 vs. 15.9, and 6.3 vs. 10.4; follow up at 1-2 years: 30.0 vs. 45.7, 23.2 vs. 29.6, and 6.1 vs. 16.3; follow up at 5-7 years: 31.2 vs. 43.1, 25.0 vs. 31.2, and 5.2 vs. 10.8, respectively; p < .01). However, compared to before surgery, εR and εCD did represent a significantly increased trend at follow-ups (median εR and εCD: 16.8→27.5 and 1.1→23%, respectively). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. Conclusion: This study showed that LA impairments existed even after repair in patients with TAPVC, suggesting that LA function loss mediated by less blood flow persist in childhood.

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Introduction: Although the management of body water balance on cardiac surgery with cardiopulmonary bypass (CPB), especially in children, is essential, little is known how body water composition changes around CPB. We aim to evaluate a change of intracellular (ICW), extracellular (ECW), and total body water composition (TBW) before and after CPB, using bioelectrical impedance analysis (BIA). Hypothesis: There is a possibility that the fluid distribution and water balance change depends on intraoperative water balance management and age. Then we investigate the impact of (1) intraoperative water balance management and (2) age at operation on changes of body water composition. Methods: The forty-five children weighing over 5Kg (Age ranges 3mo-11yo) were enrolled to this study. The body water balance was measured at before, at immediately after CPB, at 1 day, and at 2 days, respectively. Each parameter was compared by the factor of intraoperative water balance and that of age at operation. Results: The minus water balance during CPB results in significant shortage of ICW which not fully recovered by 2 days after CPB (Figure A). Cases >2yo developed a decrease of ICW and ECW 2 days after CPB, while those <2yo increased (Figure B). Conclusions: The body water composition significantly changes along CPB than expected. Care must be taken to prevent a harmful dehydration by controlling intraoperative water balance management and by age on the basis of BIA.