Abstract

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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