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Thrombus formation is a feared complication following bidirectional cavopulmonary shunt (BCPS). We aimed to investigate the effect of thrombus formation on outcome. BCPS was performed in 525 patients at our center between 1998 and 2018. The impacts of thrombus formation on survival and probability of Fontan completion were analyzed, and risk factors for thrombus formation were examined. Thrombus formation occurred in 30 patients (5.7%). Compared with the remaining 495 patients, there was no significant difference in the median age at BCPS (4.9 vs 4.7 months; P = 0.587). However, unbalanced atrioventricular septal defects (17 vs 5%; P = 0.008) and preoperative ventricular dysfunction (23.3 vs 8%; P = 0.004) were more frequent in patients who developed a thrombus. Thrombolytic therapy was performed in all patients and surgical thrombus removal was required in 13 patients. In-hospital mortality was higher in patients with thrombus (30.0 vs 2.2%; P0.001). Of 505 hospital survivors, an estimated survival at 1 year after hospital discharge following BCPS was 84.4% (95% CI, 76.1-92.7%) in patients with thrombus and 96.8% (95% CI, 96.0-97.6%) in those without (P0.001). Cumulative incidence of Fontan completion at 3 years after BCPS was 52.8% (95% CI, 30.3-75.2%) in patients with thrombus and 90.1% (95% CI, 87.2-92.9%) in those without (P = 0.004). Higher left atrial pressure (OR = 1.165; P = 0.029) and longer cardiopulmonary bypass time (OR = 1.013, P = 0.001) at BCPS were independent risk factors for thrombus formation after BCPS. Thrombus formation after BCPS poses a significant risk for survival and Fontan completion. Preoperative higher left atrial pressure and longer cardiopulmonary bypass time are significant risk factors.

Bilateral superior vena cava (SVC), which occurs following bilateral bidirectional cavopulmonary shunt (BCPS), is an anomaly marked by unique hemodynamics. This study aimed to determine its effects on outcomes after Fontan completion. Among 405 patients who underwent BCPS and total cavopulmonary connection (TCPC) between 1997 and 2017, 40 required a bilateral-BCPS. The dominant SVC prior to TCPC was identified according to the direction of blood flow in the central pulmonary artery, and its relationship to the inferior vena cava (IVC) was classified as a concordant or discordant relationship. Preoperative factors were analyzed to identify the risk factors for specific adverse outcomes. The length of intensive care unit (ICU) stay after TCPC was longer in the 40 patients who underwent bilateral-BCPS than in those who underwent unilateral BCPS (p = 0.024), and the survival rate was lower in the former group than in the latter group (p = 0.004). In the patients who underwent bilateral-BCPS, the dominant SVC was concordant with the IVC in 30 patients and discordant in 10 patients. With regard to whether certain morphological, hemodynamic, and flow dynamics-related variables were risk factors for adverse outcomes following TCPC, a discordant relationship between dominant SVC and IVC was identified as an independent risk factor for both a longer ICU stay (p = 0.037, HR 2.370) and worse survival (p = 0.019, HR 13.880). Therefore, in patients with a bilateral SVC who have previously undergone bilateral-BCPS, a discordant relationship between dominant SVC and IVC might contribute to worse outcomes following TCPC. Electronic supplementary material The online version of this article (10.1007/s00246-020-02318-x) contains supplementary material, which is available to authorized users.

OBJECTIVES The goal of this study was to identify the risk factors for prolonged length of stay (LOS) in the intensive care unit (ICU) after a bidirectional cavopulmonary shunt (BCPS) procedure and its impact on the number of deaths. METHODS In total, 556 patients who underwent BCPS between January 1998 and December 2019 were included in the study. RESULTS Eighteen patients died while in the ICU, and 35 died after discharge from the ICU. Reduced ventricular function was significantly associated with death during the ICU stay (P = 0.002). In patients who were discharged alive from the ICU, LOS in the ICU [hazard ratio (HR) 1.04, 95% confidence interval (CI) 1.02–1.06; P CONCLUSIONS Prolonged LOS in the ICU with a cut-off value of 19 days after BCPS was a significant risk factor for mortality. High pulmonary artery pressure at BCPS was a significant risk factor for a prolonged ICU stay.