Stanley Helen M, Faerber Jennifer A, Cohen Meryl S, Callahan Ryan, Fuller Stephanie M, White Brian R
Department of Pediatrics, Division of Cardiology, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Healthcare Analytics Unit, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Echocardiography. 2025 Mar;42(3):e70124. doi: 10.1111/echo.70124.
Development of postoperative obstruction in total anomalous pulmonary venous connection (TAPVC) is a major cause of morbidity and mortality. Although preoperative echocardiography has often been cited as prognostic of postoperative outcome, its predictive value has not been fully evaluated. Pulmonary venous variability index (PVVI) is an echocardiographic metric developed at our center and previously shown to correlate with preoperative clinical markers and catheterization findings of obstruction. We hypothesized that preoperative PVVI would be superior to maximum and mean velocity for prediction of postsurgical outcome in TAPVC.
We performed a retrospective review of TAPVC patients repaired at our center. Preoperative echocardiograms were reviewed for clinical read, and measures of pulmonary venous obstruction including maximum, mean, and minimum velocity and PVVI ([maximum velocity-minimum velocity]/mean velocity) were calculated from spectral Doppler of the pulmonary venous pathway. The outcome was time to surgical or catheter-based pulmonary vein reintervention.
In total, 162 patients were included and 33 (20%) underwent reintervention. On univariate Cox proportional hazards model, single ventricle status, mixed-type TAPVC, and PVVI ≤ 0.5 were predictive of reintervention (hazard ratios of 2.7, p = 0.01; 3.2, p = 0.01; and 2.2, p = 0.03, respectively). Absolute echocardiographic velocities were not associated with the outcome. On multivariate analysis, single ventricle status and mixed-type TAPVC remained significant predictors of reintervention, while PVVI did not.
Though preoperative PVVI was associated with an increased risk of postoperative reintervention in TAPVC by univariate analysis, multivariate analysis suggests that single ventricle status and TAPVC subtype are the strongest drivers of postoperative outcomes. Preoperative velocities are not predictive of outcome in TAPVC.
完全性肺静脉异位连接（TAPVC）术后梗阻的发生是发病和死亡的主要原因。尽管术前超声心动图常被认为可预测术后结果，但其预测价值尚未得到充分评估。肺静脉变异指数（PVVI）是我们中心开发的一种超声心动图指标，此前已证明它与术前梗阻的临床标志物及心导管检查结果相关。我们假设术前PVVI在预测TAPVC手术结果方面优于最大和平均速度。
我们对在本中心接受修复的TAPVC患者进行了回顾性研究。回顾术前超声心动图的临床解读，并从肺静脉通路的频谱多普勒中计算肺静脉梗阻的指标，包括最大、平均和最小速度以及PVVI（[最大速度 - 最小速度]/平均速度）。结果指标为手术或基于导管的肺静脉再次干预的时间。
总共纳入162例患者，其中33例（20%）接受了再次干预。在单因素Cox比例风险模型中，单心室状态、混合型TAPVC和PVVI≤0.5可预测再次干预（风险比分别为2.7，p = 0.01；3.2，p = 0.01；和2.2，p = 0.03）。绝对超声心动图速度与结果无关。多因素分析显示，单心室状态和混合型TAPVC仍然是再次干预的显著预测因素，而PVVI不是。
虽然单因素分析显示术前PVVI与TAPVC术后再次干预风险增加相关，但多因素分析表明单心室状态和TAPVC亚型是术后结果的最强驱动因素。术前速度不能预测TAPVC的结果。
