Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
J Am Soc Echocardiogr. 2018 Jul;31(7):831-842. doi: 10.1016/j.echo.2018.02.001. Epub 2018 Apr 12.
Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx).
RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery. The composite end point of death or HTx was examined. Receiver operating characteristic analysis was performed, and cutoff values optimizing sensitivity and specificity were derived.
At a median follow-up of 5.0 years (interquartile range, 2.8-6.4 years), 13 patients meeting the composite end point had lower longitudinal strain and SR, circumferential SR, and RVFAC compared with survivors (n = 51). The conventional cutoff of RVFAC < 35% was specific for death or HTx (86%) but had poor sensitivity (46%), with an area under the curve of 0.73. Speckle-tracking echocardiographic variables showed similar areas under the curve (range, 0.69-0.79), with negative predictive values >90%. Addition of speckle-tracking echocardiographic variables to RVFAC < 35% showed no added benefit. However, in a subpopulation of patients with RVFAC ≥ 35% (n = 44), those meeting the composite end point (n = 7) had lower longitudinal SR (median, -1.0 1/sec [interquartile range, -0.8 to -1.1 1/sec] vs -1.21/sec [interquartile range, -1.0 to -1.3 1/sec], P = .03). Interobserver reproducibility was superior for longitudinal strain and SR (intraclass correlation coefficient > 0.92) compared with RVFAC (intraclass correlation coefficient = 0.75).
Children with hypoplastic left heart syndrome with normal RVFAC and ventricular deformation before bidirectional cavopulmonary anastomosis have a low likelihood of death or HTx in the medium term. In the presence of reduced RVFAC, speckle-tracking echocardiography does not provide additional prognostic value. However, in patients with "normal" RVFAC, it may have a role in improving outcome prediction and warrants further investigation.
左心发育不全综合征患儿存在心室功能障碍与发病率和死亡率增加相关。本研究旨在评估双向格林手术前常规和斑点追踪超声心动图右心室（RV）功能测量在预测死亡或需要心脏移植（HTx）方面的诊断性能。
前瞻性纳入 64 例左心发育不全综合征患儿，在双向格林手术前获取超声心动图，测量 RV 局部射血分数（RVFAC）、纵向应变和应变率（SR）以及圆周应变和 SR。终点为死亡或 HTx。进行受试者工作特征（ROC）曲线分析，确定最佳敏感性和特异性的截断值。
中位随访 5.0 年（四分位间距，2.8-6.4 年），13 例符合复合终点的患者与幸存者（n=51）相比，纵向应变和 SR、圆周 SR 以及 RVFAC 均较低。RVFAC＜35%的常规截断值特异性预测死亡或 HTx（86%），但敏感性差（46%），曲线下面积为 0.73。斑点追踪超声心动图变量的曲线下面积相似（范围 0.69-0.79），阴性预测值＞90%。将斑点追踪超声心动图变量添加到 RVFAC＜35%中并未显示出额外的益处。然而，在 RVFAC≥35%的患者亚组（n=44）中，符合复合终点的 7 例患者纵向 SR 更低（中位数：-1.0 1/sec [四分位间距，-0.8 至-1.1 1/sec] 比 -1.21/sec [四分位间距，-1.0 至-1.3 1/sec]，P=0.03）。与 RVFAC（组内相关系数=0.75）相比，纵向应变和 SR 的观察者间可重复性更好（组内相关系数＞0.92）。
双向格林手术前 RVFAC 和心室变形正常的左心发育不全综合征患儿在中期死亡或 HTx 的可能性较低。在 RVFAC 降低的情况下，斑点追踪超声心动图并不能提供额外的预后价值。然而，在 RVFAC“正常”的患者中，它可能在改善预后预测方面发挥作用，值得进一步研究。
