Background

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).

Methods

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.

Results

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).

Conclusions

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.

中文翻译：

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发育不良的左心综合征患儿双向腔静脉吻合术前右心室分数变化，应变和应变率降低与中期死亡率相关

背景

发育不良的左心综合征儿童的心室功能障碍与发病率和死亡率增加有关。这项研究的目的是评估常规和斑点跟踪超声心动图对双向心腔肺吻合术缓解之前的右心室（RV）功能的诊断性能，以预测死亡或需要进行心脏移植（HTx）。

方法

根据双向腔静脉吻合术前获得的超声心动图，对64例预期增生的左心发育不全患者进行了RV面积变化（RVFAC）和纵向和周向应变以及应变率（SR）的测量。检查了死亡或HTx的复合终点。进行接收器工作特性分析，得出优化灵敏度和特异性的临界值。

结果

在中位随访时间为5.0年（四分位间距为2.8-6.4年）中，13例达到复合终点的患者的纵向应变和SR，圆周SR和RVFAC低于幸存者（n  = 51）。RVFAC的常规截止值<35％对死亡或HTx具有特异性（86％），但敏感性较差（46％），曲线下面积为0.73。斑点跟踪超声心动图变量显示曲线下的相似区域（范围0.69–0.79），阴性预测值> 90％。RVFAC <35％时，增加了斑点跟踪超声心动图变量，则未显示出额外的益处。但是，在RVFAC≥35％的患者人群中（n  = 44），满足复合终点的患者（n = 7）具有较低的纵向SR（中位数，-1.0 1 /秒[四分位间距，-0.8至-1.1 1 / sec]与-1.21 / sec [四分位间距，-1.0至-1.3 1 / sec]，P  =。 03）。与RVFAC（类内相关系数= 0.75）相比，纵向应变和SR（类内相关系数> 0.92）的观察者间可重复性更好。

结论

RVFAC正常且在双向腔肺吻合之前出现心室畸形的发育不良的左心综合征儿童在中期内死亡或HTx的可能性较低。在RVFAC降低的情况下，散斑跟踪超声心动图不能提供额外的预后价值。但是，对于RVFAC“正常”的患者，它可能在改善预后方面具有一定作用，需要进一步研究。