Harake Danielle, Kapravelou Eva, Eckersley Luke, Tham Edythe, Colen Timothy, Lin Lily, Shigemitsu Sachie, Beigh Mirza, Moray Amol, Khoo Nee Scze
Stollery Children's Hospital and University of Alberta, Edmonton, Canada.
Echocardiography. 2025 Jul;42(7):e70244. doi: 10.1111/echo.70244.
Echocardiography parameters of right ventricular (RV) dysfunction, fractional area change (RVFAC), and global longitudinal strain during the first interstage have been shown to be associated with death or transplantation in patients with HLHS. However, both parameters lack adequate discriminatory characteristics. This study sought to examine global post-systolic contraction (PSC), a marker of myocardial inefficiency, as a predictor of death or transplantation (Tx) in patients with classic hypoplastic left heart syndrome (HLHS).
We performed a post-hoc analysis of 62 prospectively recruited patients with HLHS prior to the bidirectional cavopulmonary anastomosis (BCPA) surgery. We measured RV function parameters: RVFAC, global longitudinal strain, strain rate, mechanical dispersion index (MDI), and PSC. For each parameter, the receiver operating characteristic analysis determined the optimal cut-offs for the primary endpoint of death/Tx, followed by a Kaplan-Meier analysis. Parameters interobserver variability testing was performed.
Median follow-up from pre-BCPA echocardiogram was 7.4 years. HLHS with outcome of death/Tx (n = 14) had lower RVFAC, longitudinal strain and strain rate, and increased PSC when compared with survivors. PSC of >5% performed the best, with the greatest area under the curve 0.75, sensitivity 64% and specificity 83%, hazard ratio 5.54 (95% CI 1.63-18.66), for the primary endpoint of death/Tx. PSC and strain parameters had excellent reproducibility.
Increased global post-systolic contraction during the first interstage is associated with an outcome of death or transplantation in the medium term follow up of patients with HLHS. PSC has a greater specificity and reproducibility than global longitudinal strain and RV FAC. Investigation into its use in clinical practice as a predictor of outcome is warranted.
在首次分期手术期间,右心室(RV)功能障碍的超声心动图参数、面积变化分数(RVFAC)和整体纵向应变已被证明与左心发育不全综合征(HLHS)患者的死亡或移植相关。然而,这两个参数都缺乏足够的鉴别特征。本研究旨在检验心肌效率低下的标志物——整体收缩后收缩(PSC),作为经典左心发育不全综合征(HLHS)患者死亡或移植(Tx)的预测指标。
我们对62例前瞻性招募的HLHS患者在双向腔肺吻合术(BCPA)手术前进行了事后分析。我们测量了右心室功能参数:RVFAC、整体纵向应变、应变率、机械离散指数(MDI)和PSC。对于每个参数,通过受试者工作特征分析确定死亡/Tx主要终点的最佳截断值,随后进行Kaplan-Meier分析。进行了参数的观察者间变异性测试。
从BCPA术前超声心动图开始的中位随访时间为7.4年。与幸存者相比,死亡/Tx结局的HLHS患者(n = 14)的RVFAC、纵向应变和应变率较低,PSC增加。对于死亡/Tx主要终点,PSC>5%表现最佳,曲线下面积最大为0.75,敏感性为64%,特异性为83%,危险比为5.54(95%CI 1.63 - 18.66)。PSC和应变参数具有出色的可重复性。
在HLHS患者的中期随访中,首次分期手术期间整体收缩后收缩增加与死亡或移植结局相关。PSC比整体纵向应变和RV FAC具有更高的特异性和可重复性。有必要研究其在临床实践中作为结局预测指标的应用。