DeCarlo Dana M, Cha Christine, Pierce Kristyn, Singh Rakesh K, Srinivasan Ranjini
Division of Pediatric Cardiology, Hassenfeld Children's Hospital at NYU Langone, New York, USA.
Pediatric Cardiology, Nicklaus Children's Hospital, Miami, FL, USA.
Pediatr Cardiol. 2024 Aug 9. doi: 10.1007/s00246-024-03607-5.
Patients with hypoplastic left heart syndrome (HLHS) and its variants rely on the right ventricle (RV) to provide cardiac output. Diminished RV systolic function has been associated with poor clinical outcomes in this population. Echocardiographic strain has emerged as a useful method to quantify RV deformation. We aimed to describe fetal strain in the systemic RV and further investigate if there was any correlation with clinical outcomes. We conducted a retrospective, single center study evaluating strain in fetuses with systemic RV. We measured fetal RV global longitudinal strain (GLS) and segmental strain using Tomtec 2D speckle tracking software and compared these findings to controls. Fifty patients with systemic RV were included in the study group with controls matched one to one for each echocardiogram. Ten patients died after first-stage palliation. GLS was reproducible, with interobserver ICC 0.82. There was no statistically significant difference in GLS among different HLHS subtypes. Abnormal GLS did not correlate with worse clinical outcomes. GLS in systemic RVs in the 2nd and 3rd trimester did not vary significantly throughout gestation and did not correlate with clinical outcomes. Risk factors associated with poor outcome were mainly postnatal. Multi-centered studies are needed to determine if these findings hold true in a larger sample size.
患有左心发育不全综合征（HLHS）及其变体的患者依赖右心室（RV）来提供心输出量。右心室收缩功能减弱与该人群不良的临床结局相关。超声心动图应变已成为量化右心室变形的一种有用方法。我们旨在描述系统性右心室的胎儿应变，并进一步研究其与临床结局是否存在任何相关性。我们进行了一项回顾性单中心研究，评估系统性右心室胎儿的应变情况。我们使用Tomtec二维斑点追踪软件测量胎儿右心室整体纵向应变（GLS）和节段应变，并将这些结果与对照组进行比较。研究组纳入了50例系统性右心室患者，对照组与每例超声心动图进行一对一匹配。10例患者在一期姑息治疗后死亡。GLS具有可重复性，观察者间的组内相关系数（ICC）为0.82。不同HLHS亚型之间的GLS无统计学显著差异。异常的GLS与较差的临床结局无关。妊娠中期和晚期系统性右心室的GLS在整个妊娠期无显著变化，且与临床结局无关。与不良结局相关的危险因素主要在出生后。需要进行多中心研究以确定这些发现在更大样本量中是否成立。
