Wang Yue, Luo Gang, Sun Yi, Chen Taotao, Pan Silin
Heart Center, Women and Children's Hospital, Qingdao University, Qingdao, China.
Department of Obstetric Ultrasound, Women and Children's Hospital, Qingdao University, Qingdao, China.
Front Pediatr. 2025 Jan 10;12:1518898. doi: 10.3389/fped.2024.1518898. eCollection 2024.
This study aimed to assess right ventricular (RV) endocardial fibroelastosis (EFE) in fetuses with critical pulmonary stenosis (CPS) and pulmonary atresia with intact ventricular septum (PA-IVS) and to investigate the implications of RV EFE for circulatory outcomes.
Fetal echocardiographic data from July 2018 to January 2021 were collected. Three reviewers independently graded EFE based on the presence and extent of endocardial echogenicity. Since this is a novel study on grading RV EFE, intra- and interobserver comparisons were performed. The associations among EFE severity, anatomic variables, and late-gestational circulatory outcomes were analysed.
Eighty-one patients with RV EFE were identified. By consensus, EFE severity was assessed as Grade 1 ( = 66, 81.5%) or Grade 2 ( = 15, 18.5%). At the first consultation, RV sphericity values were greater in Grade 2 EFE fetuses than in Grade 1 EFE fetuses, implying more severe noncompliance and worse diastolic function. From the first consultation to late gestation, significant differences were observed in the changes in the tricuspid/mitral valve (TV/MV) annulus diameter ( = 0.042) and TV -score ( = 0.001) between the Grade 1 and Grade 2 RV EFE groups. Among the ten patients who underwent fetal cardiac intervention (FCI), the restoration of the TV -score was more significant in Grade 2 RV EFE fetuses than in Grade 1 EFE fetuses. Among Grade 2 EFE cases, fetuses who underwent FCI exhibited greater changes in the right/left ventricular (RV/LV) long-axis dimension, TV/MV, and RV sphericity compared to non-FCI fetuses, indicating that FCI benefited Grade 2 EFE fetuses by restoring the development of ventricular structure.
This study graded RV EFE in fetuses with CPS/PA-IVS, shedding light on its implications for circulatory outcomes. FCI offered benefits in Grade 2 RV EFE patients, suggesting its potential to preserve cardiac development in affected fetuses with CPS/PA-IVS.
本研究旨在评估患有严重肺动脉狭窄（CPS）和室间隔完整的肺动脉闭锁（PA-IVS）胎儿的右心室（RV）心内膜纤维弹性组织增生症（EFE），并探讨RV EFE对循环结局的影响。
收集2018年7月至2021年1月的胎儿超声心动图数据。三名评估者根据心内膜回声的有无及程度对EFE进行独立分级。由于这是一项关于RV EFE分级的新研究，因此进行了观察者内和观察者间的比较。分析了EFE严重程度、解剖学变量与孕晚期循环结局之间的关联。
共识别出81例患有RV EFE的患者。经共识，EFE严重程度被评估为1级（n = 66，81.5%）或2级（n = 15，18.5%）。在首次会诊时，2级EFE胎儿的RV球形度值高于1级EFE胎儿，这意味着更严重的顺应性降低和更差的舒张功能。从首次会诊到孕晚期，1级和2级RV EFE组之间在三尖瓣/二尖瓣（TV/MV）环直径变化（P = 0.042）和TV评分变化（P = 0.001）方面存在显著差异。在接受胎儿心脏介入治疗（FCI）的10例患者中，2级RV EFE胎儿的TV评分恢复比1级EFE胎儿更显著。在2级EFE病例中，与未接受FCI的胎儿相比，接受FCI的胎儿在右/左心室（RV/LV）长轴尺寸、TV/MV和RV球形度方面表现出更大的变化，这表明FCI通过恢复心室结构的发育使2级EFE胎儿受益。
本研究对患有CPS/PA-IVS的胎儿的RV EFE进行了分级，揭示了其对循环结局的影响。FCI对2级RV EFE患者有益，表明其在保护患有CPS/PA-IVS的受影响胎儿心脏发育方面具有潜力。
