Data Sheet 1_Assessment of right ventricular endocardial fibroelastosis in fetuses with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum.pdf

BackgroundThis 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.
MethodsFetal 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.
ResultsEighty-one patients with RV EFE were identified. By consensus, EFE severity was assessed as Grade 1 (n = 66, 81.5%) or Grade 2 (n = 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 (P = 0.042) and TV z-score (P = 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 z-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.
ConclusionsThis 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.

背景 本研究旨在评估合并重度肺动脉狭窄（CPS）及室间隔完整型肺动脉闭锁（PA-IVS）胎儿的右心室（RV）心内膜弹力纤维增生症（EFE）情况，并探讨RV EFE对胎儿循环结局的影响。
方法 收集2018年7月至2021年1月的胎儿超声心动图数据。由3名评审员基于心内膜回声强度的有无及范围，独立对EFE进行分级。鉴于本研究是针对RV EFE分级的首创性研究，故开展了观察者内及观察者间一致性比较。分析了EFE严重程度、解剖学变量与妊娠晚期循环结局之间的关联。
结果 共纳入81例RV EFE胎儿。经共识评定，EFE严重程度分为1级（n=66，占比81.5%）与2级（n=15，占比18.5%）。首次就诊时，2级EFE胎儿的右心室球形度数值高于1级EFE胎儿，提示其心室顺应性下降更显著、舒张功能更差。从首次就诊至妊娠晚期，1级与2级RV EFE组的三尖瓣/二尖瓣（TV/MV）瓣环直径变化量（P=0.042）及三尖瓣Z评分变化量（P=0.001）均存在显著差异。在10例行胎儿心脏介入治疗（FCI）的胎儿中，2级RV EFE胎儿的三尖瓣Z评分恢复程度较1级EFE胎儿更为显著。在2级EFE病例中，与未行FCI的胎儿相比，行FCI的胎儿在右心室/左心室（RV/LV）长轴径、TV/MV瓣环直径及右心室球形度的变化幅度更大，表明FCI可通过恢复心室结构发育，使2级EFE胎儿获益。
结论 本研究对合并CPS/PA-IVS的胎儿RV EFE进行了分级，阐明了其对循环结局的潜在影响。胎儿心脏介入治疗（FCI）可使2级RV EFE患者获益，提示该治疗手段或可保护合并CPS/PA-IVS的患病胎儿的心脏发育。