Assessment of right ventricular endocardial fibroelastosis in fetuses with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum

Yue Wang; Gang Luo; Yi Sun; Taotao Chen; Silin Pan

doi:10.3389/fped.2024.1518898

Assessment of right ventricular endocardial fibroelastosis in fetuses with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum

Front Pediatr. 2025 Jan 10:12:1518898. doi: 10.3389/fped.2024.1518898. eCollection 2024.

Authors

Yue Wang¹, Gang Luo¹, Yi Sun¹, Taotao Chen², Silin Pan¹

Affiliations

¹ Heart Center, Women and Children's Hospital, Qingdao University, Qingdao, China.
² Department of Obstetric Ultrasound, Women and Children's Hospital, Qingdao University, Qingdao, China.

Abstract

Background: 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.

Methods: 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.

Results: Eighty-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.

Conclusions: 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.

Keywords: critical pulmonary stenosis; endocardial fibroelastosis; fetal echocardiogram; pulmonary atresia with intact ventricular septum; right ventricle.