{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T08:19:15Z","timestamp":1792225155461,"version":"4.4.0"},"reference-count":29,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T00:00:00Z","timestamp":1736467200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Eighty-one patients with RV EFE were identified. By consensus, EFE severity was assessed as Grade 1 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200966, 81.5%) or Grade 2 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200915, 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 (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.042) and TV\n                      <jats:italic>z<\/jats:italic>\n                      -score (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.001) 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\n                      <jats:italic>z<\/jats:italic>\n                      -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.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2024.1518898","type":"journal-article","created":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T01:14:12Z","timestamp":1736471652000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Assessment of right ventricular endocardial fibroelastosis in fetuses with critical pulmonary stenosis and pulmonary atresia with intact ventricular 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