Perinatal outcomes following early prenatal diagnosis: insights from a single-center experience with Ebstein anomaly and tricuspid valve dysplasia.

Autor: Dedeoglu R; Department of Pediatric Cardiology, İstanbul University-Cerrahpaşa Cerrahpaşa Medical School, Istanbul, Turkey. reyhandedeoglu@gmail.com., Akbulut DG; Department of Pediatric Cardiology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey., Turkmen E; Department of Obstetrics and Gynecology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey., Dedeoglu S; Department of Pediatrics, Uskudar University Medical Faculty, Istanbul, Turkey., Bornaun H; Department of Pediatric Cardiology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey.
Jazyk: angličtina
Zdroj: Archives of gynecology and obstetrics [Arch Gynecol Obstet] 2024 Sep; Vol. 310 (3), pp. 1491-1497. Date of Electronic Publication: 2024 Apr 29.
DOI: 10.1007/s00404-024-07509-y
Abstrakt: Purpose: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) represent uncommon congenital malformations of the tricuspid valve. The purpose of this study is to report on current perinatal outcomes of EA/TVD in our center and to investigate clinical and fetal echocardiographic predictors of perinatal mortality.
Methods and Results: We performed a retrospective study among fetuses diagnosed from January 2014 to December 2023. Clinical and echocardiographic data were obtained from hospital records of Research and Education Hospital. The primary outcome was perinatal mortality. Of 21 fetuses diagnosed, there were 1 lost to follow-up, 1 termination, and 7 demises. In the live-born cohort of 12 live-born patients, 2 died before discharge, yielding an overall perinatal mortality of 50%. The median gestational age at diagnosis was 23 for non-survivors and 24 weeks for survivors. Birth weight was lower in non-survivors (2430 g vs 2990 g). Tricuspid insufficiency severity varied insignificantly. Non-survivors exhibited higher rates of hydrops, functional atresia, and absent antegrade flow (p < 0.05). Two infants with severe tricuspid insufficiency and congenital abnormalities died postnatally. The limited dataset enables further analysis for a predictive model. Notably, all non-survivors displayed hydrops, functional atresia, and absent antegrade flow, hindering definitive determination of the most impactful parameter on survival estimation.
Conclusion: Perinatal mortality remains notably elevated in fetuses with EA/TVD. The individuals at the highest risk are those with antegrade flow loss and functional atresia of pulmonary valve, this high-risk subgroup could benefit from targeted interventions, such as novel prenatal therapies or a more comprehensive perinatal approach involving optimized timing of delivery and postnatal interventional strategies.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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