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

  • Maternal-Fetal Medicine
  • Published:
Archives of Gynecology and Obstetrics Aims and scope Submit manuscript

Abstract

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.

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Data availability

Data sharing is not applicable to this study as no new data were created in this article.

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Authors and Affiliations

  1. Department of Pediatric Cardiology, İstanbul University-Cerrahpaşa Cerrahpaşa Medical School, Istanbul, Turkey

    Reyhan Dedeoglu

  2. Department of Pediatric Cardiology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey

    Damla Gokcer Akbulut & Helen Bornaun

  3. Department of Obstetrics and Gynecology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey

    Emine Turkmen

  4. Department of Pediatrics, Uskudar University Medical Faculty, Istanbul, Turkey

    Savas Dedeoglu

Authors
  1. Reyhan Dedeoglu
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  2. Damla Gokcer Akbulut
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  3. Emine Turkmen
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  4. Savas Dedeoglu
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  5. Helen Bornaun
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Contributions

Reyhan Dedeoglu, Helen Bornaun, Savas Dedeoglu contributed to the design and data analysis and critically read the final manuscript. Damla Gokçer Akbulut and Emine Turkmen were responsible for the collection of field data of epidemiological investigation and follow-up work. The authors read and approved the final manuscript.

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Correspondence to Reyhan Dedeoglu.

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Conflict of interest

The authors declare that they have no conflicts of interest to disclose.

Ethical approval

Informed consent was obtained from the parents or legal guardians of all individual participants included in the study. The study was approved by the institution review board.

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Informed consent was obtained from all patients included in this article.

Human rights statements

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later versions.  

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Dedeoglu, R., Akbulut, D.G., Turkmen, E. et al. Perinatal outcomes following early prenatal diagnosis: insights from a single-center experience with Ebstein anomaly and tricuspid valve dysplasia. Arch Gynecol Obstet (2024). https://doi.org/10.1007/s00404-024-07509-y

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  • DOI: https://doi.org/10.1007/s00404-024-07509-y

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