DOPPLER INDICES AND PERINATAL OUTCOMES IN FETUSES WITH EBSTEIN’S ANOMALY

Abstract

Objective: Ebstein’s anomaly (EA) is a rare congenital malformation associated with high perinatal mortality. In this study, we aimed to assess perinatal outcomes and factors associated with mortality in fetuses with EA. Material and Method: In this study, fetuses diagnosed with EA from 2016 to 2022 were included. Clinical information, ultrasonographic findings, and Doppler parameters were collected retrospectively. Result: A total of 14 fetuses with EA were included. Twelve patients had isolated EA, while one had unilateral renal agenesis and the other had mega cisterna magna. Amniocentesis was performed in six cases and normal results were obtained. Nine fetuses reached term pregnancy. All fetuses survived to birth. Three cases died in the first week after birth. Survivors were found to have lower tricuspid valve annulus diameter z score when compared with non-survivors (4.81±3.05 vs 6.97±1.26, respectively, p=0.05). Reversal pulmonary artery flow (2/11 vs 4/4 in survivors and non-survivors, respectively, p=0.01) and pulmonary atresia (1/11 vs 4/4 in survivors and non-survivors, respectively, p<0.01) were significantly more frequent in non-survivors. Conclusion: Perinatal mortality remains high in EA. Higher tricuspid valve annulus diameter z score, presence of reversal pulmonary artery flow, and pulmonary atresia during prenatal diagnosis are associated with poorer diagnosis. Future multicenter studies are warranted to identify risk factors and guide perinatal management.

Keywords

• Doppler
• echocardiography
• ultrasonography
• Ebstein’s anomaly

Ethical Statement

Çalışma, Zeynep Kamil Kadın ve Çocuk Hastalıkları Eğitim ve Araştırma Hastanesi Etik Komitesi tarafından onaylanmıştır (Onay numarası: [05.04.2023-52])

EBSTEİN ANOMALİLİ FETÜSLERDE DOPPLER İNDEKSLERİ VE PERİNATAL SONUÇLAR

Abstract

Amaç: Ebstein anomalisi (EA) yüksek perinatal mortalite ile ilişkili nadir bir konjenital malformasyondur. Bu çalışmada, EA'lı fetüslerde perinatal sonuçları ve mortalite ile ilişkili faktörleri değerlendirme amaçlandı. Gereç ve Yöntem: Bu çalışmaya, 2016-2022 yılları arasında EA tanısı alan fetüsler çalışmaya dahil edilmiştir. Klinik bilgiler, ultrasonografik bulgular ve Doppler parametreleri retrospektif olarak toplandı. Bulgular: EA'lı toplam 14 fetüs çalışmaya dahil edildi. On iki hastada izole EA, birinde tek taraflı renal agenezi ve diğerinde mega sisterna magna vardı. Amniyosentez altı olguda yapıldı ve normal sonuçlar elde edildi. Dokuz fetüs term gebeliğe ulaştı. Tüm fetüsler doğuma kadar hayatta kaldı. Üç olgu doğumdan sonraki ilk hafta içinde kaybedildi. Hayatta kalanların triküspit kapak anulus çapı z skoru hayatta kalmayanlara göre daha düşük bulundu (sırasıyla 4,81±3,05 vs 6,97±1,26, p=0,05). Pulmoner arterde ters akım (hayatta kalanlarda ve hayatta kalmayanlarda sırasıyla 2/11 vs 4/4, p=0,01) ve pulmoner atrezi (hayatta kalanlarda ve hayatta kalmayanlarda sırasıyla 1/11 vs 4/4, p<0,01) hayatta kalmayanlarda anlamlı olarak daha sıktı. Sonuç: EA'da perinatal mortalite hala yüksektir. Prenatal tanı sırasında daha yüksek triküspit kapak anulus çapı z skoru, pulmoner arterde ters akım ve pulmoner atrezi varlığı daha kötü prognoz ile ilişkilidir. Risk faktörlerini belirlemek ve perinatal dönemi yönetmek için gelecekte yapılacak çok merkezli çalışmalara ihtiyaç vardır.

Keywords

• Doppler
• ekokardiyografi
• ultrasonografi
• Ebstein anomalisi

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