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Clinical spectrum of ebstein’s anomaly: From fetal life to adolescence
Trends in Pediatrics 2026 Cilt 2 Sayı 7
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Objective: Ebstein’s anomaly is a rare congenital heart defect featuring a varied spectrum of clinical presentation. Anatomical variability in the malformation, together with a frequent association with other cardiac defects and arrhythmias, can complicate diagnosis and long-term management. Methods: Data from a total of 26 patients with Ebstein’s anomaly followed up at two university clinics between 2009 and 2025 were analyzed retrospectively. Cases diagnosed from the prenatal period through adolescent period were included. Results: The cohort consisted of 26 patients; 69.2% (18/26) were males with a median diagnostic age of 8 days. Diagnoses were established prenatally 15.4% (4/26), neonatally 50.0% (13/26), or during later childhood and adolescence 34.6% (9/26). Diagnosis was made during the index NICU admission in 34.6% (9/26) of patients and based on referral for murmur 26.9% (7/26) or cyanosis 7.7% (2/26). Common associated anomalies included atrial-level shunts 61.5% (16/26), mitral regurgitation 23.1% (6/26), and ventricular septal defects 15.4% (4/26). Arrhythmias were documented in 23.1% (6/21) of the cohort; 42.3% (11/26) underwent catheter-based procedures, and 30.8% (8/26) required surgery (median age at first surgery, 8.4 months), with 15.4%(4/26) undergoing reoperation. Over a median 5-year follow-up, mortality was 15.4% (4/26), with no perioperative deaths. Among prenatally diagnosed cases (n=4), three deaths occurred (3/4, 75%). Conclusions: The clinical spectrum of Ebstein’s anomaly is exceptionally broad. Our findings show frequent left heart involvement and a substantial need for catheter-based and surgical interventions, supporting the importance of individualized assessment and follow-up across the disease course.
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Kaynak: TRENDS IN PEDIATRICS · s. 137-144
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Dergi Trends in Pediatrics
ISSN 2792-0429
Yıl 2026 / 7. ay
Cilt / Sayı 2 / 7
Sayfalar 137 – 144
Makale Türü Özgün Makale
Hakemlik Hakemli
Endeks ESCI
Yayın Dili İngilizce
Kapsam Uluslararası
Toplam Yazar 8 kişi
Erişim Türü Basılı+Elektronik
Alan Sağlık Bilimleri Temel Alanı Çocuk Kardiyolojisi (Çocuk Sağlığı ve Hastalıkları)

YÖKSİS Yazar Kaydı

Yazar Adı SARAÇ UĞUR,PAYDAŞ AYŞE BÜŞRA,DOĞAN İREM,DOĞAN MELİH TİMUÇİN,SERT AHMET,ŞAP FATİH,BAYSAL TAMER,OFLAZ MEHMET BURHAN
YÖKSİS ID 9634649

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Havuz Atıfları 0
Yazar Sayısı 8