Scimitar syndrome with different features in pediatric patients: a single-center experience Çocuk hastalarda farklı özellikleriyle Scimitar sendromu: tek merkez deneyimi
Pamukkale Medical Journal, cilt.16, sa.1, ss.37-42, 2023 (Scopus, TRDizin)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 16 Sayı: 1
- Basım Tarihi: 2023
- Doi Numarası: 10.31362/patd.1094198
- Dergi Adı: Pamukkale Medical Journal
- Derginin Tarandığı İndeksler: Scopus, TR DİZİN (ULAKBİM)
- Sayfa Sayıları: ss.37-42
- Anahtar Kelimeler: Aortopulmonary collateral, children, meandering pulmonary veins, Scimitar syndrome
- Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Purpose: Scimitar syndrome is a rare congenital cardiac anomaly in which pulmonary veins are drained to the inferior vena cava (IVC) instead of the left atrium and it is often associated with additional cardiac and pulmonary anomalies. In this article, the different clinical features of pediatric patients with Scimitar syndrome are reported. Material and methods: All patients with scimitar syndrome were diagnosed in our hospital between 2000 and 2020. The clinical findings, angiographic and surgical procedures, and follow-up of the patients are evaluated. Results: Five pediatric patients aged between 1.5 months and 10 years were diagnosed as Scimitar syndrome. All patients were symptomatic. One patient had cor triatriatum and pulmonary venous stenosis, one patient had left atrial isomerism and absence of the IVC (azygous continuity), and another patient had coarctation of the aorta. One patient had dual drainage: the IVC and left atrium with meandering pulmonary veins, which we determined as a Scimitar variant. One infant patient with pulmonary hypertension and two patients with pulmonary to systemic flow ratio (Qp/Qs)>1.5 were treated surgically with the reanastomosis technique. Vascular embolization of the aortopulmonary collaterals was performed in two patients using vascular plugs, coils, and onyx. Conclusion: Treatment should be planned individually in this syndrome due to Scimitar syndrome has a wide range of anatomic and clinical variations.