Abstract
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.
References
- Wang CC, Wu ET, Chen SJ, Lu F, et al. Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 2008;167:155-160. Gao Y-A, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. Journal of the American College of Cardiology. 1993;22:873-882. Vida VL, Padrini M, Boccuzzo G, et al. Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: a multicenter study of the Italian society of pediatric cardiology. Rev Esp Cardiol (Engl Ed). 2013;66:556-560.
Abstract
Amaç: Scimitar sendromu, pulmoner venlerin sol atriyum yerine inferior vena kavaya (IVC) açıldığı ve sıklıkla ek kardiyak ve pulmoner anomalilerle ilişkili olduğu nadir görülen bir doğuştan kalp anomalisidir. Bu yazıda farklı klinik özellikleri olan Scimitar sendromlu pediyatrik hastalar tartışılmıştır.
Gereç ve yöntem: Hastanemizde 2000-2020 yılları arasında Scimitar sendromlu tanısı alan olgular çalışmaya alındı. Hastaların klinik bulguları, anjiyografik ve cerrahi işlemler ve takipleri değerlendirildi.
Bulgular: Çalışma süresi içinde yaşları 1,5 ay ile 10 yaş arasında Scimitar sendromu tanısıolan beş hasta değerlendirildi. Tüm hastalar semptomatikti. Bir hastada kor triatriatum ve pulmoner venöz stenoz, bir hastada sol atriyal izomerizm ve IVC yokluğu (azigos ven devamlılığı) ve diğer hastada aort koarktasyonu vardı. Diğer bir hastada ise çift drenaj saptandı. Bu hastada İVC’nin meandering pulmoner venler aracılığıyla sol atriyum ile olan bağlantısı Scimitar varyantı olarak değerlendirildi. Pulmoner hipertansiyonlu infantil bir hasta ve pulmoner / sistemik akım oranı (Qp/Qs)>1,5 olan iki hasta reanastomoz tekniği ile cerrahi olarak tedavi edildi. İki hastada ise aortopulmoner kollateraller saptanarak bu olgulara vasküler embolizasyon (vasküler plug, koil ve onyx kullanılarak) yapıldı.
Sonuç: Scimitar sendromunun çok çeşitli anatomik ve klinik varyasyonları olması nedeniyle tedavi ve takip kişiye özel olarak planlanmalıdır.
References
- Wang CC, Wu ET, Chen SJ, Lu F, et al. Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 2008;167:155-160. Gao Y-A, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. Journal of the American College of Cardiology. 1993;22:873-882. Vida VL, Padrini M, Boccuzzo G, et al. Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: a multicenter study of the Italian society of pediatric cardiology. Rev Esp Cardiol (Engl Ed). 2013;66:556-560.
Details
| Primary Language | English |
|---|---|
| Subjects | Cardiovascular Surgery, Paediatrics |
| Journal Section | Research Article |
| Authors | |
| Publication Date | January 31, 2023 |
| Submission Date | March 30, 2022 |
| Acceptance Date | September 29, 2022 |
| Published in Issue | Year 2023 Volume: 16 Issue: 1 |
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