Scimitar syndrome with different features in pediatric patients: a single-center experience
Öz
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.
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.
Anahtar Kelimeler
Aortopulmonary collateral, children, meandering pulmonary veins, Scimitar syndrome
Kaynakça
- 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.
