Scimitar Syndrome with Different Features in Pediatric Patients: A Single-Center Experience

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. Scimitar syndrome has a wide range of anatomic and clinical variations. In this article, the different clinical features of five pediatric patients with Scimitar syndrome are reported.
 Material and methods: Five 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; two of the five patients were infants. 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. Transcatheter occlusion of the aortopulmonary collaterals was performed in two patients using vascular plugs, coils, and onyx.
 Conclusion: In this article, the clinical findings of five pediatric patients with Scimitar syndrome were evaluated in detailed. Treatment should be planned individually in this syndrome due to Scimitar syndrome has a wide range of anatomic and clinical variations.