Scimitar syndrome: restoration of native pulmonary venous connection

Abstract

We report a case of a 10-year-old girl with a typical scimitar syndrome. As the patient had dilated inferior vena cava along with an associated atrial septal defect, the surgical management consisted of intra-caval/intra-atrial baffle to direct the scimitar vein blood flow into the left atrium through the atrial septal defect. What is atypical is that at the 6-month follow-up, the echocardiography showed mild gradient in the baffle of 3 mmHg with normal right ventricular systolic pressure and at 1 year no flow in the baffle with normal native right-sided pulmonary venous flow, which was never detected before. The cardiac catheterization showed complete obstruction of the baffle and of scimitar vein and, to our pleasant surprise, complete restoration of the native right-sided pulmonary venous connection and normal pulmonary pressure.