A case of an asymptomatic 4-year-old girl with hemodynamically significant type II atrial septal defect is described. The patient underwent TEE in order to assess the eligibility for interventional device closure. Due to unfavorable anatomy and suspicion of the sinus venous type of the defect the child was selected for surgical treatment. No abnormalities were detected during surgery but a superfluous left atrial return of the venous blood during the cardiopulmonary bypass was noticed. The postoperative catheterisation was performed to elucidate this finding since the intraoperative TEE was normal. An anomalous venous drainage from superior and medial lobes of the right lung to the inferior vena cava was revealed. The 'scimitar' syndrome was diagnosed. Next, the patient underwent a successful surgical correction of this abnormality. The study presents the epidemiology, symptomatology and treatment modalities of the syndrome.
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