Three-dimensional transesophageal echocardiography enhances multimodal imaging of a successful repair of a case with scimitar syndrome.

Scimitar syndrome is a rare congenital disorder with an incidence of approximately 2 per 100 000 live births.1 The hallmark of this syndrome is partial or complete anomalous venous return from the right lung, directly into the inferior vena cava (IVC), thereby creating a left-to-right shunt.2 The syndrome is almost invariably associated with other cardiac and extracardiac abnormalities including atrial septal defects (ASD), dextrocardia, abnormal right lung lobation, and right lung hypoplasia.3 The infantile syndrome is highly associated with ASDs, ventricular septal defects, patent ductus arteriosus, coarctation, and tetralogy of Fallot.3 The abnormal venous drainage gives the characteristic radiographic appearance akin to that of a “scimitar” (Turkish sword) with a shadow on the right side of the heart progressively widening as it approaches the cardiophrenic angle.3 The expected natural course of uncorrected scimitar syndrome is similar to uncorrected large ASD with eventual right heart failure, and therefore even asymptomatic adults with Qp:Qs ratio of more than 1.5:1 should undergo corrective surgery.1 Catheterization is often recommended and advised to assess pulmonary vascular resistance and suitability for operative repair. Other imaging modalities include preoperative echocardiography, cardiac MRI, or computed tomography (CT). Which of these latter techniques is chosen depends on institutional preference and experience, with factors such as cost, the potential need for patient sedation, exposure to ionizing radiation, and exposure to intravenous contrast material also to be considered in the choice of imaging modality. Perioperative transesophageal echocardiography (TEE) has previously been shown to be useful for demonstrating the abnormal scimitar vein anatomy and also for confirming unobstructed blood flow after anastomosis of the scimitar vein to the left atrium after repair.4 In this article, we